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Sample records for perinatal asphyxial encephalopathy

  1. The TOBY Study. Whole body hypothermia for the treatment of perinatal asphyxial encephalopathy: A randomised controlled trial

    PubMed Central

    Azzopardi, Dennis; Brocklehurst, Peter; Edwards, David; Halliday, Henry; Levene, Malcolm; Thoresen, Marianne; Whitelaw, Andrew

    2008-01-01

    Background A hypoxic-ischaemic insult occurring around the time of birth may result in an encephalopathic state characterised by the need for resuscitation at birth, neurological depression, seizures and electroencephalographic abnormalities. There is an increasing risk of death or neurodevelopmental abnormalities with more severe encephalopathy. Current management consists of maintaining physiological parameters within the normal range and treating seizures with anticonvulsants. Studies in adult and newborn animals have shown that a reduction of body temperature of 3–4°C after cerebral insults is associated with improved histological and behavioural outcome. Pilot studies in infants with encephalopathy of head cooling combined with mild whole body hypothermia and of moderate whole body cooling to 33.5°C have been reported. No complications were noted but the group sizes were too small to evaluate benefit. Methods/Design TOBY is a multi-centre, prospective, randomised study of term infants after perinatal asphyxia comparing those allocated to "intensive care plus total body cooling for 72 hours" with those allocated to "intensive care without cooling". Full-term infants will be randomised within 6 hours of birth to either a control group with the rectal temperature kept at 37 +/- 0.2°C or to whole body cooling, with rectal temperature kept at 33–34°C for 72 hours. Term infants showing signs of moderate or severe encephalopathy +/- seizures have their eligibility confirmed by cerebral function monitoring. Outcomes will be assessed at 18 months of age using neurological and neurodevelopmental testing methods. Sample size At least 236 infants would be needed to demonstrate a 30% reduction in the relative risk of mortality or serious disability at 18 months. Recruitment was ahead of target by seven months and approvals were obtained allowing recruitment to continue to the end of the planned recruitment phase. 325 infants were recruited. Primary outcome Combined

  2. The mechanisms and treatment of asphyxial encephalopathy

    PubMed Central

    Wassink, Guido; Gunn, Eleanor R.; Drury, Paul P.; Bennet, Laura; Gunn, Alistair J.

    2013-01-01

    Acute post-asphyxial encephalopathy occurring around the time of birth remains a major cause of death and disability. The recent seminal insight that allows active neuroprotective treatment is that even after profound asphyxia (the “primary” phase), many brain cells show initial recovery from the insult during a short “latent” phase, typically lasting approximately 6 h, only to die hours to days later after a “secondary” deterioration characterized by seizures, cytotoxic edema, and progressive failure of cerebral oxidative metabolism. Although many of these secondary processes are potentially injurious, they appear to be primarily epiphenomena of the “execution” phase of cell death. Animal and human studies designed around this conceptual framework have shown that moderate cerebral hypothermia initiated as early as possible but before the onset of secondary deterioration, and continued for a sufficient duration to allow the secondary deterioration to resolve, has been associated with potent, long-lasting neuroprotection. Recent clinical trials show that while therapeutic hypothermia significantly reduces morbidity and mortality, many babies still die or survive with disabilities. The challenge for the future is to find ways of improving the effectiveness of treatment. In this review, we will dissect the known mechanisms of hypoxic-ischemic brain injury in relation to the known effects of hypothermic neuroprotection. PMID:24578682

  3. Asphyxiants.

    PubMed

    Borron, Stephen W; Bebarta, Vikhyat S

    2015-02-01

    Asphyxiants deprive the body of oxygen. Simple asphyxiants displace oxygen from the lungs, whereas systemic asphyxiants interfere with transport of oxygen by hemoglobin or with mitochondrial oxidative phosphorylation. Asphyxiants may be gases, liquids, or solids, or their metabolites. The typical clinical picture of asphyxiant poisoning is one of progressive mental status changes, alteration of breathing, progressively abnormal vital signs, coma, seizures, and eventually cardiovascular collapse and death. Treatment of asphyxiant poisoning is aggressive supportive care, with control of the airway and ventilation and maintenance of cardiac output. Supportive care is often enhanced by the administration of specific antidotes.

  4. Blood Biomarkers for Evaluation of Perinatal Encephalopathy

    PubMed Central

    Graham, Ernest M.; Burd, Irina; Everett, Allen D.; Northington, Frances J.

    2016-01-01

    Recent research in identification of brain injury after trauma shows many possible blood biomarkers that may help identify the fetus and neonate with encephalopathy. Traumatic brain injury shares many common features with perinatal hypoxic-ischemic encephalopathy. Trauma has a hypoxic component, and one of the 1st physiologic consequences of moderate-severe traumatic brain injury is apnea. Trauma and hypoxia-ischemia initiate an excitotoxic cascade and free radical injury followed by the inflammatory cascade, producing injury in neurons, glial cells and white matter. Increased excitatory amino acids, lipid peroxidation products, and alteration in microRNAs and inflammatory markers are common to both traumatic brain injury and perinatal encephalopathy. The blood-brain barrier is disrupted in both leading to egress of substances normally only found in the central nervous system. Brain exosomes may represent ideal biomarker containers, as RNA and protein transported within the vesicles are protected from enzymatic degradation. Evaluation of fetal or neonatal brain derived exosomes that cross the blood-brain barrier and circulate peripherally has been referred to as the “liquid brain biopsy.” A multiplex of serum biomarkers could improve upon the current imprecise methods of identifying fetal and neonatal brain injury such as fetal heart rate abnormalities, meconium, cord gases at delivery, and Apgar scores. Quantitative biomarker measurements of perinatal brain injury and recovery could lead to operative delivery only in the presence of significant fetal risk, triage to appropriate therapy after birth and measure the effectiveness of treatment. PMID:27468268

  5. [Perinatal encephalopathy sequelae identified by a neurobehavioral scale in one year old infants].

    PubMed

    Chávez-Torres, Raquel; Sánchez-Pérez, Carmen; Pérez-Tejada, Haroldo Elorza; Flores-Huerta, Samuel; Klünder Klünder, Miguel; Ruiz-Chávez, Jaime; Luna-Sánchez, Yolanda; Campos-Campos, Laura; Gómez-Barrera, Raúl; Villanueva-Padrón, Laura; Maldonado-Jiménez, Georgina

    2012-01-01

    to identify neurodevelopmental sequelae in one year old infants with perinatal encephalopathy utilizing the neurobehavioral scale named Vanedela. a cohort of 75 newborns with perinatal encephalopathy was assessed with a neurobehavioral follow-up scale at age of 1, 4, 8 and 12 months. A distinction was made between functional, structural and combined encephalopathy. Two groups of neurodevelopmental outcome at one year were identified: with or without sequelae. Nonparametric statistics was used. infants with functional encephalopathy had the best scores, followed by those with structural encephalopathy, while infants with a combined encephalopathy had the lowest scores. At one year of age, the group with neurobehavioral sequelae exhibited the lowest scores and retarded growth. At the same age, the group with functional encephalopathy exhibited no neurobehavioral sequelae, and reached better scores and growth. the neurobehavioral follow-up scale is able to identify the neurodevelopmental sequelae at the age of one year in infants with perinatal encephalopathy. The application of Vanedela in the clinical field requires of little time, its results are trustworthy and very useful for the neurobehavioral follow-up assessment.

  6. Efficacy of passive hypothermia and adverse events during transport of asphyxiated newborns according to the severity of hypoxic-ischemic encephalopathy.

    PubMed

    Carreras, Nuria; Alsina, Miguel; Alarcon, Ana; Arca-Díaz, Gemma; Agut, Thais; García-Alix, Alfredo

    2017-08-18

    To determine if the efficacy of passive hypothermia and adverse events during transport are related to the severity of neonatal hypoxic-ischemic encephalopathy. This was a retrospective study of 67 infants with hypoxic-ischemic encephalopathy, born between April 2009 and December 2013, who were transferred for therapeutic hypothermia and cooled during transport. Fifty-six newborns (84%) were transferred without external sources of heat and 11 (16%) needed an external heat source. The mean temperature at departure was 34.4±1.4°C and mean transfer time was 3.3±2.0h. Mean age at arrival was 5.6±2.5h. Temperature at arrival was between 33 and 35°C in 41 (61%) infants, between 35°C and 36.5°C in 15 (22%) and <33°C in 11 (16%). Infants with severe hypoxic-ischemic encephalopathy had greater risk of having an admission temperature<33°C (OR: 4.5; 95% CI: 1.1-19.3). The severity of hypoxic-ischemic encephalopathy and the umbilical artery pH were independent risk factors for a low temperature on admission (p<0.05). Adverse events during transfer, mainly hypotension and bleeding from the endotracheal tube, occurred in 14 infants (21%), with no differences between infants with moderate or severe hypoxic-ischemic encephalopathy. The risk of overcooling during transport is greater in newborns with severe hypoxic-ischemic encephalopathy and those with more severe acidosis at birth. The most common adverse events during transport are related to physiological deterioration and bleeding from the endotracheal tube. This observation provides useful information to identify those asphyxiated infants who require closer clinical surveillance during transport. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  7. Anatomical patterns and correlated MRI findings of non-perinatal hypoxic–ischaemic encephalopathy

    PubMed Central

    White, M L; Zhang, Y; Helvey, J T; Omojola, M F

    2013-01-01

    ABSTRACT. Non-perinatal hypoxic–ischaemic encephalopathy (HIE) has varying anatomical patterns dependent on the type of insult, the degree and duration of cerebral hypoxia, or presence and degree of hypoperfusion. Profound insults can affect the entire cerebral cortex or just the perirolandic cortex, the cerebellum and the deep grey matter structures. Less severe insults may affect only the watershed regions. The objective of this article is to review the anatomical patterns of non-perinatal HIEs by MRI. PMID:23255548

  8. Anatomical patterns and correlated MRI findings of non-perinatal hypoxic-ischaemic encephalopathy.

    PubMed

    White, M L; Zhang, Y; Helvey, J T; Omojola, M F

    2013-01-01

    Non-perinatal hypoxic-ischaemic encephalopathy (HIE) has varying anatomical patterns dependent on the type of insult, the degree and duration of cerebral hypoxia, or presence and degree of hypoperfusion. Profound insults can affect the entire cerebral cortex or just the perirolandic cortex, the cerebellum and the deep grey matter structures. Less severe insults may affect only the watershed regions. The objective of this article is to review the anatomical patterns of non-perinatal HIEs by MRI.

  9. HIV-1 Encephalopathy among Perinatally Infected Children: Neuropathogenesis and Response to Highly Active Antiretroviral Therapy

    ERIC Educational Resources Information Center

    Mitchell, Charles D.

    2006-01-01

    HIV-1 encephalopathy among perinatally infected children in the United States was initially defined by a classic triad of findings that included: (1) developmental delay, (2) secondary or acquired microcephaly, and (3) pyramidal tract neuromotor deficits. The most severe form of this disorder typically occurred among young children who developed…

  10. HIV-1 Encephalopathy among Perinatally Infected Children: Neuropathogenesis and Response to Highly Active Antiretroviral Therapy

    ERIC Educational Resources Information Center

    Mitchell, Charles D.

    2006-01-01

    HIV-1 encephalopathy among perinatally infected children in the United States was initially defined by a classic triad of findings that included: (1) developmental delay, (2) secondary or acquired microcephaly, and (3) pyramidal tract neuromotor deficits. The most severe form of this disorder typically occurred among young children who developed…

  11. Impact of HAART and CNS-penetrating antiretroviral regimens on HIV encephalopathy among perinatally infected children and adolescents.

    PubMed

    Patel, Kunjal; Ming, Xue; Williams, Paige L; Robertson, Kevin R; Oleske, James M; Seage, George R

    2009-09-10

    Prior to antiretroviral treatment, HIV-infected children frequently developed encephalopathy, resulting in debilitating morbidity and mortality. This is the first large study to evaluate the impact of HAART and central nervous system (CNS)-penetrating antiretroviral regimens on the incidence of HIV encephalopathy and survival after diagnosis of HIV encephalopathy among perinatally infected children. A total of 2398 perinatally HIV-infected children with at least one neurological examination were followed in a US-based prospective cohort study conducted from 1993 to 2007. Trends in incidence rates over calendar time were described and Cox regression models were used to estimate the effects of time-varying HAART and CNS-penetrating antiretroviral regimens on HIV encephalopathy and on survival after diagnosis of HIV encephalopathy. During a median of 6.4 years of follow-up, 77 incident cases of HIV encephalopathy occurred [incidence rate 5.1 cases per 1000 person-years, 95% confidence interval (CI) 4.0-6.3]. A 10-fold decline in incidence was observed beginning in 1996, followed by a stable incidence rate after 2002. HAART regimens were associated with a 50% decrease (95% CI 14-71%) in the incidence of HIV encephalopathy compared with non-HAART regimens. High CNS-penetrating regimens were associated with a substantial survival benefit (74% reduction in the risk of death, 95% CI 39-89%) after HIV encephalopathy diagnosis compared with low CNS-penetrating regimens. A dramatic decrease in the incidence of HIV encephalopathy occurred after the introduction of HAART. The use of HAART was highly effective in reducing the incidence of HIV encephalopathy among perinatally infected children and adolescents. Effective CNS-penetrating antiretroviral regimens are important in affecting survival after diagnosis of HIV encephalopathy. 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins

  12. Impact of HAART and CNS-penetrating antiretroviral regimens on HIV encephalopathy among perinatally infected children and adolescents

    PubMed Central

    Patel, Kunjal; Ming, Xue; Williams, Paige L.; Robertson, Kevin R.; Oleske, James M.; Seage, George R.

    2010-01-01

    Objectives Prior to antiretroviral treatment, HIV-infected children frequently developed encephalopathy, resulting in debilitating morbidity and mortality. This is the first large study to evaluate the impact of HAART and central nervous system (CNS)-penetrating antiretroviral regimens on the incidence of HIV encephalopathy and survival after diagnosis of HIV encephalopathy among perinatally infected children. Design A total of 2398 perinatally HIV-infected children with at least one neurological examination were followed in a US-based prospective cohort study conducted from 1993 to 2007. Methods Trends in incidence rates over calendar time were described and Cox regression models were used to estimate the effects of time-varying HAART and CNS-penetrating antiretroviral regimens on HIV encephalopathy and on survival after diagnosis of HIV encephalopathy. Results During a median of 6.4 years of follow-up, 77 incident cases of HIV encephalopathy occurred [incidence rate 5.1 cases per 1000 person-years, 95% confidence interval (CI) 4.0–6.3]. A 10-fold decline in incidence was observed beginning in 1996, followed by a stable incidence rate after 2002. HAART regimens were associated with a 50% decrease (95% CI 14–71%) in the incidence of HIV encephalopathy compared with non-HAART regimens. High CNS-penetrating regimens were associated with a substantial survival benefit (74% reduction in the risk of death, 95% CI 39–89%) after HIV encephalopathy diagnosis compared with low CNS-penetrating regimens. Conclusion A dramatic decrease in the incidence of HIV encephalopathy occurred after the introduction of HAART. The use of HAART was highly effective in reducing the incidence of HIV encephalopathy among perinatally infected children and adolescents. Effective CNS-penetrating antiretroviral regimens are important in affecting survival after diagnosis of HIV encephalopathy. PMID:19644348

  13. The association between antioxidant enzyme polymorphisms and cerebral palsy after perinatal hypoxic-ischaemic encephalopathy.

    PubMed

    Esih, Katarina; Goričar, Katja; Dolžan, Vita; Rener-Primec, Zvonka

    2016-09-01

    Hypoxic-ischaemic perinatal brain injury leads to the formation of reactive oxygen species (ROS) and the resultant cell and tissue damage may cause neurological sequelae such as cerebral palsy and/or epilepsy. A decrease in the capacity for defending against ROS may increase the susceptibility to cerebral palsy. The aim of this study was to investigate the impact of common functional polymorphisms in the antioxidant genes SOD2, GPX1 and CAT, associated with a decreased capacity for defending against ROS, in patients with perinatal hypoxic-ischaemic encephalopathy (HIE). 80 patients previously diagnosed with perinatal HIE were included. Genomic DNA was isolated from buccal swabs and genotyped for SOD2 rs4880, GPX1 rs1050450 and CAT rs1001179 using real-time PCR-based methods. Among patients with neonatal HIE, carriers of at least one polymorphic CAT rs1001179 T allele were significantly associated with development of cerebral palsy compared to non-carriers (univariate logistic regression, p = 0.026; OR = 3.36; 95% CI = 1.16-9.76). This difference remained statistically significant after accounting for prematurity. The investigated SOD2 and GPX1 polymorphisms were not associated with cerebral palsy after perinatal HIE. CAT rs1001179 polymorphism could be used to identify children that have a higher susceptibility to cerebral palsy after perinatal HIE. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  14. [Oxidative stress in perinatal asphyxia and hypoxic-ischaemic encephalopathy].

    PubMed

    Nuñez, Antonio; Benavente, Isabel; Blanco, Dorotea; Boix, Héctor; Cabañas, Fernando; Chaffanel, Mercedes; Fernández-Colomer, Belén; Fernández-Lorenzo, José Ramón; Loureiro, Begoña; Moral, María Teresa; Pavón, Antonio; Tofé, Inés; Valverde, Eva; Vento, Máximo

    2017-06-22

    Birth asphyxia is one of the principal causes of early neonatal death. In survivors it may evolve to hypoxic-ischaemic encephalopathy and major long-term neurological morbidity. Prolonged and intense asphyxia will lead to energy exhaustion in tissues exclusively dependent on aerobic metabolism, such as the central nervous system. Energy deficit leads to ATP-dependent pumps blockage, with the subsequent loss of neuronal transmembrane potential. The most sensitive areas of the brain will die due to necrosis. In more resistant areas, neuronal hyper-excitability, massive entrance of ionic calcium, activation of NO-synthase, free radical generation, and alteration in mitochondrial metabolism will lead to a secondary energy failure and programmed neuronal death by means of the activation of the caspase pathways. A third phase has recently been described that includes persistent inflammation and epigenetic changes that would lead to a blockage of oligodendrocyte maturation, alteration of neurogenesis, axonal maturation, and synaptogenesis. In this scenario, oxidative stress plays a critical role causing direct damage to the central nervous system and activating metabolic cascades leading to apoptosis and inflammation. Moderate whole body hypothermia to preserve energy stores and to reduce the formation of oxygen reactive species attenuates the mechanisms that lead to the amplification of cerebral damage upon resuscitation. The combination of hypothermia with coadjuvant therapies may contribute to improve the prognosis. Copyright © 2017. Publicado por Elsevier España, S.L.U.

  15. Anticonvulsant effect of xenon on neonatal asphyxial seizures.

    PubMed

    Azzopardi, Denis; Robertson, Nicola J; Kapetanakis, Andrew; Griffiths, James; Rennie, Janet M; Mathieson, Sean R; Edwards, A David

    2013-09-01

    Xenon, a monoatomic gas with very high tissue solubility, is a non-competitive inhibitor of N-methyl-D-aspartate (NMDA) glutamate receptor, has antiapoptotic effects and is neuroprotective following hypoxic ischaemic injury in animals. Xenon may be expected to have anticonvulsant effects through glutamate receptor blockade, but this has not previously been demonstrated clinically. We examined seizure activity on the real time and amplitude integrated EEG records of 14 full-term infants with perinatal asphyxial encephalopathy treated within 12 h of birth with 30% inhaled xenon for 24 h combined with 72 h of moderate systemic hypothermia. Seizures were identified on 5 of 14 infants. Seizures stopped during xenon therapy but recurred within a few minutes of withdrawing xenon and stopped again after xenon was restarted. Our data show that subanaesthetic levels of xenon may have an anticonvulsant effect. Inhaled xenon may be a valuable new therapy in this hard-to-treat population.

  16. Actualities on molecular pathogenesis and repairing processes of cerebral damage in perinatal hypoxic-ischemic encephalopathy.

    PubMed

    Distefano, Giuseppe; Praticò, Andrea D

    2010-09-16

    Hypoxic-ischemic encephalopathy (HIE) is the most important cause of cerebral damage and long-term neurological sequelae in the perinatal period both in term and preterm infant. Hypoxic-ischemic (H-I) injuries develop in two phases: the ischemic phase, dominated by necrotic processes, and the reperfusion phase, dominated by apoptotic processes extending beyond ischemic areas. Due to selective ischemic vulnerability, cerebral damage affects gray matter in term newborns and white matter in preterm newborns with the typical neuropathological aspects of laminar cortical necrosis in the former and periventricular leukomalacia in the latter. This article summarises the principal physiopathological and biochemical processes leading to necrosis and/or apoptosis of neuronal and glial cells and reports recent insights into some endogenous and exogenous cellular and molecular mechanisms aimed at repairing H-I cerebral damage.

  17. Actualities on molecular pathogenesis and repairing processes of cerebral damage in perinatal hypoxic-ischemic encephalopathy

    PubMed Central

    2010-01-01

    Hypoxic-ischemic encephalopathy (HIE) is the most important cause of cerebral damage and long-term neurological sequelae in the perinatal period both in term and preterm infant. Hypoxic-ischemic (H-I) injuries develop in two phases: the ischemic phase, dominated by necrotic processes, and the reperfusion phase, dominated by apoptotic processes extending beyond ischemic areas. Due to selective ischemic vulnerability, cerebral damage affects gray matter in term newborns and white matter in preterm newborns with the typical neuropathological aspects of laminar cortical necrosis in the former and periventricular leukomalacia in the latter. This article summarises the principal physiopathological and biochemical processes leading to necrosis and/or apoptosis of neuronal and glial cells and reports recent insights into some endogenous and exogenous cellular and molecular mechanisms aimed at repairing H-I cerebral damage. PMID:20846380

  18. Motor Testing at 1 Year Improves the Prediction of Motor and Mental Outcome at 2 Years after Perinatal Hypoxic-Ischaemic Encephalopathy

    ERIC Educational Resources Information Center

    van Schie, Petra Em; Becher, Jules G.; Dallmeijer, Annet J.; Barkhof, Frederik; van Weissenbruch, Mirjam M.; Vermeulen, R. Jeroen

    2010-01-01

    Aim: To investigate the predictive value of motor testing at 1 year for motor and mental outcome at 2 years after perinatal hypoxic-ischaemic encephalopathy (HIE) in term neonates. Method: Motor and mental outcome at 2 years was assessed with the Bayley Scales of Infant Development, 2nd edition (BSID-II) in 32 surviving children (20 males, 12…

  19. Motor Testing at 1 Year Improves the Prediction of Motor and Mental Outcome at 2 Years after Perinatal Hypoxic-Ischaemic Encephalopathy

    ERIC Educational Resources Information Center

    van Schie, Petra Em; Becher, Jules G.; Dallmeijer, Annet J.; Barkhof, Frederik; van Weissenbruch, Mirjam M.; Vermeulen, R. Jeroen

    2010-01-01

    Aim: To investigate the predictive value of motor testing at 1 year for motor and mental outcome at 2 years after perinatal hypoxic-ischaemic encephalopathy (HIE) in term neonates. Method: Motor and mental outcome at 2 years was assessed with the Bayley Scales of Infant Development, 2nd edition (BSID-II) in 32 surviving children (20 males, 12…

  20. Melatonin in the management of perinatal hypoxic-ischemic encephalopathy: light at the end of the tunnel?

    PubMed Central

    Hendaus, Mohamed A; Jomha, Fatima A; Alhammadi, Ahmed H

    2016-01-01

    Perinatal hypoxic-ischemic encephalopathy (HIE) affects one to three per 1,000 live full-term births and can lead to severe and permanent neuropsychological sequelae, such as cerebral palsy, epilepsy, mental retardation, and visual motor or visual perceptive dysfunction. Melatonin has begun to be contemplated as a good choice in order to diminish the neurological sequelae from hypoxic-ischemic brain injury. Melatonin emerges as a very interesting medication, because of its capacity to cross all physiological barriers extending to subcellular compartments and its safety and effectiveness. The purpose of this commentary is to detail the evidence on the use of melatonin as a neuroprotection agent. The pharmacologic aspects of the drug as well as its potential neuroprotective characteristics in human and animal studies are described in this study. Melatonin seems to be safe and beneficial in protecting neonatal brains from perinatal HIE. Larger randomized controlled trials in humans are required, to implement a long-awaited feasible treatment in order to avoid the dreaded sequelae of HIE. PMID:27729791

  1. Hypothermia therapy for newborns with hypoxic ischemic encephalopathy.

    PubMed

    Silveira, Rita C; Procianoy, Renato S

    2015-01-01

    Therapeutic hypothermia reduces cerebral injury and improves the neurological outcome secondary to hypoxic ischemic encephalopathy in newborns. It has been indicated for asphyxiated full-term or near-term newborn infants with clinical signs of hypoxic-ischemic encephalopathy (HIE). A search was performed for articles on therapeutic hypothermia in newborns with perinatal asphyxia in PubMed; the authors chose those considered most significant. There are two therapeutic hypothermia methods: selective head cooling and total body cooling. The target body temperature is 34.5 °C for selective head cooling and 33.5 °C for total body cooling. Temperatures lower than 32 °C are less neuroprotective, and temperatures below 30 °C are very dangerous, with severe complications. Therapeutic hypothermia must start within the first 6h after birth, as studies have shown that this represents the therapeutic window for the hypoxic-ischemic event. Therapy must be maintained for 72 h, with very strict control of the newborn's body temperature. It has been shown that therapeutic hypothermia is effective in reducing neurologic impairment, especially in full-term or near-term newborns with moderate hypoxic-ischemic encephalopathy. Therapeutic hypothermia is a neuroprotective technique indicated for newborn infants with perinatal asphyxia and hypoxic-ischemic encephalopathy. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  2. Proinflammatory Cytokines, Enolase and S-100 as Early Biochemical Indicators of Hypoxic-Ischemic Encephalopathy Following Perinatal Asphyxia in Newborns.

    PubMed

    Chaparro-Huerta, Verónica; Flores-Soto, Mario Eduardo; Merin Sigala, Mario Ernesto; Barrera de León, Juan Carlos; Lemus-Varela, María de Lourdes; Torres-Mendoza, Blanca Miriam de Guadalupe; Beas-Zárate, Carlos

    2017-02-01

    Estimation of the neurological prognosis of infants suffering from perinatal asphyxia and signs of hypoxic-ischemic encephalopathy is of great clinical importance; however, it remains difficult to satisfactorily assess these signs with current standard medical practices. Prognoses are typically based on data obtained from clinical examinations and neurological tests, such as electroencephalography (EEG) and neuroimaging, but their sensitivities and specificities are far from optimal, and they do not always reliably predict future neurological sequelae. In an attempt to improve prognostic estimates, neurological research envisaged various biochemical markers detectable in the umbilical cord blood of newborns (NB). Few studies examining these biochemical factors in the whole blood of newborns exist. Thus, the aim of this study was to determine the expression and concentrations of proinflammatory cytokines (TNF-α, IL-1β and IL-6) and specific CNS enzymes (S-100 and enolase) in infants with perinatal asphyxia. These data were compared between the affected infants and controls and were related to the degree of HIE to determine their utilities as biochemical markers for early diagnosis and prognosis. The levels of the proinflammatory cytokines and enzymes were measured by enzyme-linked immunosorbent assay (ELISA) and Reverse Transcription polymerase chain reaction (RT-PCR). The expression and serum levels of the proinflammatory cytokines, enolase and S-100 were significantly increased in the children with asphyxia compared with the controls. The role of cytokines after hypoxic-ischemic insult has been determined in studies of transgenic mice that support the use of these molecules as candidate biomarkers. Similarly, S-100 and enolase are considered promising candidates because these markers have been correlated with tissue damage in different experimental models. Copyright © 2016. Published by Elsevier B.V.

  3. Brain-Specific Superoxide Dismutase 2 Deficiency Causes Perinatal Death with Spongiform Encephalopathy in Mice.

    PubMed

    Izuo, Naotaka; Nojiri, Hidetoshi; Uchiyama, Satoshi; Noda, Yoshihiro; Kawakami, Satoru; Kojima, Shuji; Sasaki, Toru; Shirasawa, Takuji; Shimizu, Takahiko

    2015-01-01

    Oxidative stress is believed to greatly contribute to the pathogenesis of various diseases, including neurodegeneration. Impairment of mitochondrial energy production and increased mitochondrial oxidative damage are considered early pathological events that lead to neurodegeneration. Manganese superoxide dismutase (Mn-SOD, SOD2) is a mitochondrial antioxidant enzyme that converts toxic superoxide to hydrogen peroxide. To investigate the pathological role of mitochondrial oxidative stress in the central nervous system, we generated brain-specific SOD2-deficient mice (B-Sod2(-/-)) using nestin-Cre-loxp system. B-Sod2(-/-) showed perinatal death, along with severe growth retardation. Interestingly, these mice exhibited spongiform neurodegeneration in motor cortex, hippocampus, and brainstem, accompanied by gliosis. In addition, the mutant mice had markedly decreased mitochondrial complex II activity, but not complex I or IV, in the brain based on enzyme histochemistry. Furthermore, brain lipid peroxidation was significantly increased in the B-Sod2(-/-), without any compensatory alterations of the activities of other antioxidative enzymes, such as catalase or glutathione peroxidase. These results suggest that SOD2 protects the neural system from oxidative stress in the perinatal stage and is essential for infant survival and central neural function in mice.

  4. Pharmacokinetics and clinical efficacy of phenobarbital in asphyxiated newborns treated with hypothermia: a thermopharmacological approach.

    PubMed

    van den Broek, M P H; Groenendaal, F; Toet, M C; van Straaten, H L M; van Hasselt, J G C; Huitema, A D R; de Vries, L S; Egberts, A C G; Rademaker, C M A

    2012-10-01

    Therapeutic hypothermia can influence the pharmacokinetics and pharmacodynamics of drugs, the discipline which is called thermopharmacology. We studied the effect of therapeutic hypothermia on the pharmacokinetics of phenobarbital in asphyxiated neonates, and the clinical efficacy and the effect of phenobarbital on the continuous amplitude-integrated electroencephalography (aEEG) in a prospective study. Data were obtained from the prospective SHIVER study, performed in two of the ten Dutch level III neonatal intensive care units. Phenobarbital data were collected between 2008 and 2010. Newborns were eligible for inclusion if they had a gestational age of at least 36 weeks and presented with perinatal asphyxia and encephalopathy. According to protocol in both hospitals an intravenous (repeated) loading dose of phenobarbital 20 mg/kg divided in 1-2 doses was administered if seizures occurred or were suspected before or during the hypothermic phase. Phenobarbital plasma concentrations were measured in plasma using a fluorescence polarization immunoassay. aEEG was monitored continuously. A one-compartmental population pharmacokinetic/pharmacodynamic model was developed using a multi-level Markov transition model. No (clinically relevant) effect of moderate therapeutic hypothermia on phenobarbital pharmacokinetics could be identified. The observed responsiveness was 66%. While we still advise an initial loading dose of 20 mg/kg, clinicians should not be reluctant to administer an additional dose of 10-20 mg/kg. An additional dose should be given before switching to a second-line anticonvulsant drug. Based on our pharmacokinetic/pharmacodynamic model, administration of phenobarbital under hypothermia seems to reduce the transition rate from a continuous normal voltage (CNV) to discontinuous normal voltage aEEG background level in hypothermic asphyxiated newborns, which may be attributed to the additional neuroprotection of phenobarbital in infants with a CNV pattern.

  5. Angiogenesis Dysregulation in Term Asphyxiated Newborns Treated with Hypothermia

    PubMed Central

    Shaikh, Henna; Boudes, Elodie; Khoja, Zehra; Shevell, Michael; Wintermark, Pia

    2015-01-01

    Background Neonatal encephalopathy following birth asphyxia is a major predictor of long-term neurological impairment. Therapeutic hypothermia is currently the standard of care to prevent brain injury in asphyxiated newborns but is not protective in all cases. More robust and versatile treatment options are needed. Angiogenesis is a demonstrated therapeutic target in adult stroke. However, no systematic study examines the expression of angiogenesis-related markers following birth asphyxia in human newborns. Objective This study aimed to evaluate the expression of angiogenesis-related protein markers in asphyxiated newborns developing and not developing brain injury compared to healthy control newborns. Design/Methods Twelve asphyxiated newborns treated with hypothermia were prospectively enrolled; six developed eventual brain injury and six did not. Four healthy control newborns were also included. We used Rules-Based Medicine multi-analyte profiling and protein array technologies to study the plasma concentration of 49 angiogenesis-related proteins. Mean protein concentrations were compared between each group of newborns. Results Compared to healthy newborns, asphyxiated newborns not developing brain injury showed up-regulation of pro-angiogenic proteins, including fatty acid binding protein-4, glucose-6-phosphate isomerase, neuropilin-1, and receptor tyrosine-protein kinase erbB-3; this up-regulation was not evident in asphyxiated newborns eventually developing brain injury. Also, asphyxiated newborns developing brain injury showed a decreased expression of anti-angiogenic proteins, including insulin-growth factor binding proteins -1, -4, and -6, compared to healthy newborns. Conclusions These findings suggest that angiogenesis pathways are dysregulated following birth asphyxia and are putatively involved in brain injury pathology and recovery. PMID:25996847

  6. Association of hypoglycemia, hypocalcemia and hypomagnesemia in neonates with perinatal asphyxia.

    PubMed

    Saha, D; Ali, M A; Haque, M A; Ahmed, M S; Sutradhar, P K; Latif, T; Sarkar, D; Husain, F

    2015-04-01

    The clinical evidence of neurological menifestations associated with asphyxia is described as hypoxic ischaemic encephalopathy (HIE). A variety of metabolic problems are present in asphyxiated newborns including hypoglycemia, hypocalcemia, hypomagnesemia and others metabolic abnormalities. Some of these biochemical disturbances may trigger seizure or potentiate further brain damage. This cross sectional case-control study was done in Mymensingh Medical College Hospital, to identify the association of hypoglycemia, hypocalcemia, hypomagnesemia in neonates with perinatal asphyxia. Study period was six months. Sample size was 60. Among total sample 30 term asphyxiated newborns of <24 hours age were case and equal number term healthy newborns <24 hours age were control. The main clinical presentations were delayed cry after birth along with respiratory distress, convulsion and absence of cry in asphyxiated newborns. Major physical findings were cyanosis, convulsion and tachypnoea in asphyxiated group. The mean value of serum calcium level was significantly lower in asphyxiated newborns (7.37 ± 0.10mg/dl) than control value (8.04±0.09mg/dl). Hypocalcemia was found among 23.33% babies in case group. On the contrary, hypocalcemia was found in single baby among control group. The mean value of serum magnesium was significantly lower in asphyxiated newborns (1.83 ± 0.04mg/dl) than control value (1.96 ± 0.05mg/dl). Hypomagnesemia was found among 3(10%) newborns but none was found among control group. Hypoglycemia was found in 7(23.33%) cases though the mean value of blood glucose was higher in case group (5.72 ± 0.62mmol/l) than control group (4.87 ± 0.15mmol/l) difference was not statistically significant. Combined hypoglycemia, hypocalcemia and hypomagnesemia were found in 1(3.33%) case; combined hypoglycemia and hypocalcemia were found in 2(6.67%) cases; and combined hypocalcemia and hypomagnesemia were found in 1(3.33%) case. During the study period, 3(10.0%) cases

  7. Impact of perinatal systemic hypoxic-ischemic injury on the brain of male offspring rats: an improved model of neonatal hypoxic-ischemic encephalopathy in early preterm newborns.

    PubMed

    Huang, Yuejun; Lai, Huihong; Xu, Hongwu; Wu, Weizhao; Lai, Xiulan; Ho, Guyu; Chen, Yunbin; Ma, Lian

    2013-01-01

    In this study, we attempted to design a model using Sprague-Dawley rats to better reproduce perinatal systemic hypoxic-ischemic encephalopathy (HIE) in early preterm newborns. On day 21 of gestation, the uterus of pregnant rats were exposed and the blood supply to the fetuses of neonatal HIE groups were thoroughly abscised by hemostatic clamp for 5, 10 or 15 min. Thereafter, fetuses were moved from the uterus and manually stimulated to initiate breathing in an incubator at 37 °C for 1 hr in air. We showed that survival rates of offspring rats were decreased with longer hypoxic time. TUNEL staining showed that apoptotic cells were significant increased in the brains of offspring rats from the 10 min and 15 min HIE groups as compared to the offspring rats in the control group at postnatal day (PND) 1, but there was no statistical difference between the offspring rats in the 5 min HIE and control groups. The perinatal hypoxic treatment resulted in decreased neurons and increased cleaved caspase-3 protein levels in the offspring rats from all HIE groups at PND 1. Platform crossing times and the percentage of the time spent in the target quadrant of Morris Water Maze test were significantly reduced in the offspring rats of all HIE groups at PND 30, which were associated with decreased brain-derived neurotrophic factor levels and neuronal cells in the hippocampus of offspring rats at PND 35. These data demonstrated that perinatal ischemic injury led to the death of neuronal cells and long-lasting impairment of memory. This model reproduced hypoxic ischemic encephalopathy in early preterm newborns and may be appropriate for investigating therapeutic interventions.

  8. EEG, evoked potentials and pulsed Doppler in asphyxiated term infants.

    PubMed

    Julkunen, Mia K; Himanen, Sari-Leena; Eriksson, Kai; Janas, Martti; Luukkaala, Tiina; Tammela, Outi

    2014-09-01

    To evaluate electroencephalograms (EEG), evoked potentials (EPs) and Doppler findings in the cerebral arteries as predictors of a 1-year outcome in asphyxiated newborn infants. EEG and EPs (brain stem auditory (BAEP), somatosensory (SEP), visual (VEP) evoked potentials) were assessed in 30 asphyxiated and 30 healthy term infants during the first days (range 1-8). Cerebral blood flow velocities (CBFV) were measured from the cerebral arteries using pulsed Doppler at ∼24h of age. EEG, EPs, Doppler findings, symptoms of hypoxic ischemic encephalopathy (HIE) and their combination were evaluated in predicting a 1-year outcome. An abnormal EEG background predicted poor outcome in the asphyxia group with a sensitivity of 67% and 81% specificity, and an abnormal SEP with 75% and 79%, respectively. Combining increased systolic CBFV (mean+3SD) with abnormal EEG or SEP improved the specificity, but not the sensitivity. The predictive values of abnormal BAEP and VEP were poor. Normal EEG and SEP predicted good outcome in the asphyxia group with sensitivities from 79% to 81%. The combination of normal EEG, normal SEP and systolic CBFV<3SD predicted good outcome with a sensitivity of 74% and 100% specificity. Combining abnormal EEG or EPs findings with increased systolic CBFV did not improve prediction of a poor 1-year outcome of asphyxiated infants. Normal EEG and normal SEP combined with systolic CBFV<3SD at about 24 h can be valuable in the prediction of normal 1-year outcome. Combining systolic CBFV at 24 h with EEG and SEP examinations can be of use in the prediction of normal 1-year outcome among asphyxiated infants. Copyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  9. Therapeutic hypothermia increases the risk of cardiac arrhythmia for perinatal hypoxic ischaemic encephalopathy: A meta-analysis

    PubMed Central

    2017-01-01

    Objective To determine whether therapeutic hypothermia after hypoxic ischaemic encephalopathy (HIE) in neonates increases the risk of cardiac arrhythmia during intervention. Design A meta-analysis was conducted using a fixed-effect model. Risk ratios, risk differences, and 95% confidence intervals, were measured. Data sources Studies identified from the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, Google Scholar, previous reviews, and abstracts from onset to August, 2016. Review methods Reports that compared therapeutic hypothermia with normal care for neonates with HIE and that included data on safety or cardiac arrhythmia, which is of interest to patients and clinicians, were selected. Results We found seven trials, encompassing 1322 infants that included information on safety or cardiac arrhythmia during intervention. Therapeutic hypothermia considerably increased the combined rate of cardiac arrhythmia in the seven trials (risk ratio 2.42, 95% confidence interval 1.23 to 4.76. p = 0.01; risk difference 0.02, 95% CI 0.01 to 0.04) during intervention. Conclusions In infants with hypoxic ischaemic encephalopathy, therapeutic hypothermia is associated with a consistent increase in cardiac arrhythmia during intervention. PMID:28273115

  10. [Laryngomalacia in a follow-up of a child development cohort for antecedents of perinatal encephalopathy. Implications for nosologic conceptualization].

    PubMed

    Mandujano-Valdés, Mario Antonio; Sánchez-Pérez, María del Carmen

    2004-01-01

    We conducted a retrospective analysis of 10 cases of congenital laryngeal stridor. Reports of laryngeal endoscopy and diagnosis define laryngomalacia as laryngeal flaccidity and stridor. Some authors postulate that in addition to immaturity of cartilage, there exist the possibility of laryngeal uncoordination and dyskinesia. They support this idea in cases of late presentation, neurological damage, and atypical cases related with functional state or anesthesia. Laryngeal endoscopies were carried out in 10 cases included in a cohort of subjects from a longitudinal follow-up diagnosed with neurologica damage of perinatal origin. One case was diagnosed with postoperative unilateral paralysis of vocal chord and another identified vascular ring. The eight remaining cases fulfilled laryngomalacia criteria of diagnosis, but because of their characteristics origin is not an anatomic alteration but a functional hypotonia. The need to carry out an integral study to describe co-morbidity is emphasized.

  11. Troponin-T as a biomarker in neonates with perinatal asphyxia.

    PubMed

    Abiramalatha, T; Kumar, M; Chandran, S; Sudhakar, Y; Thenmozhi, M; Thomas, N

    2017-08-23

    Troponin-T is a commonly used cardiac biomarker, which could be useful in perinatal asphyxia. We aimed to analyze troponin-T concentrations in asphyxiated neonates and to correlate the concentrations with clinical outcomes. Data were collected from electronic medical records of neonates diagnosed with perinatal asphyxia over a period of four years. There were 63 neonates with moderate to severe encephalopathy, in whom serial troponin-T concentrations had been done on days 1, 3, and 7. 53 (84%) asphyxiated infants had troponin-T concentration >100 pg/ml at 2-4 h of life.The difference in troponin-T concentrations between moderate and severe encephalopathy was not statistically significant (173 vs. 263 pg/ml, p value 0.40). The difference in the concentrations at 72 hours between cooled and non-cooled neonates was not significant (48.5 vs. 62.5 pg/ml, p value 0.22). Troponin-T concentration was significantly higher in babies with hypotensive shock and hepatic injury, but not acute kidney injury. There was no significant correlation between troponin-T and the extent of resuscitation needed.Troponin-T concentration on day 1 of life was significantly higher in babies who died than who survived (407 vs. 168 pg/ml, p value 0.03). ROC curve for troponin-T to predict mortality had an area under the curve (AUC) of 0.803; the best cut-off value (190 pg/ml) had 82% sensitivity and 80% specificity. There was no significant difference in troponin-T concentrations between cooled and non-cooled neonates. Troponin-T concentration had a good predictive accuracy for mortality before discharge.

  12. Hashimoto's encephalopathy.

    PubMed

    Chen, H C; Marsharani, U

    2000-05-01

    Hashimoto's encephalopathy is a subacute condition associated with autoimmune thyroiditis. Its presentation varies from focal neurologic deficits to global confusion. Unlike encephalopathy associated with hypothyroidism, Hashimoto's encephalopathy responds to steroid therapy and not thyroxine replacement.

  13. The renal disease of thoracic asphyxiant dystrophy.

    PubMed

    Gruskin, A B; Baluarte, H J; Cote, M L; Elfenbein, I B

    1974-01-01

    In those children with thoracic asphyxiant dystrophy, a genetically determined disorder, who survive infancy, the development of renal disease may be life-threatening. This report will present data obtained in six patients from three families which deals with the renal abnormalities in thoracic asphyxiant dystrophy. Both functional and anatomic abnormalities are described. Abnormalities in solute transport in the proximal tubule may be the earliest sign of renal dysfunction in this syndrome. Early glomerular changes may be more important than previously recognized. Finally, the various phenotypic expressions of this disorder are considered.

  14. Suicide by asphyxiation due to helium inhalation.

    PubMed

    Howard, Matthew O; Hall, Martin T; Edwards, Jeffrey D; Vaughn, Michael G; Perron, Brian E; Winecker, Ruth E

    2011-03-01

    Suicide by asphyxiation using helium is the most widely-promoted method of "self-deliverance" by right-to-die advocates. However, little is known about persons committing such suicides or the circumstances and manner in which they are completed. Prior reports of suicides by asphyxiation involving helium were reviewed and deaths determined by the North Carolina Office of the Chief Medical Examiner to be helium-associated asphyxial suicides occurring between January 1, 2000 and December 31, 2008 were included in a new case series examined in this article. The 10 asphyxial suicides involving helium identified in North Carolina tended to occur almost exclusively in non-Hispanic, white men who were relatively young (M age = 41.1 T 11.6). In 6 of 10 cases, decedents suffered from significant psychiatric dysfunction; in 3 of these 6 cases, psychiatric disorders were present comorbidly with substance abuse. In none of these cases were decedents suffering from terminal illness. Most persons committing suicide with helium were free of terminal illness but suffered from psychiatric and/or substance use disorders.

  15. Cooling for newborns with hypoxic ischaemic encephalopathy.

    PubMed

    Jacobs, Susan E; Berg, Marie; Hunt, Rod; Tarnow-Mordi, William O; Inder, Terrie E; Davis, Peter G

    2013-01-31

    Newborn animal studies and pilot studies in humans suggest that mild hypothermia following peripartum hypoxia-ischaemia in newborn infants may reduce neurological sequelae without adverse effects. To determine the effect of therapeutic hypothermia in encephalopathic asphyxiated newborn infants on mortality, long-term neurodevelopmental disability and clinically important side effects. We used the standard search strategy of the Cochrane Neonatal Review Group as outlined in The Cochrane Library (Issue 2, 2007). Randomised controlled trials evaluating therapeutic hypothermia in term and late preterm newborns with hypoxic ischaemic encephalopathy were identified by searching the Oxford Database of Perinatal Trials, the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, 2007, Issue 2), MEDLINE (1966 to June 2007), previous reviews including cross-references, abstracts, conferences, symposia proceedings, expert informants and journal handsearching. We updated this search in May 2012. We included randomised controlled trials comparing the use of therapeutic hypothermia with standard care in encephalopathic term or late preterm infants with evidence of peripartum asphyxia and without recognisable major congenital anomalies. The primary outcome measure was death or long-term major neurodevelopmental disability. Other outcomes included adverse effects of cooling and 'early' indicators of neurodevelopmental outcome. Four review authors independently selected, assessed the quality of and extracted data from the included studies. Study authors were contacted for further information. Meta-analyses were performed using risk ratios (RR) and risk differences (RD) for dichotomous data, and weighted mean difference for continuous data with 95% confidence intervals (CI). We included 11 randomised controlled trials in this updated review, comprising 1505 term and late preterm infants with moderate/severe encephalopathy and evidence of intrapartum asphyxia

  16. Autoerotic Asphyxiation: A Challenge to Death Educators and Counselors.

    ERIC Educational Resources Information Center

    Garos, Sheila

    1994-01-01

    Notes that approximately 250 to 1,000 deaths in United States annually are result of autoerotic asphyxiation, hangings that were intended to enhance sexual excitement. Reviews scattered literature on autoerotic asphyxiation and includes observations by two psychiatrists and medical examiner. Notes that much remains to be learned about this…

  17. Autoerotic asphyxiation: secret pleasure--lethal outcome?

    PubMed

    Cowell, Daniel D

    2009-11-01

    Voluntary asphyxiation among children, preteens, and adolescents by hanging or other means of inducing hypoxia/anoxia to enhance sexual excitement is not uncommon and can lead to unintended death. This study addresses autoerotic asphyxiation (AEA) with the intent of increasing pediatricians' knowledge of the syndrome and awareness of its typical onset among young patients. AEA is characteristically a clandestine and elusive practice. Provided with relevant information, pediatricians can identify the syndrome, demonstrate a willingness to discuss concerns about it, ameliorate distress, and possibly prevent a tragedy. A retrospective study was undertaken of published cases both fatal and nonfatal and included personal communications, referenced citations, clinical experience, and theoretical formulations as to causation. Characteristic AEA manifestations, prevalence, age range, methods of inducing hypoxia/anoxia, and gender weighting are presented. All sources were used as a basis for additional considerations of etiology and possibilities for intervention. AEA can be conceptualized as a personalized, ritualized, and symbolic biopsychosocial drama. It seems to be a reenactment of intense emotional feeling-states involving an identification and sadomasochistic relationship with a female figure. Inept AEA practitioners can miscalculate the peril of the situation that they have contrived and for numerous reasons lose their gamble with death. Pediatricians should be alert to the earliest manifestations of AEA. Awareness of choking games among the young and, of those, a subset who eventually progress to potentially fatal AEA is strongly encouraged among all primary care professionals who may be able to interrupt the behavior.

  18. Toxic Encephalopathy

    PubMed Central

    Kim, Jae Woo

    2012-01-01

    This article schematically reviews the clinical features, diagnostic approaches to, and toxicological implications of toxic encephalopathy. The review will focus on the most significant occupational causes of toxic encephalopathy. Chronic toxic encephalopathy, cerebellar syndrome, parkinsonism, and vascular encephalopathy are commonly encountered clinical syndromes of toxic encephalopathy. Few neurotoxins cause patients to present with pathognomonic neurological syndromes. The symptoms and signs of toxic encephalopathy may be mimicked by many psychiatric, metabolic, inflammatory, neoplastic, and degenerative diseases of the nervous system. Thus, the importance of good history-taking that considers exposure and a comprehensive neurological examination cannot be overemphasized in the diagnosis of toxic encephalopathy. Neuropsychological testing and neuroimaging typically play ancillary roles. The recognition of toxic encephalopathy is important because the correct diagnosis of occupational disease can prevent others (e.g., workers at the same worksite) from further harm by reducing their exposure to the toxin, and also often provides some indication of prognosis. Physicians must therefore be aware of the typical signs and symptoms of toxic encephalopathy, and close collaborations between neurologists and occupational physicians are needed to determine whether neurological disorders are related to occupational neurotoxin exposure. PMID:23251840

  19. The relationship between serial sexual murder and autoerotic asphyxiation.

    PubMed

    Myers, Wade C; Bukhanovskiy, Alexandr; Justen, Elle; Morton, Robert J; Tilley, John; Adams, Kenneth; Vandagriff, Virgil L; Hazelwood, Robert R

    2008-04-07

    This case series documents and examines the association between autoerotic asphyxiation, sadomasochism, and serial sexual murderers. Autoerotic asphyxiation, along with other paraphilias found in this population, is reviewed. Five cases of serial sexual killers who engaged in autoerotic asphyxiation were identified worldwide: four from the United States and one from Russia. Case reports for each are provided. All (100%) were found to have sexual sadism in addition to autoerotic asphyxiation. Furthermore, two (40%) had bondage fetishism, and two (40%) had transvestic fetishism, consistent with these paraphilias co-occurring in those with autoerotic asphyxiation. Overall the group averaged 4.0 lifetime paraphilias. Some possible relationships were observed between the offenders' paraphilic orientation and their modus operandi, e.g., all of these serial killers strangled victims-suggesting an association between their sadistic and asphyxiative paraphilic interests. The overlap of seemingly polar opposite paraphilias in this sample--sexual sadism and autoerotic asphyxiation--is explored from a historical and clinical perspective. Multiple commonalities shared between these five offenders and serial sexual murderers in general are addressed. A primary limitation of this study is its small sample size and empirical basis; the results may not be generalizable beyond the sample. The findings from this study support the supposition that crime scene behaviors often reflect paraphilic disturbances in those who commit serial sexual homicides.

  20. Parent Experience of Neonatal Encephalopathy.

    PubMed

    Lemmon, Monica E; Donohue, Pamela K; Parkinson, Charlamaine; Northington, Frances J; Boss, Renee D

    2017-03-01

    We aimed to characterize the parent experience of caring for an infant with neonatal encephalopathy. In this mixed-methods study, we performed semistructured interviews with parents whose infants were enrolled in an existing longitudinal cohort study of therapeutic hypothermia between 2011 and 2014. Thematic saturation was achieved after 20 interviews. Parent experience of caring for a child with neonatal encephalopathy was characterized by 3 principal themes. Theme 1: Many families described cumulative loss and grief throughout the perinatal crisis, critical neonatal course, and subsequent missed developmental milestones. Theme 2: Families experienced entangled infant and broader family interests. Theme 3: Parents evolved into and found meaning in their role as an advocate. These data offer insight into the lived experience of parenting an infant with neonatal encephalopathy. Primary data from parents can serve as a useful framework to guide the development and interpretation of parent-centered outcomes.

  1. Ischemic injury suppresses hypoxia-induced electrographic seizures and the background EEG in a rat model of perinatal hypoxic-ischemic encephalopathy

    PubMed Central

    Zayachkivsky, A.; Lehmkuhle, M. J.; Ekstrand, J. J.

    2015-01-01

    The relationship among neonatal seizures, abnormalities of the electroencephalogram (EEG), brain injury, and long-term neurological outcome (e.g., epilepsy) remains controversial. The effects of hypoxia alone (Ha) and hypoxia-ischemia (HI) were studied in neonatal rats at postnatal day 7; both models generate EEG seizures during the 2-h hypoxia treatment, but only HI causes an infarct with severe neuronal degeneration. Single-channel, differential recordings of acute EEG seizures and background suppression were recorded with a novel miniature telemetry device during the hypoxia treatment and analyzed quantitatively. The waveforms of electrographic seizures (and their behavioral correlates) appeared virtually identical in both models and were identified as discrete events with high power in the traditional delta (0.1–4 Hz) and/or alpha (8–12 Hz) bands. Although the EEG patterns during seizures were similar in Ha- and HI-treated animals at the beginning of the hypoxic insult, Ha caused a more severe electrographic seizure profile than HI near the end. Analyses of power spectral density and seizure frequency profiles indicated that the electrographic seizures progressively increased during the 2-h Ha treatment, while HI led to a progressive decrease in the seizures with significant suppression of the EEG background. These data show that 1) the hypoxia component of these two models drives the seizures; 2) the seizures during Ha are substantially more robust than those during HI, possibly because ongoing neuronal damage blunts the electrographic activity; and 3) a progressive decrease in background EEG, rather than the presence of electrographic seizures, indicates neuronal degeneration during perinatal HI. PMID:26354320

  2. Epileptic Encephalopathies.

    PubMed

    Germain, Blair; Maria, Bernard L

    2017-01-01

    Epileptic encephalopathies encompass a heterogeneous group of epilepsy syndromes that manifest with cognitive, behavioral, and neurologic deficits, seizures that are often intractable and multiform, aggressive electroencephalographic paroxysmal activity, and sometimes early death. As more is learned about the etiologies and manifestations of epileptic encephalopathies, progress has been made toward better treatment options. However, there is still a great need for further randomized controlled trials and research to help create clinically effective therapies. The 2015 Neurobiology of Disease in Children symposium, held in conjunction with the 44th annual meeting of the Child Neurology Society, aimed to (1) describe the clinical concerns involving diagnosis and treatment, (2) review the current status of understanding in the pathogenesis of epileptic encephalopathy, (3) discuss clinical management and therapies for epileptic encephalopathy, and (4) define future directions of research. This article summarizes the presentations and includes an edited transcript of question-and-answer sessions.

  3. [Wernicke encephalopathy].

    PubMed

    Djelantik, M; Bloemkolk, D; Tijdink, J

    2015-01-01

    Wernicke encephalopathy is an acute neuropsychiatric disease with heterogeneous symptoms, including changes in mental status, ataxia and ocular abnormalities; if left untreated, these symptoms can lead to morbidity and even to mortality. The treatment is thiamine suppletion. Because of the heterogeneity of the symptoms and the high risk of morbidity and mortality if the symptoms are not treated, it is vitally important that on observing a patient's early symptoms the clinician immediately suspects that the symptoms could point to Wernicke encephalopathy.

  4. Necropsy findings in neonatal asphyxiating thoracic dystrophy.

    PubMed Central

    Turkel, S B; Diehl, E J; Richmond, J A

    1985-01-01

    Asphyxiating thoracic dystrophy is an autosomal recessive disorder characterised by an abnormally small thorax, variable shortening of the extremities, and pelvic anomalies. Renal and pancreatic symptoms are found in longer survivors, although most cases die in infancy of respiratory failure. Seven neonatal cases were studied at necropsy. These cases ranged in gestational age from 32 to 40 weeks. One was stillborn and the other six survived from 1 hour to 10 days. Two were sibs born to consanguineous parents. Dwarfing was not pronounced and the extremities were shortened in only one infant who also had polydactyly. All seven showed visceral changes in addition to abnormalities of bone. Endochondral ossification was irregular in sections of femur, vertebra, and rib. Pulmonary hypoplasia was associated with the small thorax typical of this disorder. Periportal fibrosis and bile duct proliferation were seen in sections of liver, and in one case cirrhosis was found. Pancreatic fibrosis was variable. These necropsy findings correlate with later clinical manifestations of the disease and emphasise the multisystem nature of this disorder. Images PMID:3989824

  5. The “Neurovascular Unit approach” to Evaluate Mechanisms of Dysfunctional Autoregulation in Asphyxiated Newborns in the era of Hypothermia Therapy

    PubMed Central

    Chalak, Lina F.; Tarumi, Takashi; Zhang, Rong

    2014-01-01

    Despite improvements in obstetrical and neonatal care, and introduction of hypothermia as a neuroprotective therapy, perinatal brain injury remains a frequent cause of cerebral palsy, mental retardation and epilepsy. The recognition of dysfunction of cerebral autoregulation is essential for a real time measure of efficacy to identify those who are at highest risk for brain injury. This article will focus on the “neurovascular unit” approach to the care of asphyxiated neonates to review 1) potential mechanisms of dysfunctional cerebral blood flow (CBF) regulation, 2) optimal monitoring methodology such as NIRS (near infrared spectroscopy), and TCD (transcutaneous Doppler), and 3) clinical implications of monitoring in the neonatal intensive care setting in asphyxiated newborns undergoing hypothermia and rewarming. Critical knowledge of the functional regulation of the neurovascular unit may lead to improved ability to predict outcomes in real time during hypothermia, as well as differentiate nonresponders who might benefit from additional therapies. PMID:25062804

  6. Antecedents of Neonatal Encephalopathy in the Vermont Oxford Network Encephalopathy Registry

    PubMed Central

    Bingham, Peter; Edwards, Erika M.; Horbar, Jeffrey D.; Kenny, Michael J.; Inder, Terrie; Pfister, Robert H.; Raju, Tonse; Soll, Roger F.

    2012-01-01

    BACKGROUND: Neonatal encephalopathy (NE) is a major predictor of death and long-term neurologic disability, but there are few studies of antecedents of NE. OBJECTIVES: To identify antecedents in a large registry of infants who had NE. METHODS: This was a maternal and infant record review of 4165 singleton neonates, gestational age of ≥36 weeks, meeting criteria for inclusion in the Vermont Oxford Network Neonatal Encephalopathy Registry. RESULTS: Clinically recognized seizures were the most prevalent condition (60%); 49% had a 5-minute Apgar score of ≤3 and 18% had a reduced level of consciousness. An abnormal maternal or fetal condition predated labor in 46%; maternal hypertension (16%) or small for gestational age (16%) were the most frequent risk factors. In 8%, birth defects were identified. The most prevalent birth complication was elevated maternal temperature in labor of ≥37.5°C in 27% of mothers with documented temperatures compared with 2% to 3.2% in controls in population-based studies. Clinical chorioamnionitis, prolonged membrane rupture, and maternal hypothyroidism exceeded rates in published controls. Acute asphyxial indicators were reported in 15% (in 35% if fetal bradycardia included) and inflammatory indicators in 24%. Almost one-half had neither asphyxial nor inflammatory indicators. Although most infants with NE were observably ill since the first minutes of life, only 54% of placentas were submitted for examination. CONCLUSIONS: Clinically recognized asphyxial birth events, indicators of intrauterine exposure to inflammation, fetal growth restriction, and birth defects were each observed in term infants with NE, but much of NE in this large registry remained unexplained. PMID:23071210

  7. Antecedents of neonatal encephalopathy in the Vermont Oxford Network Encephalopathy Registry.

    PubMed

    Nelson, Karin B; Bingham, Peter; Edwards, Erika M; Horbar, Jeffrey D; Kenny, Michael J; Inder, Terrie; Pfister, Robert H; Raju, Tonse; Soll, Roger F

    2012-11-01

    Neonatal encephalopathy (NE) is a major predictor of death and long-term neurologic disability, but there are few studies of antecedents of NE. To identify antecedents in a large registry of infants who had NE. This was a maternal and infant record review of 4165 singleton neonates, gestational age of ≥ 36 weeks, meeting criteria for inclusion in the Vermont Oxford Network Neonatal Encephalopathy Registry. Clinically recognized seizures were the most prevalent condition (60%); 49% had a 5-minute Apgar score of ≤ 3 and 18% had a reduced level of consciousness. An abnormal maternal or fetal condition predated labor in 46%; maternal hypertension (16%) or small for gestational age (16%) were the most frequent risk factors. In 8%, birth defects were identified. The most prevalent birth complication was elevated maternal temperature in labor of ≥ 37.5 °C in 27% of mothers with documented temperatures compared with 2% to 3.2% in controls in population-based studies. Clinical chorioamnionitis, prolonged membrane rupture, and maternal hypothyroidism exceeded rates in published controls. Acute asphyxial indicators were reported in 15% (in 35% if fetal bradycardia included) and inflammatory indicators in 24%. Almost one-half had neither asphyxial nor inflammatory indicators. Although most infants with NE were observably ill since the first minutes of life, only 54% of placentas were submitted for examination. Clinically recognized asphyxial birth events, indicators of intrauterine exposure to inflammation, fetal growth restriction, and birth defects were each observed in term infants with NE, but much of NE in this large registry remained unexplained.

  8. Lethal Injection for Execution: Chemical Asphyxiation?

    PubMed Central

    Zimmers, Teresa A; Sheldon, Jonathan; Lubarsky, David A; López-Muñoz, Francisco; Waterman, Linda; Weisman, Richard; Koniaris, Leonidas G

    2007-01-01

    Background Lethal injection for execution was conceived as a comparatively humane alternative to electrocution or cyanide gas. The current protocols are based on one improvised by a medical examiner and an anesthesiologist in Oklahoma and are practiced on an ad hoc basis at the discretion of prison personnel. Each drug used, the ultrashort-acting barbiturate thiopental, the neuromuscular blocker pancuronium bromide, and the electrolyte potassium chloride, was expected to be lethal alone, while the combination was intended to produce anesthesia then death due to respiratory and cardiac arrest. We sought to determine whether the current drug regimen results in death in the manner intended. Methods and Findings We analyzed data from two US states that release information on executions, North Carolina and California, as well as the published clinical, laboratory, and veterinary animal experience. Execution outcomes from North Carolina and California together with interspecies dosage scaling of thiopental effects suggest that in the current practice of lethal injection, thiopental might not be fatal and might be insufficient to induce surgical anesthesia for the duration of the execution. Furthermore, evidence from North Carolina, California, and Virginia indicates that potassium chloride in lethal injection does not reliably induce cardiac arrest. Conclusions We were able to analyze only a limited number of executions. However, our findings suggest that current lethal injection protocols may not reliably effect death through the mechanisms intended, indicating a failure of design and implementation. If thiopental and potassium chloride fail to cause anesthesia and cardiac arrest, potentially aware inmates could die through pancuronium-induced asphyxiation. Thus the conventional view of lethal injection leading to an invariably peaceful and painless death is questionable. PMID:17455994

  9. Molecular hydrogen affords neuroprotection in a translational piglet model of hypoxic-ischemic encephalopathy.

    PubMed

    Nemeth, J; Toth-Szuki, V; Varga, V; Kovacs, V; Remzso, G; Domoki, F

    2016-10-01

    Hypoxic-ischemic encephalopathy (HIE) is the major consequence of perinatal asphyxia (PA) in term neonates. Although the newborn piglet is an accepted large animal PA/HIE model, there is no consensus on PA-induction methodology to produce clinically relevant HIE. We aimed to create and to characterize a novel PA model faithfully reproducing all features of asphyxiation including severe hypercapnia resulting in HIE, and to test whether H2 is neuroprotective in this model. Piglets were anaesthetised, artificially ventilated, and intensively monitored (electroencephalography, core temperature, O2 saturation, arterial blood pressure and blood gases). Asphyxia (20 min) was induced by ventilation with a hypoxic-hypercapnic (6%O2 - 20%CO2) gas mixture. Asphyxia-induced changes in the cortical microcirculation were assessed with laser-speckle contrast imaging and analysis. Asphyxia was followed by reventilation with air or air containing hydrogen (2.1%H2, 4 hours). After 24 hours survival, the brains were harvested for neuropathology. Our PA model was characterized by the development of severe hypoxia (pO2 = 27 ± 4 mmHg), and combined acidosis (pH = 6.76 ± 0.04; pCO2 = 114 ± 11 mmHg; lactate = 12.12 ± 0.83 mmol/L), however, cortical ischemia did not develop during the stress. Severely depressed electroencephalography (EEG), and marked neuronal injury indicated the development of HIE. H2 was neuroprotective shown both by the enhanced recovery of EEG and by the significant preservation of neurons in the cerebral cortex, hippocampus, basal ganglia, and the thalamus. H2 appeared to reduce oxidative stress shown by attenuation of 8-hydroxy-2'-deoxyguanosine immunostaining. In summary, this new PA piglet model is able to induce moderate/severe HIE, and the efficacy of hydrogen post-treatment to preserve neuronal activity/function in this PA/HIE model suggests the feasibility of this safe and inexpensive approach in the treatment of asphyxiated babies.

  10. Pancreatic encephalopathy

    PubMed Central

    Sharf, B.; Bental, E.

    1971-01-01

    A 58 year old woman presenting with abdominal distress and a neuropsychiatric disturbance with evidence of focal neurological deficit is described. A diagnosis of pancreatic encephalopathy was made, and the patient was treated accordingly with pancreatic anti-enzymes. A survey of the literature is presented. Images PMID:5315218

  11. Deferoxamine prevents cerebral glutathione and vitamin E depletions in asphyxiated neonatal rats: role of body temperature.

    PubMed

    Kletkiewicz, Hanna; Nowakowska, Anna; Siejka, Agnieszka; Mila-Kierzenkowska, Celestyna; Woźniak, Alina; Caputa, Michał; Rogalska, Justyna

    2016-01-01

    Hypoxic-ischaemic brain injury involves increased oxidative stress. In asphyxiated newborns iron deposited in the brain catalyses formation of reactive oxygen species. Glutathione (GSH) and vitamin E are key factors protecting cells against such agents. Our previous investigation has demonstrated that newborn rats, showing physiological low body temperature as well as their hyperthermic counterparts injected with deferoxamine (DF) are protected against iron-mediated, delayed neurotoxicity of perinatal asphyxia. Therefore, we decided to study the effects of body temperature and DF on the antioxidant status of the brain in rats exposed neonatally to critical anoxia. Two-day-old newborn rats were exposed to anoxia in 100% nitrogen atmosphere for 10 min. Rectal temperature was kept at 33 °C (physiological to rat neonates), or elevated to the level typical of healthy adult rats (37 °C), or of febrile adult rats (39 °C). Half of the rats exposed to anoxia under extremely hyperthermic conditions (39 °C) were injected with DF. Cerebral concentrations of malondialdehyde (MDA, lipid peroxidation marker) and the levels of GSH and vitamin E were determined post-mortem, (1) immediately after anoxia, (2) 3 days, (3) 7 days, and (4) 2 weeks after anoxia. There were no post-anoxic changes in MDA, GSH and vitamin E concentrations in newborn rats kept at body temperature of 33 °C. In contrast, perinatal anoxia at elevated body temperatures intensified oxidative stress and depleted the antioxidant pool in a temperature-dependent manner. Both the depletion of antioxidants and lipid peroxidation were prevented by post-anoxic DF injection. The data support the idea that hyperthermia may extend perinatal anoxia-induced brain lesions.

  12. Cephalopod Susceptibility to Asphyxiation via Ocean Incalescence, Deoxygenation, and Acidification.

    PubMed

    Seibel, Brad A

    2016-11-01

    Squids are powerful swimmers with high metabolic rates despite constrained oxygen uptake and transport. They have evolved novel physiological strategies for survival in extreme environments that provide insight into their susceptibility to asphyxiation under anthropogenic ocean incalescence (warming), deoxygenation, and acidification. Plasticity of ecological and physiological traits, in conjunction with vertical and latitudinal mobility, may explain their evolutionary persistence and ensure their future survival.

  13. [Hashimoto encephalopathy].

    PubMed

    Pocsay, Gábor; Gazdag, Andrea; Engelhardt, József; Szaniszló, István; Szolnoki, Zoltán; Forczek, Gabriella; Mikló, László

    2013-08-18

    The authors present a case report and review the literature on Hashimoto encephalopathy. The onset of the disease may be marked by focal and then progressively generalized seizures or other neurological symptoms, but a cognitive decline or various psychiatric symptoms may also emerge. High levels of anti-thyroid peroxidase antibodies and/or anti-thyroglobulin antibodies are present in the serum. Corticosteroid treatment usually results in an improvement of symptoms. The syndrome is frequently overlooked and, therefore, the authors strongly recommend testing serum thyroid autoantibodies in cases with encephalopathy of unknown origin independently on the presence of thyroid disease in the patient or family history. The importance of long-term immunosuppressive treatment should also be stressed.

  14. The diagnostic value of both troponin T and creatinine kinase isoenzyme (CK-MB) in detecting combined renal and myocardial injuries in asphyxiated infants.

    PubMed

    Sadoh, Wilson E; Eregie, Charles O; Nwaneri, Damian U; Sadoh, Ayebo E

    2014-01-01

    Troponin T (cTnT) and Creatinine Kinase Isoenzyme (CK-MB) are both markers of myocardial injuries. However, CK-MB is also elevated in acute kidney injury. The diagnostic value of both cTnT and cardiac CK-MB in combined myocardial and acute kidney injuries (AKI) in asphyxiated neonates was evaluated. 40 asphyxiated infants and 40 non-asphyxiated controls were consecutively recruited. Serum levels of cTnT, CK-MB and creatinine were measured. Myocardial injury and AKI were defined as cTnT >95th percentile of the control and serum creatinine >1.0 mg/dl respectively. Of the 40 subjects, 9 (22.50%), 8 (20.00%) and 4 (10.00%) had myocardial injury, AKI and combined AKI and myocardial injuries respectively. The mean cTnT and CK-MB values were highest in infants with combined AKI and myocardial injuries. The Mean cTnT in infants with AKI, myocardial injury and combined AKI and myocardial injuries were 0.010±0.0007 ng/ml, 0.067±0.040 ng/ml and 0.084±0.067 ng/ml respectively, p = 0.006. The mean CK-MB in infants with AKI, myocardial injury and combined AKI and myocardial injuries were 2.78±0.22 ng/ml, 1.28±0.11 ng/ml and 4.58±0.52 ng/ml respectively, p = <0.0001. In severe perinatal asphyxia, renal and myocardial injuries could co-exist. Elevated cTnT signifies the presence of myocardial injury. Elevated CK-MB indicates either myocardial injury, AKI or both. Therefore renal injury should be excluded in asphyxiated infants with elevated CK-MB.

  15. Hypoxic Ischemic Encephalopathy in the Term Infant

    PubMed Central

    Fatemi, Ali; Wilson, Mary Ann; Johnston, Michael V.

    2010-01-01

    Synopsis Hypoxia-ischemia in the perinatal period is an important cause of cerebral palsy and associated disabilities in children. There has been significant research progress in hypoxic-ischemic encephalopathy over the last two decades and many new molecular mechanisms have been identified. Despite all these advances, therapeutic interventions are still limited. In this review paper, we discuss a number of molecular pathways involved in hypoxia-ischemia, and potential therapeutic targets. PMID:19944838

  16. Perinatal neuroprotection

    PubMed Central

    Jelin, Angie C.; Thiet, Mari-Paule

    2014-01-01

    Fetal or neonatal brain injury can result in lifelong neurologic disability. The most significant risk factor for perinatal brain injury is prematurity; however, in absolute numbers, full-term infants represent the majority of affected children. Research on strategies to prevent or mitigate the impact of perinatal brain injury (“perinatal neuroprotection”) has established the mitigating roles of magnesium sulfate administration for preterm infants and therapeutic hypothermia for term infants with suspected perinatal brain injury. Banked umbilical cord blood, erythropoietin, and a number of other agents that may improve neuronal repair show promise for improving outcomes following perinatal brain injury in animal models. Other preventative strategies include delayed umbilical cord clamping in preterm infants and progesterone in women with prior preterm birth or short cervix and avoidance of infections. Despite these advances, we have not successfully decreased the rate of preterm birth, nor are we able to predict term infants at risk of hypoxic brain injury in order to intervene prior to the hypoxic event. Further, we lack the ability to modulate the sequelae of neuronal cell insults or the ability to repair brain injury after it has been sustained. As a consequence, despite exciting advances in the field of perinatal neuroprotection, perinatal brain injury still impacts thousands of newborns each year with significant long-term morbidity and mortality. PMID:24592318

  17. Childhood asphyxiation by food. A national analysis and overview.

    PubMed

    Harris, C S; Baker, S P; Smith, G A; Harris, R M

    1984-05-04

    Data on all identified food-related asphyxiations of infants and children aged 0 to 9 years in 41 states from 1979 to 1981 were analyzed by type of food and age of child. Nationally, one death occurred approximately every five days. More than 90% occurred in infants and children younger than 5 years and 65% in infants younger than 2 years. Round foods were most often mentioned of the 103 foods specifically identified on death certificates. Most frequently cited were hot dog products (17 cases, 17%), candy, ten; nuts, nine; and grapes, eight. Hot dogs caused deaths from infancy through 3 years (more than two thirds of all deaths from meat products) and seven of ten deaths in 3-year-olds. Characteristics of foods, children, and environment can be related to three phases of food asphyxiation: penetration, occlusion, and expulsion. Preventive measures include product modification, warning labels, and dissemination of information on high-risk foods.

  18. Communication Challenges in Neonatal Encephalopathy.

    PubMed

    Lemmon, Monica E; Donohue, Pamela K; Parkinson, Charlamaine; Northington, Frances J; Boss, Renee D

    2016-09-01

    Families must process complex information related to neonatal encephalopathy and therapeutic hypothermia. In this mixed methods study, semi-structured interviews were performed with parents whose infants were enrolled in an existing longitudinal cohort study of therapeutic hypothermia between 2011 and 2014. Thematic saturation was achieved after 20 interviews. Parental experience of communicating with clinicians was characterized by 3 principle themes. Theme 1 highlighted that a fragmented communication process mirrored the chaotic maternal and neonatal course. Parents often received key information about neonatal encephalopathy and therapeutic hypothermia from maternal clinicians. Infant medical information was often given to 1 family member (60%), who felt burdened by the responsibility to relay that information to others. Families universally valued the role of the bedside nurse, who was perceived as the primary source of communication for most (75%) families. Theme 2 encompassed the challenges of discussing the complex therapy of therapeutic hypothermia: families appreciated clinicians who used lay language and provided written material, and they often felt overwhelmed by technical information that made it hard to understand the "big picture" of their infant's medical course. Theme 3 involved the uncertain prognosis after neonatal encephalopathy. Parents appreciated specific expectations about their infant's long-term development, and experienced long-term distress about prognostic uncertainty. Communicating complex and large volumes of information in the midst of perinatal crisis presents inherent challenges for both clinicians and families. We identified an actionable set of communication challenges that can be addressed with targeted interventions. Copyright © 2016 by the American Academy of Pediatrics.

  19. The usefulness of early ultrasonography, electroencephalography and clinical parameters in predicting adverse outcomes in asphyxiated term infants.

    PubMed

    Ong, L C; Kanaheswari, Y; Chandran, V; Rohana, J; Yong, S C; Boo, N Y

    2009-07-01

    The early identification of asphyxiated infants at high risk of adverse outcomes and the early selection of those who might benefit from neuroprotective therapies are required. A prospective observational study was conducted to determine if there were any early clinical, neuroimaging or neurophysiological parameters that might predict the outcome in term newborns with asphyxia. 44 term newborns with acute asphyxia had a cranial ultrasonography (US), electroencephalography (EEG) and clinical examination performed between three and eight hours of life to determine the parameters that might predict outcome. US findings were classified as normal or abnormal (ventricular dilatation or compression and/or focal/diffuse echogenicities). EEG background activity was classified into two categories: normal/mildly abnormal/intermediate, or severely abnormal (low voltage activity or "suppression-burst"). An intrapartum score (based on graded abnormalities of foetal heart monitoring, umbilical arterial base deficit and five-minute Apgar score) and a hypoxic ischaemic encephalopathy (HIE) score (based on graded abnormalities of the neurological and respiratory status at 3-8 hours of life) was also obtained. At one year of life, eight infants had died, six had defaulted follow-up, five had major impairment, two minor impairment and 23 were normal. On univariate analysis, poor outcome (death or major impairment) was associated with abnormal cranial US, severely abnormal EEG and a high HIE score (greater than or equal to 15). The positive predictive value was 54.5, 100 and 100 percent, respectively, while the negative predictive value was 93.8, 80.6 and 80.6 percent, respectively. Combining these factors did not improve the predictive values. There was no added advantage in combining EEG or US parameters over a clinical neurological scoring system alone in predicting the outcome of asphyxiated term newborns.

  20. [Perinatal mortality].

    PubMed

    de la Garza Quintanilla, C; González Salinas, M V

    1995-05-01

    Eighty six cases of perinatal mortality at Hospital de Ginecoobstetricia, Garza García, N.L. Subsecretaría Estatal, from january, 1992 to December, 1993, were reviewed. Perinatal mortality was 12.0 by one thousand births, less than in other reports. The highest incidence was in young patients, 20 to 29 years old, with 47.7% and with parity of 1 to 3, 80.2%; highest frequency in term pregnancies, 37 to 42 weeks, 39.6%; 35% of the products with weight over 2,500 g; and 65% with lesser weight; fetal death occurred most frequently during pre-partum, 55.8%, and less during intra-partum, 19.8%. Most frequent causes of peri-natal death were placental failure, 27.9% and fetal immaturity, with 24.4%. It is concluded that an adequate pre-natal control and delivery surveillance produce a diminution in fetal mortality.

  1. Energy distribution in the spectrograms of the cries of normal and birth asphyxiated infants.

    PubMed

    Pearce, S; Taylor, B

    1993-08-01

    This paper describes the distribution of energy and energy variance with frequency in the cries of normal and birth asphyxiated infants recorded within eight days of delivery. Single-variable statistical analysis suggested that asphyxiated infants have their cries shifted up in frequency compared to control infants, up to a frequency of 10 kHz.

  2. [Asphyxiating thoracic dysplasia (Jeune syndrome): about two cases].

    PubMed

    Harou, K; L'Hermite, M

    2010-04-01

    Asphyxiating thoracic dysplasia (Jeune syndrome) is an osteochondrodysplasia with autosomal recessive inheritance, characterised by a nanism with rhizomelic predominance, associated with a narrow thorax. It induces an alteration of the respiratory function that conditions the prognosis, which is worsened in case of associated visceral lesions (probably related to mutations of genes implicated in ciliary development, as recently described). We report the observation of two severe cases of Jeune syndrome to emphasize the advancement of imaging, especially echography, and molecular biology in establishing prenatal diagnosis as well as prognosis of this syndrome. (c) 2009 Elsevier Masson SAS. All rights reserved.

  3. Autoimmune encephalopathies

    PubMed Central

    Leypoldt, Frank; Armangue, Thaís; Dalmau, Josep

    2014-01-01

    Over the last 10 years the continual discovery of novel forms of encephalitis associated with antibodies to cell-surface or synaptic proteins has changed the paradigms for diagnosing and treating disorders that were previously unknown or mischaracterized. We review here the process of discovery, the symptoms, and the target antigens of twelve autoimmune encephatilic disorders, grouped by syndromes and approached from a clinical perspective. Anti-NMDAR encephalitis, several subtypes of limbic encephalitis, stiff-person spectrum disorders, and other autoimmune encephalitides that result in psychosis, seizures, or abnormal movements are described in detail. We include a novel encephalopathy with prominent sleep dysfunction that provides an intriguing link between chronic neurodegeneration and cell-surface autoimmunity (IgLON5). Some of the caveats of limited serum testing are outlined. In addition, we review the underlying cellular and synaptic mechanisms that for some disorders confirm the antibody pathogenicity. The multidisciplinary impact of autoimmune encephalitis has been expanded recently by the discovery that herpes simplex encephalitis is a robust trigger of synaptic autoimmunity, and that some patients may develop overlapping syndromes, including anti-NMDAR encephalitis and neuromyelitis optica or other demyelinating diseases. PMID:25315420

  4. Pathogenesis of Hepatic Encephalopathy

    PubMed Central

    Ciećko-Michalska, Irena; Szczepanek, Małgorzata; Słowik, Agnieszka; Mach, Tomasz

    2012-01-01

    Hepatic encephalopathy can be a serious complication of acute liver failure and chronic liver diseases, predominantly liver cirrhosis. Hyperammonemia plays the most important role in the pathogenesis of hepatic encephalopathy. The brain-blood barrier disturbances, changes in neurotransmission, neuroinflammation, oxidative stress, GABA-ergic or benzodiazepine pathway abnormalities, manganese neurotoxicity, brain energetic disturbances, and brain blood flow abnormalities are considered to be involved in the development of hepatic encephalopathy. The influence of small intestine bacterial overgrowth (SIBO) on the induction of minimal hepatic encephalopathy is recently emphasized. The aim of this paper is to present the current views on the pathogenesis of hepatic encephalopathy. PMID:23316223

  5. Deaths from asphyxiation and poisoning at work in the United States 1984-6.

    PubMed Central

    Suruda, A; Agnew, J

    1989-01-01

    A review of 4756 deaths investigated by the Occupational Safety and Health Administration (OSHA) in 1984-6 found 233 deaths from asphyxiation and poisoning, excluding asphyxiations from trench cave-ins. The highest rates were in the oil and gas industry and in utilities. Toxic gases were the largest group (65) followed by simple asphyxiants (48), mechanical causes (42), and solvents (35). Deaths from solvents were significantly more likely in young workers. Nine deaths were caused by improper air supply to respirators and five by recreational inhalation of gas or vapours. Of the 146 deaths in confined spaces, only 12% were in rescuers, fewer than previously reported. PMID:2775673

  6. Fatal asphyxiations in children involving drawstrings on clothing.

    PubMed Central

    Petruk, J; Shields, E; Cummings, G E; Francescutti, L H

    1996-01-01

    Injuries account for more deaths and hospital admissions among children and adolescents than all diseases combined. The authors report two deaths by asphyxiation that resulted from drawstrings on the children's clothing becoming entangled on slides. Although such incidents are not common, they are preventable. The authors urge physicians to counsel parents and guardians to remove drawstrings from children's clothing, and they call upon the government and the clothing industry to work toward improving the safety standards for the design, manufacture and importation of children's clothing and banning the sale of children's clothing with drawstrings in Canada. In addition, they provide several resources for readers interested in helping reduce playground hazards in their communities. PMID:8943929

  7. Toxicological findings in three cases of suicidal asphyxiation with helium.

    PubMed

    Oosting, Roelof; van der Hulst, Rogier; Peschier, Leo; Verschraagen, Miranda

    2015-11-01

    Toxicological findings in deaths by asphyxiation due to a pure inert gas like helium are rare. We present three suicide cases of asphyxial death attributed to anoxia caused by inhalation of helium in a plastic bag positioned over the head. In one case, lung tissue, brain tissue and heart blood were obtained during standard autopsy procedures. In two cases, samples were obtained differently: heart blood, femoral blood, brain tissue, lung tissue and/or air from the lungs were directly sealed into headspace vials during autopsy. Air from the lungs was collected using a syringe and transferred into an aluminum gas sampling bag which was heat sealed as soon as possible. Semi-quantitative gas analyses were performed using headspace gas chromatography-thermal conductivity detection (HS-GC/TCD) with a molsieve column capable of separating permanent gasses. Nitrogen was used as carrier gas. In the first case no helium was detected in lung tissue, brain tissue and heart blood. In the second case the presence of helium was detected in lung tissue (approximately 5% helium in gaseous phase) but not in femoral blood. In the third case the presence of helium was detected in air from the lungs (0.05%), lung tissue (0.4%), brain tissue (0.1%) and heart blood (0.04%). Helium is easily lost if sampling is not performed properly. The presented cases suggest that quick sample collection of various matrices during autopsy is suitable to detect gasses like helium in postmortem cases. Use of HS-GC/TCD enables to detect an inert gas like helium. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  8. To Study the Correlation of Thompson Scoring in Predicting Early Neonatal Outcome in Post Asphyxiated Term Neonates

    PubMed Central

    Sharma, Manisha; Dolker, Stanzin; Kothapalli, Sharada

    2016-01-01

    Introduction Throughout the world each year, an estimated 23% of the 4 million neonatal deaths and 8% of all deaths in <5 years of age are associated with signs of asphyxia at birth. Aim To study the role of cord arterial blood gas analysis at birth and serial Thompson score in predicting the early neonatal outcome in post asphyxiated term neonates. Materials and Methods The study was conducted in Department of Paediatrics, in Neonatal Intensive Care Unit (NICU), Hindu Rao Hospital, New Delhi from May 2014 to February. 2015. This study was a prospective cross-sectional study. During this period, a total of 145 post asphyxiated term neonates born in labour room/obstetric operation theatre were recruited. An informed consent was taken from all the parents. The protocol was approved by the institutional ethical committee. Inclusion criteria were full-term babies with low-Apgar score i.e., 1 min score of ≤ 7 National Neonatal Perinatal Database 2010 (NNPD 2010). Statistical Analysis SPSS 17.0 Software has been used for data analysis. The data were expressed in terms of Means, Standard Deviation and Proportion, followed by comparison between groups through chi-square test or Fisher’s-exact test. A p-value of less than 0.05 was considered as statistically significant. Results The present study was carried out on 145 post asphyxiated full-term babies with low-Apgar score i.e., 1min score of ≤7mild Thompson score on day I,2,3 were 96 (66.2%), 119 (82.06%), 125 (86.20%), moderate Thompson score on day 1,3, 7 were 13 (8.9%), 6 (4.13%), 2 (1.37%) and severe Thompson score on day 1, 3, 7 were 36 (24.8%), 13 (8.96%), 7 (4.82%) respectively. Total 11 patients died out of 145 post asphyxiated full-term babies within 7 days, among 11 patients, 7 died within 3 days. There was clinical improvement among HIE patients as indicated by serial Thompson score done on day 1, 3 and 7. Among 145 patients 62(42.8%) had seizure and 83(57.2%) did not have seizure. Most common type of

  9. To Study the Correlation of Thompson Scoring in Predicting Early Neonatal Outcome in Post Asphyxiated Term Neonates.

    PubMed

    Bhagwani, Dalip Kumar; Sharma, Manisha; Dolker, Stanzin; Kothapalli, Sharada

    2016-11-01

    Throughout the world each year, an estimated 23% of the 4 million neonatal deaths and 8% of all deaths in <5 years of age are associated with signs of asphyxia at birth. To study the role of cord arterial blood gas analysis at birth and serial Thompson score in predicting the early neonatal outcome in post asphyxiated term neonates. The study was conducted in Department of Paediatrics, in Neonatal Intensive Care Unit (NICU), Hindu Rao Hospital, New Delhi from May 2014 to February. 2015. This study was a prospective cross-sectional study. During this period, a total of 145 post asphyxiated term neonates born in labour room/obstetric operation theatre were recruited. An informed consent was taken from all the parents. The protocol was approved by the institutional ethical committee. Inclusion criteria were full-term babies with low-Apgar score i.e., 1 min score of ≤ 7 National Neonatal Perinatal Database 2010 (NNPD 2010). SPSS 17.0 Software has been used for data analysis. The data were expressed in terms of Means, Standard Deviation and Proportion, followed by comparison between groups through chi-square test or Fisher's-exact test. A p-value of less than 0.05 was considered as statistically significant. The present study was carried out on 145 post asphyxiated full-term babies with low-Apgar score i.e., 1min score of ≤7mild Thompson score on day I,2,3 were 96 (66.2%), 119 (82.06%), 125 (86.20%), moderate Thompson score on day 1,3, 7 were 13 (8.9%), 6 (4.13%), 2 (1.37%) and severe Thompson score on day 1, 3, 7 were 36 (24.8%), 13 (8.96%), 7 (4.82%) respectively. Total 11 patients died out of 145 post asphyxiated full-term babies within 7 days, among 11 patients, 7 died within 3 days. There was clinical improvement among HIE patients as indicated by serial Thompson score done on day 1, 3 and 7. Among 145 patients 62(42.8%) had seizure and 83(57.2%) did not have seizure. Most common type of seizure was subtle seizure in 25 (40.3%) followed by multifocal in 21 (33

  10. Does Glucagon Improve Survival in a Porcine (Sus Scrofa) of Adult Asphyxial Cardiac Arrest in Addition to Standard Epinephrine Therapy?

    DTIC Science & Technology

    2012-01-17

    UDIIILI: oa. I..UN I ItA!.. I NUMDI:It Does Glucagon improve survival in a porcine (Sus Scrofa ) of adult asphyxial cardiac arrest in addition to...EXPIRATION DATE: 25 Mar 13 PROTOCOL TITLE: Does Glucagon Improve Survival in a Porcine (Sus scrofa ) Model of Adult Asphyxial Cardiac Arrest in Addition...Additions: Deletions: 2 Protocol No: A-2007-03 Protocol Title: Does Glucagon Improve Survival in a Porcine (Sus scrofa ) Model of Adult Asphyxial

  11. Bovine Spongiform Encephalopathy

    USDA-ARS?s Scientific Manuscript database

    Bovine spongiform encephalopathy (BSE), also referred to as “mad cow disease” is a chronic, non-febrile, neuro-degenerative disease affecting the central nervous system. The transmissible spongiform encephalopathies (TSEs) of domestic animals, of which BSE is a member includes scrapie of sheep...

  12. Neonatal withdrawal syndrome and perinatal asphyxia. How to manage the patient? Case Report.

    PubMed

    Brucknerová, Ingrid; Mach, Mojmír; Dubovický, Michal; Ujházy, Eduard

    2015-01-01

    The aim of this work is to present the pitfalls of management of newborns with neonatal withdrawal syndrome (NWS) of different forms, which were complicated with the presence of severe perinatal asphyxia. The authors present some case reports of asphyxiated newborns of different gestational age with different forms of NWS. Prenatal and perinatal asphyxia determines the prognosis of future development of newborn. The combination of the asphyxia and NWS is stressful not only for the patient, but also for the physician. The most important step in management of this group of patients is to know the detailed mother's and patient's history and to perform detailed physical investigation. The optimal prenatal, perinatal and postnatal management with good cooperation between gynecologist and neonatologist can improve the quality of newborn's life. Care of newborn requires all the time teamwork.

  13. Suicidal asphyxiation with helium: report of three cases.

    PubMed

    Grassberger, Martin; Krauskopf, Astrid

    2007-01-01

    Helium is an inert gas that among other things is used medically to alleviate the symptoms of airway obstruction, as part of a diving mix in deep-sea diving or as balloon gas. In recent years the so-called right-to-die literature has suggested suffocation with inhaled helium as an effective and peaceful means of self-deliverance for terminally ill patients. Helium displaces oxygen and carbon dioxide and can thus lead to asphyxia. We report three cases of suicidal asphyxiation with helium gas that were examined at the Department of Forensic Medicine Vienna within three months in 2006. In all three cases, autopsy was unrewarding from the point of view of gross pathology. Special autopsy techniques and devices are required for collection of the gas from the lungs. Gas-chromatography is used to examine the gas for helium; however, this requires replacement of the carrier gas, which is itself usually helium. The fact that three people in Vienna committed suicide using this method within a short period of time, together with the abundance of detailed how-to literature on the Internet, suggests a possible future increase in the number of deaths associated with the inhalation of inert gases, particularly helium. Because of the diagnostic obstacles involved, it is necessary to rely on good death-scene investigation for situational evidence when the body is discovered.

  14. Noncirrhotic hyperammonaemic encephalopathy.

    PubMed

    Laish, Ido; Ben Ari, Ziv

    2011-10-01

    Adult hyperammonaemia is associated with severe liver disease in 90% of cases. In the remainder, noncirrhotic causes should be considered. Measurements of serum ammonia level must be part of the basic work-up in all patients presenting with encephalopathy of unknown origin, even when liver function is normal. Clinician awareness of noncirrhotic hyperammonaemic encephalopathy can contribute to early diagnosis and the initiation of sometimes life-saving treatment. This review focuses on the physiology, aetiology and underlying mechanisms of noncirrhotic hyperammonaemic encephalopathy and discusses the available treatment modalities.

  15. Heavy metal burdens in patches of asphyxiated swamp areas within the Qua Iboe estuary mangrove ecosystem.

    PubMed

    Essien, J P; Essien, V; Olajire, A A

    2009-08-01

    This study examined the burden of Zn, Cu, Ni, Pb, Cr and V in patches of asphyxiated mangrove swamp areas within Qua Iboe Estuary mangrove ecosystem by sediments and surface water analysis; in order to establish natural background levels of these metals and to assess anthropogenic influences on them. The analysis shows that the mean concentrations (mg/kg, dw) of Zn, Cu, Ni, Pb, Cr and V in sediments from asphyxiated and healthy mangrove ecosystems of Qua Iboe vary from 36.3-179.4, 29.2-43.2, 3.6-37.4, 39.6-93.8, 0.15-0.53 and 2.9-9.3, with the former exhibiting higher metal accumulating potential. Although heavy metal concentrations in surface water of the asphyxiated swamp were low, their accumulative effect is significant. The concentrations of Cu and Pb in surface water of this ecosystem exceeded the water quality criteria while Ni and Cr were under the maximum concentration for drinking water quality and protection of aquatic life. The values of pollution load index (PLI), which are generally greater than unity, show that the sediments and the surface water from the asphyxiated mangrove ecosystem were polluted with heavy metals, thus suggesting anthropogenic activities as a possible source of these metals. The mean concentrations of Zn, Ni and Pb exceeded the effects range-low (ERL), indicating that there may be some ecotoxicological risk to organisms living in asphyxiated mangrove sediments.

  16. Right Ventricular Myocardial Ischemia with Arrhythmia in an Asphyxiated Newborn

    PubMed Central

    Solevåg, Anne Lee; Schmölzer, Georg M.; Cheung, Po-Yin

    2016-01-01

    Background Infant and neonatal myocardial infarction (MI) has been described in association with congenital heart disease, coronary artery abnormalities, myocarditis, and tumors. MI in the perinatal period in a structurally normal heart and with ventricular arrhythmia as a presenting feature has not been thoroughly described. Published case reports describe treatment methods extrapolated from adult MI. However, due to the rare occurrence, the most appropriate acute treatment for both MI and ventricular arrhythmia in newborn infants remains unknown. Case A male term infant with perinatal asphyxia and need for extensive cardiopulmonary resuscitation at birth had ventricular tachyarrhythmia and ST-elevations on electrocardiogram. Four hours after birth, he died from cardiogenic failure. A thrombus at the right coronary artery was found on autopsy. Conclusion MI in the perinatal period in a structurally normal heart is very rare and mortality is high. Although acute treatments extrapolated from adult MI has been described to result in favorable outcomes in newborn infants, guidelines are lacking on how to manage acute MI and associated ventricular arrhythmia. PMID:27280062

  17. Hypertensive brain stem encephalopathy.

    PubMed

    Liao, Pen-Yuan; Lee, Chien-Chang; Chen, Cheng-Yu

    2015-01-01

    A 48-year-old man presented with headache and extreme hypertension. Computed tomography showed diffuse brain stem hypodensity. Magnetic resonance imaging revealed diffuse brain stem vasogenic edema. Hypertensive brain stem encephalopathy is an uncommon manifestation of hypertensive encephalopathy, which classically occurs at parietooccipital white matter. Because of its atypical location, the diagnosis can be challenging. Moreover, the coexistence of hypertension and brain stem edema could also direct clinicians toward a diagnosis of ischemic infarction, leading to a completely contradictory treatment goal.

  18. Short and long term prognosis in perinatal asphyxia: An update

    PubMed Central

    Ahearne, Caroline E; Boylan, Geraldine B; Murray, Deirdre M

    2016-01-01

    Interruption of blood flow and gas exchange to the fetus in the perinatal period, known as perinatal asphyxia, can, if significant, trigger a cascade of neuronal injury, leading on to neonatal encephalopathy (NE) and resultant long-term damage. While the majority of infants who are exposed to perinatal hypoxia-ischaemia will recover quickly and go on to have a completely normal survival, a proportion will suffer from an evolving clinical encephalopathy termed hypoxic-ischaemic encephalopathy (HIE) or NE if the diagnosis is unclear. Resultant complications of HIE/NE are wide-ranging and may affect the motor, sensory, cognitive and behavioural outcome of the child. The advent of therapeutic hypothermia as a neuroprotective treatment for those with moderate and severe encephalopathy has improved prognosis. Outcome prediction in these infants has changed, but is more important than ever, as hypothermia is a time sensitive intervention, with a very narrow therapeutic window. To identify those who will benefit from current and emerging neuroprotective therapies we must be able to establish the severity of their injury soon after birth. Currently available indicators such as blood biochemistry, clinical examination and electrophysiology are limited. Emerging biological and physiological markers have the potential to improve our ability to select those infants who will benefit most from intervention. Biomarkers identified from work in proteomics, metabolomics and transcriptomics as well as physiological markers such as heart rate variability, EEG analysis and radiological imaging when combined with neuroprotective measures have the potential to improve outcome in HIE/NE. The aim of this review is to give an overview of the literature in regards to short and long-term outcome following perinatal asphyxia, and to discuss the prediction of this outcome in the early hours after birth when intervention is most crucial; looking at both currently available tools and introducing

  19. Current pathogenetic aspects of hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy.

    PubMed

    Cichoż-Lach, Halina; Michalak, Agata

    2013-01-07

    Hepatic encephalopathy is a medical phenomenon that is described as a neuropsychiatric manifestation of chronic or acute liver disease that is characterized by psychomotor, intellectual and cognitive abnormalities with emotional/affective and behavioral disturbances. This article focuses on the underlying mechanisms of the condition and the differences between hepatic encephalopathy and noncirrhotic hyperammonemic encephalopathy. Hepatic encephalopathy is a serious condition that can cause neurological death with brain edema and intracranial hypertension. It is assumed that approximately 60%-80% of patients with liver cirrhosis develop hepatic encephalopathy. This review explores the complex mechanisms that lead to hepatic encephalopathy. However, noncirrhotic hyperammonemic encephalopathy is not associated with hepatic diseases and has a completely different etiology. Noncirrhotic hyperammonemic encephalopathy is a severe occurrence that is connected with multiple pathogeneses.

  20. The Role of Plasma and Urine Metabolomics in Identifying New Biomarkers in Severe Newborn Asphyxia: A Study of Asphyxiated Newborn Pigs following Cardiopulmonary Resuscitation

    PubMed Central

    Sachse, Daniel; Solevåg, Anne Lee; Berg, Jens Petter; Nakstad, Britt

    2016-01-01

    Background Optimizing resuscitation is important to prevent morbidity and mortality from perinatal asphyxia. The metabolism of cells and tissues is severely disturbed during asphyxia and resuscitation, and metabolomic analyses provide a snapshot of many small molecular weight metabolites in body fluids or tissues. In this study metabolomics profiles were studied in newborn pigs that were asphyxiated and resuscitated using different protocols to identify biomarkers for subject characterization, intervention effects and possibly prognosis. Methods A total of 125 newborn Noroc pigs were anesthetized, mechanically ventilated and inflicted progressive asphyxia until asystole. Pigs were randomized to resuscitation with a FiO2 0.21 or 1.0, different duration of ventilation before initiation of chest compressions (CC), and different CC to ventilation ratios. Plasma and urine samples were obtained at baseline, and 2 h and 4 h after return of spontaneous circulation (ROSC, heart rate > = 100 bpm). Metabolomics profiles of the samples were analyzed by nuclear magnetic resonance spectroscopy. Results Plasma and urine showed severe metabolic alterations consistent with hypoxia and acidosis 2 h and 4 h after ROSC. Baseline plasma hypoxanthine and lipoprotein concentrations were inversely correlated to the duration of hypoxia sustained before asystole occurred, but there was no evidence for a differential metabolic response to the different resuscitation protocols or in terms of survival. Conclusions Metabolic profiles of asphyxiated newborn pigs showed severe metabolic alterations. Consistent with previously published reports, we found no evidence of differences between established and alternative resuscitation protocols. Lactate and pyruvate may have a prognostic value, but have to be independently confirmed. PMID:27529347

  1. Neurological abnormalities in full-term asphyxiated newborns and salivary S100B testing: the "Cooperative Multitask against Brain Injury of Neonates" (CoMBINe) international study.

    PubMed

    Gazzolo, Diego; Pluchinotta, Francesca; Bashir, Moataza; Aboulgar, Hanna; Said, Hala Mufeed; Iman, Iskander; Ivani, Giorgio; Conio, Alessandra; Tina, Lucia Gabriella; Nigro, Francesco; Li Volti, Giovanni; Galvano, Fabio; Michetti, Fabrizio; Di Iorio, Romolo; Marinoni, Emanuela; Zimmermann, Luc J; Gavilanes, Antonio D W; Vles, Hans J S; Kornacka, Maria; Gruszfeld, Darek; Frulio, Rosanna; Sacchi, Renata; Ciotti, Sabina; Risso, Francesco M; Sannia, Andrea; Florio, Pasquale

    2015-01-01

    Perinatal asphyxia (PA) is a leading cause of mortality and morbidity in newborns: its prognosis depends both on the severity of the asphyxia and on the immediate resuscitation to restore oxygen supply and blood circulation. Therefore, we investigated whether measurement of S100B, a consolidated marker of brain injury, in salivary fluid of PA newborns may constitute a useful tool for the early detection of asphyxia-related brain injury. We conducted a cross-sectional study in 292 full-term newborns admitted to our NICUs, of whom 48 suffered PA and 244 healthy controls admitted at our NICUs. Saliva S100B levels measurement longitudinally after birth; routine laboratory variables, neurological patterns, cerebral ultrasound and, magnetic resonance imaging were performed. The primary end-point was the presence of neurological abnormalities at 12-months after birth. S100B salivary levels were significantly (P<0.001) higher in newborns with PA than in normal infants. When asphyxiated infants were subdivided according to a good (Group A; n = 15) or poor (Group B; n = 33) neurological outcome at 12-months, S100B was significantly higher at all monitoring time-points in Group B than in Group A or controls (P<0.001, for all). A cut-off >3.25 MoM S100B achieved a sensitivity of 100% (CI5-95%: 89.3%-100%) and a specificity of 100% (CI5-95%: 98.6%-100%) as a single marker for predicting the occurrence of abnormal neurological outcome (area under the ROC curve: 1.000; CI5-95%: 0.987-1.0). S100B protein measurement in saliva, soon after birth, is a useful tool to identify which asphyxiated infants are at risk of neurological sequelae.

  2. [Hashimoto's encephalopathy - rare encephalopathy with good prognosis].

    PubMed

    Kaczmarczyk, Aleksandra; Patalong-Ogiewa, M; Krzystanek, E

    2016-01-01

    Hashimoto's encephalopathy (HE) is a rare neuropsychiatric syndrome associated with increased level of antithyroid antibodies. Two types of clinical manifestation can be described: a vasculitic type with stroke like episodes and diffuse progressive type with deterioration of mental function. Neurologic symptoms are present in euthyreosis as well as in thyroid dysfunction. Because of good response to immunosuppressive therapy, the prompt diagnosis and management of HE are crucial. In this study we present the review of current literature and discuss two representative cases.

  3. Posterior reversible encephalopathy syndrome: a variant of hypertensive encephalopathy.

    PubMed

    Mirza, Ayoub

    2006-06-01

    Posterior reversible encephalopathy syndrome (PRES) is a recently described variant of hypertensive encephalopathy characterized by headache, visual disturbances and altered mental function. Its causes are diverse and in contrast to hypertensive encephalopathy, it can develop without significant elevation of blood pressure. This syndrome is mostly reversible when correctly managed; however, failure to recognize it can lead to cerebral infarction and death.

  4. Mechanisms underlying uremic encephalopathy.

    PubMed

    Scaini, Giselli; Ferreira, Gabriela Kozuchovski; Streck, Emilio Luiz

    2010-06-01

    In patients with renal failure, encephalopathy is a common problem that may be caused by uremia, thiamine deficiency, dialysis, transplant rejection, hypertension, fluid and electrolyte disturbances or drug toxicity. In general, encephalopathy presents with a symptom complex progressing from mild sensorial clouding to delirium and coma. This review discusses important issues regarding the mechanisms underlying the pathophysiology of uremic encephalopathy. The pathophysiology of uremic encephalopathy up to now is uncertain, but several factors have been postulated to be involved; it is a complex and probably multifactorial process. Hormonal disturbances, oxidative stress, accumulation of metabolites, imbalance in excitatory and inhibitory neurotransmitters, and disturbance of the intermediary metabolism have been identified as contributing factors. Despite continuous therapeutic progress, most neurological complications of uremia, like uremic encephalopathy, fail to fully respond to dialysis and many are elicited or aggravated by dialysis or renal transplantation. On the other hand, previous studies showed that antioxidant therapy could be used as an adjuvant therapy for the treatment of these neurological complications.

  5. Predictors of Asphyxiation Risk in Adults with Intellectual Disabilities and Dysphagia

    ERIC Educational Resources Information Center

    Samuels, R.; Chadwick, D. D.

    2006-01-01

    Background: Adults with learning disabilities referred for assessment of their eating and drinking are frequently reported to cough and choke when eating and drinking. The research literature investigating dysphagia has often overlooked asphyxiation risk, highlighting coughing and choking as indicators of aspiration only. This is a notable…

  6. Fatal Asphyxiation in Bottlenose Dolphins (Tursiops truncatus) from the Indian River Lagoon

    PubMed Central

    Stolen, Megan; St. Leger, Judy; Durden, Wendy Noke; Mazza, Teresa; Nilson, Erika

    2013-01-01

    Multiple single case reports of asphyxiation in dolphins caused by fish lodged in the esophagus exist. However, the significance of this cause of mortality in a single population has not been documented. We performed a retrospective evaluation of pathology records from stranded bottlenose dolphins (Tursiops truncatus) from the Indian River Lagoon to evaluate the impact of this cause of death on this population. From 1997 to 2011, asphyxiation due to choking was identified as the cause of death in 14 of 350 cases (4%). Sampling of an unrelated but adjacent population over this same period yielded 186 necropsy cases of bottlenose dolphins with no cases of asphyxiation. Asphyxiated animals presented with a fish lodged in the cranial esophagus associated with a dislocated and obstructed or compressed larynx. There was no clear sex predilection. Affected animals included 12 adults and two juveniles. The fish species involved included sheepshead, black chin tilapia and striped mojarra. In five cases, recreational fishing gear was also present. Cetacean choking is related to selection of prey fish species with strong dorsal spines and may be secondarily associated with fish attached to fishing gear. Prey abundance and dolphin behavior may influence these selections. Environmental alterations leading to changes in prey availability or increased interactions with fishing gear may change the significance of fatal choking in dolphin populations. PMID:23840535

  7. Fatal Asphyxiation in Bottlenose Dolphins (Tursiops truncatus) from the Indian River Lagoon.

    PubMed

    Stolen, Megan; St Leger, Judy; Durden, Wendy Noke; Mazza, Teresa; Nilson, Erika

    2013-01-01

    Multiple single case reports of asphyxiation in dolphins caused by fish lodged in the esophagus exist. However, the significance of this cause of mortality in a single population has not been documented. We performed a retrospective evaluation of pathology records from stranded bottlenose dolphins (Tursiops truncatus) from the Indian River Lagoon to evaluate the impact of this cause of death on this population. From 1997 to 2011, asphyxiation due to choking was identified as the cause of death in 14 of 350 cases (4%). Sampling of an unrelated but adjacent population over this same period yielded 186 necropsy cases of bottlenose dolphins with no cases of asphyxiation. Asphyxiated animals presented with a fish lodged in the cranial esophagus associated with a dislocated and obstructed or compressed larynx. There was no clear sex predilection. Affected animals included 12 adults and two juveniles. The fish species involved included sheepshead, black chin tilapia and striped mojarra. In five cases, recreational fishing gear was also present. Cetacean choking is related to selection of prey fish species with strong dorsal spines and may be secondarily associated with fish attached to fishing gear. Prey abundance and dolphin behavior may influence these selections. Environmental alterations leading to changes in prey availability or increased interactions with fishing gear may change the significance of fatal choking in dolphin populations.

  8. Predictors of Asphyxiation Risk in Adults with Intellectual Disabilities and Dysphagia

    ERIC Educational Resources Information Center

    Samuels, R.; Chadwick, D. D.

    2006-01-01

    Background: Adults with learning disabilities referred for assessment of their eating and drinking are frequently reported to cough and choke when eating and drinking. The research literature investigating dysphagia has often overlooked asphyxiation risk, highlighting coughing and choking as indicators of aspiration only. This is a notable…

  9. Infantile mitochondrial encephalopathy.

    PubMed

    Uziel, Graziella; Ghezzi, Daniele; Zeviani, Massimo

    2011-08-01

    Individually rare, when taken as a whole, genetic inborn errors of metabolism (IEM) account for a significant proportion of early onset encephalopathy. Prompt diagnosis is crucial to assess appropriate investigation and can sometimes warrant successful therapy. Recent improvements in technology and expansion of knowledge on the biochemical and molecular basis of these disorders allow astute child neurologists and paediatricians to improve the early diagnosis of these genetically determined defects. However, because of rarity and heterogeneity of these disorders, IEM encephalopathies are still a formidable challenge for most physicians. The most frequent cause of childhood IEM encephalopathy is mitochondrial disease, whose biochemical 'signature' is faulty energy supply due to defects of the last component of the oxidative pathways residing within mitochondria, i.e. the mitochondrial respiratory chain. Copyright © 2011. Published by Elsevier Ltd.

  10. Minimal hepatic encephalopathy.

    PubMed

    Zamora Nava, Luis Eduardo; Torre Delgadillo, Aldo

    2011-06-01

    The term minimal hepatic encephalopathy (MHE) refers to the subtle changes in cognitive function, electrophysiological parameters, cerebral neurochemical/neurotransmitter homeostasis, cerebral blood flow, metabolism, and fluid homeostasis that can be observed in patients with cirrhosis who have no clinical evidence of hepatic encephalopathy; the prevalence is as high as 84% in patients with hepatic cirrhosis. Physician does generally not perceive cirrhosis complications, and neuropsychological tests and another especial measurement like evoked potentials and image studies like positron emission tomography can only make diagnosis. Diagnosis of minimal hepatic encephalopathy may have prognostic and therapeutic implications in cirrhotic patients. The present review pretends to explore the clinic, therapeutic, diagnosis and prognostic aspects of this complication.

  11. [Hashimoto's encephalopathy and autoantibodies].

    PubMed

    Yoneda, Makoto

    2013-04-01

    Encephalopathy occasionally occurs in association with thyroid disorders, but most of these are treatable. These encephalopathies include a neuropsychiatric disorder associated with hypothyroidism, called myxedema encephalopathy. Moreover, Hashimoto's encephalopathy (HE) has been recognized as a new clinical disease based on an autoimmune mechanism associated with Hashimoto's thyroiditis. Steroid treatment was successfully administered to these patients. Recently, we discovered that the serum autoantibodies against the NH2-terminal of α-enolase (NAE) are highly specific diagnostic biomarkers for HE. Further, we analyzed serum anti-NAE autoantibodies and the clinical features in many cases of HE from institutions throughout Japan and other countries. Approximately half of assessed HE patients carry anti-NAE antibodies. The age was widely distributed with 2 peaks (20-30 years and 50-70 years). Most HE patients were in euthyroid states, and all patients had anti-thyroid (TG) antibodies and anti-thyroid peroxidase (TPO) antibodies. Anti-TSH receptor (TSH-R) antibodies were observed in some cases. The common neuropsychiatry features are consciousness disturbance and psychosis, followed by cognitive dysfunction, involuntary movements, seizures, and ataxia. Abnormalities on electroencephalography (EEG) and decreased cerebral blood flow on brain SPECT were common findings, whereas abnormal findings on brain magnetic resonance imaging (MRI) were rare. HE patients have various clinical phenotypes such as the acute encephalopathy form, the chronic psychiatric form, and other particular clinical forms, including limbic encephalitis, progressive cerebellar ataxia, and Creutzfeldt-Jakob disease (CJD)-like form. The cerebellar ataxic form of HE clinically mimics spinocerebellar degeneration (SCD) and is characterized by the absence of nystagmus, absent or mild cerebellar atrophy, and lazy background activities on EEG. Taken together, these data suggest that the possibility of

  12. Non-avalanche-related snow immersion deaths: tree well and deep snow immersion asphyxiation.

    PubMed

    Van Tilburg, Christopher

    2010-09-01

    Non-avalanche-related snow immersion death (NARSID), or snow immersion asphyxiation, is a significant winter mountain hazard for skiers and snowboarders. This phenomenon occurs predominately in western North America, where large tree wells and deep snowpacks develop. Although statistics are difficult to procure, snow immersion asphyxiation has resulted in more than 70 documented deaths in the past 2 decades. The primary purpose of this review is to examine the existing literature on NARSID to help prevent such dangerous accidents through educating wilderness medicine professionals and fostering public awareness. The exact duration of burial to time of death and the cause of death are not precisely known but can be postulated from accident reports, experimental snow burial studies, and avalanche literature. In most cases, death probably occurs within 15 to 30 minutes from the time of burial. However, survival after prolonged burial in a tree well and deep snow is possible. The cause of death is asphyxiation, probably due to one of the mechanisms that produce asphyxia in avalanche burial victims: positional asphyxia, airway obstruction, or carbon dioxide displacement asphyxia. Prevention of snow immersion asphyxiation begins with skiers and snowboarders staying within the limits of their skills, using the proper tools for deep powder, staying in control at all times, and employing a buddy system. A skier or snowboarder who falls near or into a tree well should tuck, roll, and try to land upright, grab the tree trunk or a branch, and yell or blow a whistle to alert partners. If buried upside down, the person should stay calm and create an air pocket, which is probably of paramount importance. Skiers and snowboarders should use avalanche safety equipment to lessen the risk of snow submersion asphyxiation.

  13. Burn encephalopathy in children.

    PubMed

    Mohnot, D; Snead, O C; Benton, J W

    1982-07-01

    Among 287 children with burns treated over a recent two-year period, 13 (5%) showed evidence of encephalopathy. The major clinical symptoms were an altered sensorium and seizures. The majority of symptoms began later than 48 hours after the burn and were accompanied by multiple metabolic aberrations including hypocalcemia. Three children had a relapsing course, and 1 had temporarily enlarged cerebral ventricles. Eleven children improved to normal. In the majority of instances, burn encephalopathy probably reflects central nervous system dysfunction resulting from complex metabolic, hematological, and hemodynamic abnormalities rather than from a single metabolic abnormality.

  14. Correlation of Apgar Score with Asphyxial Hepatic Injury and Mortality in Newborns: A Prospective Observational Study From India

    PubMed Central

    Sharma, Deepak; Choudhary, Mukesh; Lamba, Mamta; Shastri, Sweta

    2016-01-01

    OBJECTIVE The objective of this study is to determine the correlation of Apgar score with asphyxial hepatic injury and neonatal mortality in moderately and severely asphyxiated newborns. MATERIAL AND METHODS This is a secondary analysis of our prospective observational case-controlled study. Sixteen neonates with severe birth asphyxia (five-minute Apgar ≤3) were compared with either 54 moderate asphyxia neonates (five-minute Apgar >3) or 30 normal neonates. Liver function tests were measured on postnatal days 1, 3, and 10 in the study and control groups. Neonatal mortality was observed in the study and control population. RESULTS Correlation of Apgar score in severely asphyxiated neonates compared with normal Apgar score neonates and moderately asphyxiated neonates for deranged hepatic function showed significant correlation (odds ratio [OR] 4.88, 95% CI 3.26–5.84, P = 0.01 and OR 2.46, 95% CI 1.94–3.32, P = 0.02, respectively). There was a significant increase in serum lactate dehydrogenase (LDH) and total bilirubin on day 1 and serum LDH at age of 10th postnatal life in severely asphyxiated neonates when compared to moderately asphyxiated neonates, whereas there was a significant decrease in total bilirubin and serum albumin on day 3 in severely asphyxiated neonates. There was a significant increase in serum alanine transaminase, serum LDH, and total bilirubin on day 1, serum aspartate transaminase, serum LDH, and total bilirubin on day 3, and International Normalized Ratio on day 10 of postnatal life when severely asphyxiated neonates were compared with normal neonates. There was a significant reduction in total protein and serum albumin on day 1 and direct bilirubin on day 3 in severely asphyxiated neonates when compared with normal neonates. There was a significant increase in neonatal mortality in severely asphyxiated neonates when compared to the other two groups. Correlation of Apgar score in severely asphyxiated neonates compared with normal Apgar

  15. Management of covert hepatic encephalopathy.

    PubMed

    Waghray, Abhijeet; Waghray, Nisheet; Mullen, Kevin

    2015-03-01

    Hepatic encephalopathy is a reversible progressive neuropsychiatric disorder that encompasses a wide clinical spectrum. Covert hepatic encephalopathy is defined as patients with minimal hepatic encephalopathy and Grade I encephalopathy by West-Haven Criteria. Terminology such as "sub-clinical", "latent", and "minimal" appear to trivialize the disease and have been replaced by the term covert. The lack of clinical signs means that covert hepatic encephalopathy is rarely recognized or treated outside of clinical trials with options for therapy based on patients with episodic hepatic encephalopathy. This review discusses the current available options for therapy in covert hepatic encephalopathy and focuses on non-absorbable disacharides (lactulose or lactitol), antibiotics (rifaximin), probiotics/synbiotics and l-ornithine-l-aspartate.

  16. Management of Hepatic Encephalopathy

    PubMed Central

    Wright, G.; Chattree, A.; Jalan, R.

    2011-01-01

    Hepatic encephalopathy (HE), the neuropsychiatric presentation of liver disease, is associated with high morbidity and mortality. Reduction of plasma ammonia remains the central therapeutic strategy, but there is a need for newer novel therapies. We discuss current evidence supporting the use of interventions for both the general management of chronic HE and that necessary for more acute and advanced disease. PMID:21994873

  17. Bovine Spongiform Encephalopathy

    USDA-ARS?s Scientific Manuscript database

    Bovine spongiform encephalopathy (BSE) is caused by a novel contagion, known to as a prion. Prions are proteins capable of converting a normal cellular protein into a prion, thereby propagating an infection. BSE is the first known prion zoonotic. As such it has attracted broad scientific and, to a r...

  18. KCNQ2 encephalopathy

    PubMed Central

    Millichap, John J.; Park, Kristen L.; Tsuchida, Tammy; Ben-Zeev, Bruria; Carmant, Lionel; Flamini, Robert; Joshi, Nishtha; Levisohn, Paul M.; Marsh, Eric; Nangia, Srishti; Narayanan, Vinodh; Ortiz-Gonzalez, Xilma R.; Patterson, Marc C.; Pearl, Phillip L.; Porter, Brenda; Ramsey, Keri; McGinnis, Emily L.; Taglialatela, Maurizio; Tracy, Molly; Tran, Baouyen; Venkatesan, Charu; Weckhuysen, Sarah

    2016-01-01

    Objective: To advance the understanding of KCNQ2 encephalopathy genotype–phenotype relationships and to begin to assess the potential of selective KCNQ channel openers as targeted treatments. Methods: We retrospectively studied 23 patients with KCNQ2 encephalopathy, including 11 treated with ezogabine (EZO). We analyzed the genotype–phenotype relationships in these and 70 previously described patients. Results: The mean seizure onset age was 1.8 ± 1.6 (SD) days. Of the 20 EEGs obtained within a week of birth, 11 showed burst suppression. When new seizure types appeared in infancy (15 patients), the most common were epileptic spasms (n = 8). At last follow-up, seizures persisted in 9 patients. Development was delayed in all, severely in 14. The KCNQ2 variants identified introduced amino acid missense changes or, in one instance, a single residue deletion. They were clustered in 4 protein subdomains predicted to poison tetrameric channel functions. EZO use (assessed by the treating physicians and parents) was associated with improvement in seizures and/or development in 3 of the 4 treated before 6 months of age, and 2 of the 7 treated later; no serious side effects were observed. Conclusions: KCNQ2 variants cause neonatal-onset epileptic encephalopathy of widely varying severity. Pathogenic variants in epileptic encephalopathy are clustered in “hot spots” known to be critical for channel activity. For variants causing KCNQ2 channel loss of function, EZO appeared well tolerated and potentially beneficial against refractory seizures when started early. Larger, prospective studies are needed to enable better definition of prognostic categories and more robust testing of novel interventions. Classification of evidence: This study provides Class IV evidence that EZO is effective for refractory seizures in patients with epilepsy due to KCNQ2 encephalopathy. PMID:27602407

  19. Reflex gelastic-dacrystic seizures following hypoxic-ischaemic encephalopathy.

    PubMed

    Verma, Rajesh; Praharaj, Heramba Narayan

    2013-07-12

    Reflex or stimulus-sensitive epilepsies are uncommon epileptic syndromes triggered by exogenous-specific sensory stimulus or endogenous various mental activities. Gelastic-dacrystic seizures are rare epileptic manifestations characterised by ictal laughter and crying. Gelastic-dacrystic seizures are commonly caused by hypothalamic hamartoma but rarely described due to cortical dysplasia, lesions of frontal and temporal lobes, tumours and vascular malformations. We report a young woman who presented with somatosensory-evoked gelastic-dacrystic seizures. This patient had a positive history of perinatal insult substantiated by MRI findings. Hypoxic-ischaemic encephalopathy as the cause of gelastic-dacrystic seizures has not been reported so far in the literature.

  20. Epileptic encephalopathy in children with risk factors for brain damage.

    PubMed

    Ricardo-Garcell, Josefina; Harmony, Thalía; Porras-Kattz, Eneida; Colmenero-Batallán, Miguel J; Barrera-Reséndiz, Jesús E; Fernández-Bouzas, Antonio; Cruz-Rivero, Erika

    2012-01-01

    In the study of 887 new born infants with prenatal and perinatal risk factors for brain damage, 11 children with West syndrome that progressed into Lennox-Gastaut syndrome and another 4 children with Lennox-Gastaut syndrome that had not been preceded by West syndrome were found. In this study we present the main findings of these 15 subjects. In all infants multifactor antecedents were detected. The most frequent risk factors were prematurity and severe asphyxia; however placenta disorders, sepsis, and hyperbilirubinemia were also frequent. In all infants MRI direct or secondary features of periventricular leukomalacia were observed. Followup of all infants showed moderate to severe neurodevelopmental delay as well as cerebral palsy. It is concluded that prenatal and perinatal risk factors for brain damage are very important antecedents that should be taken into account to follow up those infants from an early age in order to detect and treat as early as possible an epileptic encephalopathy.

  1. Epileptic Encephalopathy in Children with Risk Factors for Brain Damage

    PubMed Central

    Ricardo-Garcell, Josefina; Harmony, Thalía; Porras-Kattz, Eneida; Colmenero-Batallán, Miguel J.; Barrera-Reséndiz, Jesús E.; Fernández-Bouzas, Antonio; Cruz-Rivero, Erika

    2012-01-01

    In the study of 887 new born infants with prenatal and perinatal risk factors for brain damage, 11 children with West syndrome that progressed into Lennox-Gastaut syndrome and another 4 children with Lennox-Gastaut syndrome that had not been preceded by West syndrome were found. In this study we present the main findings of these 15 subjects. In all infants multifactor antecedents were detected. The most frequent risk factors were prematurity and severe asphyxia; however placenta disorders, sepsis, and hyperbilirubinemia were also frequent. In all infants MRI direct or secondary features of periventricular leukomalacia were observed. Followup of all infants showed moderate to severe neurodevelopmental delay as well as cerebral palsy. It is concluded that prenatal and perinatal risk factors for brain damage are very important antecedents that should be taken into account to follow up those infants from an early age in order to detect and treat as early as possible an epileptic encephalopathy. PMID:22957240

  2. Periictal activity in cooled asphyxiated neonates with seizures.

    PubMed

    Major, Philippe; Lortie, Anne; Dehaes, Mathieu; Lodygensky, Gregory Anton; Gallagher, Anne; Carmant, Lionel; Birca, Ala

    2017-04-01

    Seizures are common in critically ill neonates. Both seizures and antiepileptic treatments may lead to short term complications and worsen the outcomes. Predicting the risks of seizure reoccurrence could enable individual treatment regimens and better outcomes. We aimed to identify EEG signatures of seizure reoccurrence by investigating periictal electrographic features and spectral power characteristics in hypothermic neonates with hypoxic-ischemic encephalopathy (HIE) with or without reoccurrence of seizures on rewarming. We recruited five consecutive HIE neonates, submitted to continuous EEG monitoring, with high seizure burden (>20% per hour) while undergoing therapeutic hypothermia. Two of them had reoccurrence of seizures on rewarming. We performed quantitative analysis of fifteen artifact-free consecutive seizures to appreciate spectral power changes between the interictal, preictal and ictal periods, separately for each patient. Visual analysis allowed description of electrographic features associated with ictal events. Every patient demonstrated a significant increase in overall spectral power from the interictal to preictal and ictal periods (p<0.01). Alpha power increase was more pronounced in the two patients with reoccurrence of seizures on rewarming and significant when comparing both interictal-to-preictal and interictal-to-ictal periods. This alpha activity increase could be also appreciated using visual analysis and distinguished neonates with and without seizure reoccurrence. This distinct alpha activity preceding ictal onset could represent a biomarker of propensity for seizure reoccurrence in neonates. Future studies should be performed to confirm whether quantitative periictal characteristics and electrographic features allow predicting the risks of seizure reoccurrence in HIE neonates and other critically ill patients. Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  3. Treatment of asphyxiated newborns with moderate hypothermia in routine clinical practice: how cooling is managed in the UK outside a clinical trial.

    PubMed

    Azzopardi, D; Strohm, B; Edwards, A D; Halliday, H; Juszczak, E; Levene, M; Thoresen, M; Whitelaw, A; Brocklehurst, P

    2009-07-01

    This is a phase 4 study of infants registered with the UK TOBY Cooling Register from December 2006 to February 2008. The registry was established on completion of enrolLment to the TOBY randomised trial of treatment with whole body hypothermia following perinatal asphyxia at the end of November 2006. We collected information about patient characteristics, condition at birth, resuscitation details, severity of encephalopathy, hourly temperature record, clinical complications and outcomes before hospital discharge. 120 infants born at a median of 40 (IQR 38-41) weeks' gestation and weighing a median of 3287 (IQR 2895-3710) g at birth were studied. Cooling was started at a median of 3 h 54 min (IQR 2 h-5 h 32 min) after birth. All but three infants underwent whole body cooling. The mean (SD) rectal temperature from 6 to 72 h of the cooling period was 33.57 degrees C (0.51 degrees C). The daily encephalopathy score fell: median (IQR) 11 (6-15), 9.7 (5-14), 8 (5-13) and 7 (2-12) on days 1-4 after birth, respectively. 51% of the infants established full oral feeding at a median (range) of 9 (4-24) days. 26% of the study infants died. MRI was consistent with hypoxia-ischaemia in most cases. Clinical complications were not considered to be due to hypothermia. In the UK, therapeutic hypothermia following perinatal asphyxia is increasingly being provided. The target body temperature is successfully achieved and the clinical complications observed were not attributed to hypothermia. Treatment with hypothermia may have prevented the worsening of the encephalopathy that is commonly observed following asphyxia.

  4. Asphyxiation by occlusion of nose and mouth by duct tape: two unusual suicides.

    PubMed

    deRoux, Stephen; Leffers, Beverly

    2009-11-01

    The most recent U.S. statistics (2005) determined that 22.2% of suicides are by suffocation. This number likely includes suicidal hanging. Based on previous reports the majority of nonhanging suicidal asphyxiations are accomplished by securing a plastic bag over the head. We report two instances of a far less common method of suicidal asphyxiation, occlusion of the nose and mouth by duct tape. One was a 47-year-old man with a history of paranoid schizophrenia with suicidal ideation and the other was a 52-year-old man who was depressed due to gambling debts. The value of scene investigation, including review of available video surveillance to determine the manner of death is highlighted.

  5. Fear and pulmonary stress behaviors to an asphyxial threat across cognitive states.

    PubMed

    Campbell, Margaret L

    2007-12-01

    The purpose of this exploratory study was to identify behaviors that may signify respiratory distress across cognitive states in response to an asphyxial threat. Patients undergoing a ventilator weaning trial were assessed and observed at baseline and during weaning with a capnograph/oximeter and video camera. Cognitive state was categorized at baseline, and an emotion report was elicited after the trial. Pulmonary stress and fear behaviors were similar across cognitive states. Hypercarbia predicted activation of fear behaviors. Gender differences characterized emotion reporting. An asphyxial threat may induce an innate array of behaviors that cannot be volitionally controlled and that may have the same appearance across cognitive states. Recognizing respiratory distress behaviors may improve nursing care of patients who are cognitively impaired.

  6. Continuous End-tidal Carbon Dioxide Monitoring during Resuscitation of Asphyxiated Term Lambs

    PubMed Central

    Chandrasekharan, Praveen Kumar; Rawat, Munmun; Nair, Jayasree; Gugino, Sylvia F.; Koenigschnekt, Carmon; Swartz, Daniel D.; Vali, Payam; Mathew, Bobby; Lakshminrusimha, Satyan

    2016-01-01

    Background The neonatal resuscitation program (NRP) recommends close monitoring of oxygenation during the resuscitation of newborns using a pulse oximeter. However, there are no guidelines for monitoring carbon dioxide (CO2) to assess ventilation. Considering that cerebral blood flow (CBF) correlates directly with PaCO2, continuous capnography monitoring of end-tidal CO2 (ETCO2) may limit fluctuations in PaCO2 and, therefore, CBF during resuscitation of asphyxiated infants. Objective To evaluate if continuous monitoring of ETCO2 with capnography during resuscitation of asphyxiated term lambs with meconium aspiration will prevent fluctuations in PaCO2 and carotid arterial blood flow (CABF). Methods Fifty-four asphyxiated term lambs with meconium aspiration syndrome were mechanically ventilated from birth to 60 min of age. Ventilatory parameters were adjusted based on clinical observation (chest excursion) and frequent arterial blood gas analysis in 24 lambs (control group) and 30 lambs (capnography group) received additional continuous ETCO2 monitoring. Left CABF was monitored. We aimed to maintain PaCO2 between 35–50 mmHg and ETCO2 between 30–45 mmHg. Results There was a significant correlation between ETCO2 and PaCO2 (R=0.7, p<0.001), between PaCO2 and carotid flow (R=0.52, p<0.001), and between ETCO2 and carotid flow (R=0.5, p<0.001). PaCO2 and CABF during the first 60 minutes of age showed significantly higher fluctuation in the control group compared to the capnography group. Conclusion Continuous monitoring of ETCO2 using capnography with mechanical ventilation during and after resuscitation in asphyxiated term lambs with meconium aspiration limits fluctuations in PaCO2 and CABF, and may potentially limit brain injury. PMID:26866711

  7. Neural Correlates of Consciousness at Near-Electrocerebral Silence in an Asphyxial Cardiac Arrest Model.

    PubMed

    Lee, Donald E; Lee, Lauren G; Siu, Danny; Bazrafkan, Afsheen K; Farahabadi, Maryam H; Dinh, Tin J; Orellana, Josue; Xiong, Wei; Lopour, Beth A; Akbari, Yama

    2017-04-01

    Recent electrophysiological studies have suggested surges in electrical correlates of consciousness (i.e., elevated gamma power and connectivity) after cardiac arrest (CA). This study examines electrocorticogram (ECoG) activity and coherence of the dying brain during asphyxial CA. Male Wistar rats (n = 16) were induced with isoflurane anesthesia, which was washed out before asphyxial CA. Mean phase coherence and ECoG power were compared during different stages of the asphyxial period to assess potential neural correlates of consciousness. After asphyxia, the ECoG progressed through four distinct stages (asphyxial stages 1-4 [AS1-4]), including a transient period of near-electrocerebral silence lasting several seconds (AS3). Electrocerebral silence (AS4) occurred within 1 min of the start of asphyxia, and pulseless electrical activity followed the start of AS4 by 1-2 min. AS3 was linked to a significant increase in frontal coherence between the left and right motor cortices (p < 0.05), with no corresponding increase in ECoG power. AS3 was also associated with a significant posterior shift of ECoG power, favoring the visual cortices (p < 0.05). Although the ECoG during AS3 appears visually flat or silent when viewed with standard clinical settings, our study suggests that this period of transient near-electrocerebral silence contains distinctive neural activity. Specifically, the burst in frontal coherence and posterior shift of ECoG power that we find during this period immediately preceding CA may be a neural correlate of conscious processing.

  8. Neural Correlates of Consciousness at Near-Electrocerebral Silence in an Asphyxial Cardiac Arrest Model

    PubMed Central

    Lee, Donald E.; Lee, Lauren G.; Siu, Danny; Bazrafkan, Afsheen K.; Farahabadi, Maryam H.; Dinh, Tin J.; Orellana, Josue; Xiong, Wei; Lopour, Beth A.

    2017-01-01

    Abstract Recent electrophysiological studies have suggested surges in electrical correlates of consciousness (i.e., elevated gamma power and connectivity) after cardiac arrest (CA). This study examines electrocorticogram (ECoG) activity and coherence of the dying brain during asphyxial CA. Male Wistar rats (n = 16) were induced with isoflurane anesthesia, which was washed out before asphyxial CA. Mean phase coherence and ECoG power were compared during different stages of the asphyxial period to assess potential neural correlates of consciousness. After asphyxia, the ECoG progressed through four distinct stages (asphyxial stages 1–4 [AS1-4]), including a transient period of near-electrocerebral silence lasting several seconds (AS3). Electrocerebral silence (AS4) occurred within 1 min of the start of asphyxia, and pulseless electrical activity followed the start of AS4 by 1–2 min. AS3 was linked to a significant increase in frontal coherence between the left and right motor cortices (p < 0.05), with no corresponding increase in ECoG power. AS3 was also associated with a significant posterior shift of ECoG power, favoring the visual cortices (p < 0.05). Although the ECoG during AS3 appears visually flat or silent when viewed with standard clinical settings, our study suggests that this period of transient near-electrocerebral silence contains distinctive neural activity. Specifically, the burst in frontal coherence and posterior shift of ECoG power that we find during this period immediately preceding CA may be a neural correlate of conscious processing. PMID:28398813

  9. Cat scratch encephalopathy.

    PubMed

    Silver, B E; Bean, C S

    1991-06-01

    Cat scratch disease is usually benign, self-limited and without sequelae. Margileth has established four clinical criteria, three of which must be satisfied to make the diagnosis: 1) a history of animal exposure, usually kitten, with primary skin or ocular lesions; 2) regional chronic adenopathy without other apparent cause; 3) a positive cat scratch disease antigen skin test; and 4) lymph node biopsy demonstrating noncaseating granulomas and germinal center hyperplasia. Central nervous system involvement in cat scratch disease has been previously reported, although it is extremely uncommon. In a several-month period, we encountered two cases of cat scratch disease complicated by encephalopathy. The intents of this paper are twofold: 1) to briefly review the current literature on cat scratch disease, 2) to demonstrate that cat scratch disease complicated by encephalopathy presents acutely with seizures, posturing and coma and resolves rapidly with supportive care.

  10. Hypertensive encephalopathy and cerebral infarction.

    PubMed

    Edvardsson, Bengt

    2014-01-01

    Hypertensive encephalopathy is one cause of posterior reversible encephalopathy syndrome. Hypertensive encephalopathy and cerebral infarction have only been reported in a few individual case reports. A 51-year-old woman presented with hypertensive encephalopathy. T2-weighted images from magnetic resonance imaging showed hyperintense lesions in both occipital and parietal lobes. Diffusion-weighted imaging showed that this represented cytotoxic oedema and perfusion magnetic resonance imaging revealed reduced blood volume and flow. The magnetic resonance imaging was repeated 5 months later and subtotal regression of theT2-hyperintensity had occurred. However, small bilateral infarcts were seen on T1-weighted images. Perfusion magnetic resonance imaging presented reduced blood volume and flow on the right side. The patient in this report had posterior reversible encephalopathy syndrome caused by hypertensive encephalopathy. Magnetic resonance imaging of the brain showed bilateral cytotoxic oedema that partially resolved and resulted in small infarcts. The imaging findings are compatible with posterior reversible encephalopathy syndrome with subtotal resolution and infarct evolution. The case report suggests that the presence of hypertensive encephalopathy and posterior reversible encephalopathy syndrome should alert clinicians and lead to prompt treatment in order to prevent cerebral damage.

  11. [EEG manifestations in metabolic encephalopathy].

    PubMed

    Lin, Chou-Ching K

    2005-09-01

    Normal brain function depends on normal neuronal metabolism, which is closely related to systemic homeostasis of metabolites, such as glucose, electrolytes, amino acids and ammonia. "Metabolic encephalopathy" indicates diffuse brain dysfunction caused by various systemic derangements. Electroencephalogram (EEG) is widely used to evaluate metabolic encephalopathy since 1937, when Berger first observed slow brain activity induced by hypoglycemia. EEG is most useful in differentiating organic from psychiatric conditions, identifying epileptogenicity, and providing information about the degree of cortical or subcortical dysfunction. In metabolic encephalopathy, EEG evolution generally correlates well with the severity of encephalopathy. However, EEG has little specificity in differentiating etiologies in metabolic encephalopathy. For example, though triphasic waves are most frequently mentioned in hepatic encephalopathy, they can also be seen in uremic encephalopathy, or even in aged psychiatric patients treated with lithium. Spike-and-waves may appear in hyper- or hypo-glycemia, uremic encephalopathy, or vitamin deficiencies, etc. Common principles of EEG changes in metabolic encephalopathy are (1) varied degrees of slowing, (2) assorted mixtures of epileptic discharge, (3) high incidence of triphasic waves, and (4), as a rule, reversibility after treatment of underlying causes. There are some exceptions to the above descriptions in specific metabolic disorders and EEG manifestations are highly individualized.

  12. Determining oxygen consumption rate and asphyxiation point in Chanodichthys mongolicus using an improved respirometer chamber

    NASA Astrophysics Data System (ADS)

    Geng, Longwu; Jiang, Haifeng; Tong, Guangxiang; Xu, Wei

    2017-03-01

    Knowledge of oxygen consumption rates and asphyxiation points in fish is important to determine appropriate stocking and water quality management in aquaculture. The oxygen consumption rate and asphyxiation point in Chanodichthys mongolicus were detected under laboratory conditions using an improved respirometer chamber. The results revealed that more accurate estimates can be obtained by adjusting the volume of the respirometer chamber, which may avoid system errors caused by either repeatedly adjusting fish density or selecting different equipment specifications. The oxygen consumption rate and asphyxiation point of C. mongolicus increased with increasing water temperature and decreasing fish size. Changes in the C. mongolicus oxygen consumption rate were divided into three stages at water temperatures of 11-33°C: (1) a low temperature oxygen consumption rate stage when water temperature was 11-19°C, (2) the optimum temperature oxygen consumption rate stage when water temperature was 19-23°C, and (3) a high temperature oxygen consumption rate stage when water temperature was > 27°C. The temperature quotients (Q10) obtained suggested that C. mongolicus preferred a temperature range of 19-23°C. At 19°C, C. mongolicus exhibited higher oxygen consumption rates during the day when the maximum values were observed at 10:00 and 14:00 than at night when the minimum occurred at 02:00.

  13. Cerebral Sinovenous Thrombosis in the Asphyxiated Cooled Infants: A Prospective Observational Study.

    PubMed

    Radicioni, Maurizio; Bini, Vittorio; Chiarini, Pietro; Fantauzzi, Ambra; Leone, Francesca; Scattoni, Raffaella; Camerini, Pier Giorgio

    2017-01-01

    Cerebral sinovenous thrombosis is unusual in the asphyxiated cooled infants, but reliable data regarding the incidence of this comorbidity are lacking. We assessed the incidence of sinovenous thrombosis in a population of asphyxiated cooled infants by performing routine brain magnetic resonance venography. All asphyxiated infants who underwent therapeutic cooling at our institution completed brain magnetic resonance venography after rewarming. Assessing the incidence of cerebral sinovenous thrombosis was the primary goal. Secondary analyses included group comparisons for laboratory tests and monitored parameters, relationship between variables, logistic regression models, and receiver operating characteristic curve for cerebral sinovenous thrombosis prediction. Cerebral sinovenous thrombosis was detected in 10 of 37 infants (27%), most commonly affecting the superior sagittal sinus (eight of ten). These infants manifested higher blanket (P < 0.001) and lower esophageal temperatures (P = 0.006), lower platelet counts (P = 0.045), and received more red blood cell transfusions (P = 0.038) than the cooled infants without thrombosis. Blanket temperature was independently associated with cerebral sinovenous thrombosis (P = 0.049), and 32°C/hour was the optimal cutoff value to predict the event (sensitivity, 90%; specificity, 88.5%). High incidence or cerebral sinovenous thrombosis in neonates treated with therapeutic hypothermia suggests that magnetic resonance venography may be reasonable in many of these children. High blanket temperature may be one variable that helps identify patients at higher risk. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Determining oxygen consumption rate and asphyxiation point in Chanodichthys mongolicus using an improved respirometer chamber

    NASA Astrophysics Data System (ADS)

    Geng, Longwu; Jiang, Haifeng; Tong, Guangxiang; Xu, Wei

    2016-05-01

    Knowledge of oxygen consumption rates and asphyxiation points in fish is important to determine appropriate stocking and water quality management in aquaculture. The oxygen consumption rate and asphyxiation point in Chanodichthys mongolicus were detected under laboratory conditions using an improved respirometer chamber. The results revealed that more accurate estimates can be obtained by adjusting the volume of the respirometer chamber, which may avoid system errors caused by either repeatedly adjusting fish density or selecting different equipment specifications. The oxygen consumption rate and asphyxiation point of C. mongolicus increased with increasing water temperature and decreasing fish size. Changes in the C. mongolicus oxygen consumption rate were divided into three stages at water temperatures of 11-33°C: (1) a low temperature oxygen consumption rate stage when water temperature was 11-19°C, (2) the optimum temperature oxygen consumption rate stage when water temperature was 19-23°C, and (3) a high temperature oxygen consumption rate stage when water temperature was > 27°C. The temperature quotients (Q10) obtained suggested that C. mongolicus preferred a temperature range of 19-23°C. At 19°C, C. mongolicus exhibited higher oxygen consumption rates during the day when the maximum values were observed at 10:00 and 14:00 than at night when the minimum occurred at 02:00.

  15. Hashimoto encephalopathy: literature review.

    PubMed

    Zhou, J Y; Xu, B; Lopes, J; Blamoun, J; Li, L

    2017-03-01

    Hashimoto encephalopathy (HE) presents as an encephalopathy without central nervous system infection or tumor. HE is associated with autoimmune thyroiditis and is thus considered to be an autoimmune disorder. The prevalence of HE is low, but death and status epilepticus have been reported. HE manifests with a wide range of symptoms that include behavioral changes and confusion. Elevated thyroid antibodies are present in the majority of cases and are required for the diagnosis of HE. Normal brain MRI findings are found in the majority of patients diagnosed with HE. The most consistent CSF abnormality noted in HE patients is the presence of elevated protein. Most HE patients respond well to steroid therapy. Clinical improvements are also observed with IV immunoglobulin and plasmapheresis. In conclusion, it is now generally accepted that the diagnosis of HE must include encephalopathy characterized by cognitive impairment associated with psychiatric features, such as hallucinations, delusions, and paranoia. Autoimmune encephalitis and prion disease should be considered in the differential diagnosis due to the similarity of the clinical features of these conditions to those of HE. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Pharmacoeconomics of hepatic encephalopathy.

    PubMed

    Neff, Guy

    2010-05-01

    Understanding and appreciating the science of pharmacoeconomics have become even more important for health care providers and insurers during the recent economic downturn. Evaluating the true costs of any disease is complex; both direct costs, such as costs of drug therapy and the provision of care, and indirect costs, such as lost earnings and reduced quality of life, must be taken into account. With chronic liver disease, the most recent data indicate that direct costs were more than $2 billion whereas indirect costs were more than $450 million. Hepatic encephalopathy, a common complication of chronic liver disease, contributes to this economic burden. Although patients' length of stay during hospitalization for hepatic encephalopathy decreased from almost 9 days to 6 days (and has remained stable over the past few years) from 1993 to 2007, hospitalization costs rose from $13,000 to $30,000/hospital stay. In addition, 22% of patients were discharged directly to nursing homes or rehabilitation centers, which increases total costs. When assessing therapy for hepatic encephalopathy, it is important to consider the total costs of the disease, not just treatment costs. Although more expensive on a daily basis than lactulose, rifaximin has been shown to reduce hospitalization rates, has a better adverse-effect profile, and increases patient compliance. One study found that rifaximin produced a cost savings/patient/year of more than $3000 over lactulose therapy.

  17. [Recommendations for perinatal transport].

    PubMed

    Esqué Ruiz, M; Figueras Aloy, J; García Alix, A; Alomar Ribes, A; Blanco Bravo, D; Ferández Lorenzo, J R

    2001-08-01

    Perinatal transport should be integrated into a system of perinatal care within a regional health care program and should be planned according to the healthcare map of each community. We describe the various types of transport, their advantages and disadvantages, the resources required, and the protocol that should be followed in perinatal transfer. We highlight the importance of maternal and neonatal transport. The organization of transfers receives special attention, and we discuss the different functions of the coordinating, referral and receiving centers as well as those of the transport assistance team. We also discuss ethical-legal questions.

  18. Perinatal loss among twins.

    PubMed

    Lynch, Anne; McDuffie, Robert; Lyons, Ella; Chase, Mary; Orleans, Miriam

    2007-01-01

    We evaluated prenatal factors related to perinatal loss in twins, using medical records and death certificates, to determine the main perinatal event that contributed to babies' deaths. This was a retrospective cohort study of 550 monochorionic diamniotic or diamniotic dichorionic twins who were delivered at Kaiser Permanente Colorado between 1994 and 2001. The main outcome of the study was perinatal loss (stillbirth or neonatal death). Select maternal risk factors (maternal age, race, marital status, assisted conception, past history of preterm birth, cigarette smoking, and placentation) were included in the univariable and multivariable logistic regression analysis. Data on these risk factors came from review of records from our multiple-birth perinatal database. A comprehensive review of clinical events recorded in the medical records and on the death certificate was conducted to assess the main event that contributed to the loss. In the cohort of 1100 babies, there were 12 stillbirths and 34 neonatal deaths, with an overall frequency of perinatal loss of 4.2%. We found a strong association between a monochorionic diamniotic placentation and perinatal loss (adjusted odds ratio, 3.9; 95% confidence interval, 2, 7.7). At delivery, placental pathology and spontaneous preterm birth accounted for 36% and 41%, respectively, of the clinical events contributing to the demises. Compared with the medical record, review of death certificate information did not contribute significantly to the understanding of the sequence of perinatal events leading to the demise. We conclude that loss in twins is most strongly associated with monochorionic diamniotic placentation. Although this condition is not preventable, early identification (by ultrasound) and referral to subspecialists may decrease the chances of perinatal loss. Prevention of spontaneous preterm birth in all women remains an important initiative in obstetric care to reduce perinatal mortality and neonatal morbidity

  19. Prediabetes and perinatal mortality.

    PubMed

    Wood, S L; Sauve, R; Ross, S; Brant, R; Love, E J

    2000-12-01

    The association between gestational diabetes mellitus (GDM) and perinatal outcome is largely based on case series and retrospective studies that found an increased risk of perinatal mortality and stillbirth as the onset of diabetes approached. Our objective was to assess the relationship between latency to diabetes and perinatal outcome of prediabetic pregnancies in a contemporary population of women with adult-onset diabetes. A population of 403 diabetic women from two recruitment sites completed a pretested questionnaire. Details of 1,181 pregnancy outcomes were obtained. This comprised 1,024 live births, 22 stillbirths, and 8 early neonatal deaths. Crude analysis suggested a relationship between time to diabetes (latency) < or =20 years and both perinatal death and stillbirth: odds ratio (95% CI), 2.41 (1.17-4.95) and 2.15 (0.93-4.98). Generalized additive modeling revealed a nonlinear relationship between the variables time to diabetes, and maternal age and perinatal outcome. Final logistic regression analysis was then performed for the outcomes perinatal death and stillbirth, with maternal age as a second-degree polynomial, year of birth as a continuous variable, and time to diabetes dichotomized < or =20 years to diagnosis and >20 years. This final analysis documented a significant association between time to diabetes < or =20 years and both perinatal death (4.06 [1.79-9.36]) and stillbirth (3.35 [1.25-9.05]). There appeared to be an increased risk of perinatal death and stillbirth in pregnancies occurring in the last 20 years before the diagnosis of diabetes.

  20. Newborns Referred for Therapeutic Hypothermia: Association between Initial Degree of Encephalopathy and Severity of Brain Injury (What About the Newborns with Mild Encephalopathy on Admission?).

    PubMed

    Gagne-Loranger, Maude; Sheppard, Megan; Ali, Nabeel; Saint-Martin, Christine; Wintermark, Pia

    2016-01-01

    The aim of this article was to describe the severity of brain injury and/or mortality in a cohort of newborns referred for therapeutic hypothermia, in relation to the degree of encephalopathy on admission, and to especially look at the ones with initial mild encephalopathy. Term newborns with perinatal depression referred to our neonatal intensive care unit for possible hypothermia treatment from 2008 to 2012 were enrolled prospectively. The modified Sarnat score on admission was correlated with severity of brain injury on brain imaging and/or autopsy. A total of 215 newborns were referred for possible cooling. Sixty percent (128/215) were cooled. Most of the not-cooled newborns with an available brain magnetic resonance imaging (85% = 50/59) had an initial mild encephalopathy, and 40% (20/50) developed brain injury. Some cooled newborns had an initial mild encephalopathy (12% = 13/108); only 31% (4/13) developed brain injury. Our results demonstrated that several newborns with an initial mild encephalopathy developed subsequent brain injury, especially when they were not cooled. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  1. [Posterior reversible encephalopathy syndrome].

    PubMed

    Petrović, Branko; Kostić, Vladimir; Sternić, Nadezda; Kolar, Jovo; Tasić, Nebojsa

    2003-01-01

    Reversible Posterior Leukoencephalopathy Syndrome was introduced into clinical practice in 1996 in order to describe unique syndrome, clinically expressed during hypertensive and uremic encephalopathy, eclampsia and during immunosuppressive therapy [1]. First clinical investigations showed that leucoencephalopathy is major characteristic of the syndrome, but further investigations showed no significant destruction in white cerebral tissue [2, 3, 4]. In majority of cases changes are localise in posterior irrigation area of the brain and in the most severe cases anterior region is also involved. Taking into consideration all above mentioned facts, the suggested term was Posterior Reversible Encephalopathy Syndrome (PRES) for the syndrome clinically expressed by neurological manifestations derived from cortical and subcortical changes localised in posterior regions of cerebral hemispheres, cerebral trunk and cerebellum [5]. Patient, aged 53 years, was re-hospitalized in Cardiovascular Institute "Dediwe" two months after successful aorto-coronary bypass performed in June 2001 due to the chest bone infection. During the treatment of the infection (according to the antibiogram) in September 2001, patient in evening hours developed headache and blurred vision. The recorded blood pressure was 210/120 mmHg so antihypertensive treatment was applied (Nifedipin and Furosemid). After this therapy there was no improvement and intensive headache with fatigue and loss of vision developed. Neurological examination revealed cortical blindness and left hemiparesis. Manitol (20%, 60 ccm every 3 hours) and i.v. Nytroglicerin (high blood pressure). Brain CT revealed oedema of parieto-occipital regions of both hemispheres, more emphasized on the right. (Figure 1a, b, c). There was no sign of focal ischemia even in deeper sections (Figure 1d, e, f). Following three days enormous high blood pressure values were registered. On the fourth day the significant clinical improvement occurred

  2. SCN2A encephalopathy

    PubMed Central

    Howell, Katherine B.; McMahon, Jacinta M.; Carvill, Gemma L.; Tambunan, Dimira; Mackay, Mark T.; Rodriguez-Casero, Victoria; Webster, Richard; Clark, Damian; Freeman, Jeremy L.; Calvert, Sophie; Olson, Heather E.; Mandelstam, Simone; Poduri, Annapurna; Mefford, Heather C.; Harvey, A. Simon

    2015-01-01

    Objective: De novo SCN2A mutations have recently been associated with severe infantile-onset epilepsies. Herein, we define the phenotypic spectrum of SCN2A encephalopathy. Methods: Twelve patients with an SCN2A epileptic encephalopathy underwent electroclinical phenotyping. Results: Patients were aged 0.7 to 22 years; 3 were deceased. Seizures commenced on day 1–4 in 8, week 2–6 in 2, and after 1 year in 2. Characteristic features included clusters of brief focal seizures with multiple hourly (9 patients), multiple daily (2), or multiple weekly (1) seizures, peaking at maximal frequency within 3 months of onset. Multifocal interictal epileptiform discharges were seen in all. Three of 12 patients had infantile spasms. The epileptic syndrome at presentation was epilepsy of infancy with migrating focal seizures (EIMFS) in 7 and Ohtahara syndrome in 2. Nine patients had improved seizure control with sodium channel blockers including supratherapeutic or high therapeutic phenytoin levels in 5. Eight had severe to profound developmental impairment. Other features included movement disorders (10), axial hypotonia (11) with intermittent or persistent appendicular spasticity, early handedness, and severe gastrointestinal symptoms. Mutations arose de novo in 11 patients; paternal DNA was unavailable in one. Conclusions: Review of our 12 and 34 other reported cases of SCN2A encephalopathy suggests 3 phenotypes: neonatal-infantile–onset groups with severe and intermediate outcomes, and a childhood-onset group. Here, we show that SCN2A is the second most common cause of EIMFS and, importantly, does not always have a poor developmental outcome. Sodium channel blockers, particularly phenytoin, may improve seizure control. PMID:26291284

  3. Pathophysiology of epileptic encephalopathies.

    PubMed

    Lado, Fred A; Rubboli, Guido; Capovilla, Giuseppe; Capovilla, Pippo; Avanzini, Giuliano; Moshé, Solomon L

    2013-11-01

    The application of metabolic imaging and genetic analysis, and now the development of appropriate animal models, has generated critical insights into the pathogenesis of epileptic encephalopathies. In this article we present ideas intended to move from the lesions associated with epileptic encephalopathies toward understanding the effects of these lesions on the functioning of the brain, specifically of the cortex. We argue that the effects of focal lesions may be magnified through the interaction between cortical and subcortical structures, and that disruption of subcortical arousal centers that regulate cortex early in life may lead to alterations of intracortical synapses that affect a critical period of cognitive development. Impairment of interneuronal function globally through the action of a genetic lesion similarly causes widespread cortical dysfunction manifesting as increased delta slow waves on electroencephalography (EEG) and as developmental delay or arrest clinically. Finally, prolonged focal epileptic activity during sleep (as occurring in the syndrome of continuous spike-wave in slow sleep, or CSWSS) might interfere with local slow wave activity at the site of the epileptic focus, thereby impairing the neural processes and, possibly, the local plastic changes associated with learning and other cognitive functions. Seizures may certainly add to these pathologic processes, but they are likely not necessary for the development of the cognitive pathology. Nevertheless, although seizures may be either a consequence or symptom of the underlying lesion, their effective treatment can improve outcomes as both clinical and experimental studies may suggest. Understanding their substrates may lead to novel, effective treatments for all aspects of the epileptic encephalopathy phenotype.

  4. Hypertension and hypertensive encephalopathy.

    PubMed

    Price, Raymond S; Kasner, Scott E

    2014-01-01

    The definition of hypertension has continuously evolved over the last 50 years. Hypertension is currently defined as a blood pressure greater than 140/90mmHg. One in every four people in the US has been diagnosed with hypertension. The prevalence of hypertension increases further with age, affecting 75% of people over the age of 70. Hypertension is by far the most common risk factor identified in stroke patients. Hypertension causes pathologic changes in the walls of small (diameter<300 microns) arteries and arterioles usually at short branches of major arteries, which may result in either ischemic stroke or intracerebral hemorrhage. Reduction of blood pressure with diuretics, β-blockers, calcium channel blockers, and angiotensin-converting enzyme (ACE) inhibitors have all been shown to markedly reduce the incidence of stroke. Hypertensive emergency is defined as a blood pressure greater than 180/120mmHg with end organ dysfunction, such as chest pain, shortness of breath, encephalopathy, or focal neurologic deficits. Hypertensive encephalopathy is believed to be caused by acute failure of cerebrovascular autoregulation. Hypertensive emergency is treated with intravenous antihypertensive agents to reduce blood pressure by 25% within the first hour. Selective inhibition of cerebrovascular blood vessel permeability for the treatment of hypertensive emergency is beginning early clinical trials. © 2014 Elsevier B.V. All rights reserved.

  5. Chronic traumatic encephalopathy.

    PubMed

    Omalu, Bennet

    2014-01-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative syndrome, which is caused by single, episodic, or repetitive blunt force impacts to the head and transfer of acceleration-deceleration forces to the brain. CTE presents clinically as a composite syndrome of mood disorders and behavioral and cognitive impairment, with or without sensorimotor impairment. Symptoms of CTE may begin with persistent symptoms of acute traumatic brain injury (TBI) following a documented episode of brain trauma or after a latent period that may range from days to weeks to months and years, up to 40 years following a documented episode of brain trauma or cessation of repetitive TBI. Posttraumatic encephalopathy is distinct from CTE, can be comorbid with CTE, and is a clinicopathologic syndrome induced by focal and/or diffuse, gross and/or microscopic destruction of brain tissue following brain trauma. The brain of a CTE sufferer may appear grossly unremarkable, but shows microscopic evidence of primary and secondary proteinopathies. The primary proteinopathy of CTE is tauopathy, while secondary proteinopathies may include, but are not limited to, amyloidopathy and TDP proteinopathy. Reported prevalence rates of CTE in cohorts exposed to TBI ranges from 3 to 80% across age groups.

  6. Perinatal cyanosis: long-term cognitive sequelae and behavioral consequences.

    PubMed

    Perna, Robert; Cooper, David

    2012-01-01

    Research suggests that serious perinatal asphyxia leading to long-term neurological consequences occurs in 1 to 6 out of every 1,000 newborns (Barkovich et al., 1998 ; Mcguire, 2007 ). In serious cases, encephalopathy follows the asphyxia and resultant hypoxia, leading to additional insult to the brain. The effects of brief or transient hypoxia and cyanosis have not been well researched. This study involved comparing children who had a brief perinatal episode to those who have not. The research hypothesis is that those children who have experienced a brief perinatal cyanotic episode will subsequently have cognitive and behavioral issues during childhood that will be measurable on neuropsychological testing or result in increased clinical diagnoses. A sample (N = 52) of school-aged children (M(age) = 10.5 years) was divided into those who had had a brief perinatal cyanotic episode (n = 14) and those who had not (n = 38). On neuropsychological testing, data from the tests administered did not suggest any negative effects of a brief cyanotic episode. The cyanotic group was significantly more likely to have a developmental disorder (speech or motor delay) and subsequently be diagnosed with attention-deficit hyperactivity disorder (ADHD). Given the high incidence of ADHD in the cyanotic group, it may be reasonable to construe cyanosis as a risk factor.

  7. Pulmonary hemodynamics and vascular reactivity in asphyxiated term lambs resuscitated with 21 and 100% oxygen

    PubMed Central

    Steinhorn, Robin H.; Wedgwood, Stephen; Savorgnan, Fabio; Nair, Jayasree; Mathew, Bobby; Gugino, Sylvia F.; Russell, James A.; Swartz, Daniel D.

    2011-01-01

    An increase in oxygen tension is an important factor in decreasing pulmonary vascular resistance (PVR) at birth. Birth asphyxia results in acidosis and increased PVR. We determined the effect of resuscitation with 21 vs. 100% O2 on pulmonary hemodynamics, pulmonary arterial (PA) reactivity, and oxidant stress in a lamb model of in utero asphyxia. Term fetal lambs were acutely asphyxiated by intrauterine umbilical cord occlusion for 10 min resulting in acidosis (pH 6.96 ± 0.05 and Pco2 103 ± 5 Torr), bradycardia, systemic hypotension, and increased PVR. Lambs were treated with 30 min of resuscitation with 21% or 100% O2 (n = 6 each). PaO2 was significantly elevated with 100% O2 resuscitation compared with 21% O2 (430 ± 38 vs. 64 ± 8 Torr), but changes in pH and PaCO2 were similar. The 100% O2 induced greater increase in pulmonary blood flow and decrease in PVR at 1 min of life, but subsequent values were similar to 21% O2 group between 2 and 30 min of life. Oxygen uptake from the lung and systemic oxygen extraction was similar between the two groups. Pulmonary arteries showed increased staining for superoxide anions and increased contractility to norepinephrine following resuscitation with 100% O2. The increased PA contractility induced by 100% O2 was reversed by scavenging superoxide anions with superoxide dismutase and catalase. We conclude that resuscitation of asphyxiated lambs with 100% O2 increases PaO2 but does not improve lung oxygen uptake, decrease PVR at 30 min, or increase systemic oxygen extraction ratios. Furthermore, 100% O2 also induces oxidative stress and increases PA contractility. These findings support the new neonatal resuscitation guidelines recommending 21% O2 for initial resuscitation of asphyxiated neonates. PMID:21799125

  8. Perinatal chikungunya in twins

    PubMed Central

    Karthiga, Vikneswari; Kommu, Peter Prasanth Kumar; Krishnan, Lalitha

    2016-01-01

    We report a case of vertically transmitted chikungunya infection in heterozygous twin neonates presenting as seizures, encephalopathy, midfacial hyperpigmentation, anemia, and thrombocytopenia. This could be considered as a rare cause of neonatal seizure and identification would help in appropriate management. PMID:27857791

  9. [Effects of temperature and salinity on oxygen consumption rate and asphyxiation point of Sagitta crassa].

    PubMed

    Liu, Qing; Zhu, Hai-Yan; Liu, Fang; Ding, Zi-Yuan

    2011-11-01

    A laboratory test was conducted to study the effects of different temperature and salinity on the oxygen consumption rate and asphyxiation point of chaetognath Sagitta crassa. Both temperature and salinity had significant effects on the oxygen consumption rate (IO) and specific oxygen consumption rate (SO) of S. crassa. When the temperature raised from 5 degrees C to 25 degrees C, the IO and SO of S. crassa increased first, and then presented an obvious decreasing trend, with the regression function being y = 0.0058x3-0.2956x2 +4.415x-8.7816 (R2 = 0.99, P < 0.05) for IO and y = 0.0011x3-0.0546x2+0.8161x-1.6232 (R2 = 0.99, P < 0.05) for SO. The IO and SO at different temperature were in the ranges of 6.30-11.71 microg x ind(-1) x h(-1) and 1.22-2.16 microg x mg(-1) x h(-1), respectively, and the asphyxiation point was 4.18-6.87 mg x L(-1). When the salinity increased from 10 to 40, the IO and SO of S. crassa decreased gradually, with the regression function being y = -0.0068x2-0.1412x+21.702 (R2 = 0.89, P < 0.05) for IO and y = -0.0013x2 -0.0261x+ 4.0114 (R2 = 0.89, P < 0.05) for SO. The IO and SO at different salinity were in the ranges of 4.98-17.73 microg x ind(-1) x h(-1) and 0.92-3.56 microg x mg(-1) x h(-1), respectively, and the asphyxiation point was 4.02-6.24 mg x L(-1). Based on the differences in the oxygen consumption rate and asphyxiation point between S. crassa and other aquatic animals, it was concluded that S. crassa was a stenooxybiotic zooplankton species.

  10. TCTEX1D2 mutations underlie Jeune asphyxiating thoracic dystrophy with impaired retrograde intraflagellar transport

    PubMed Central

    Schmidts, Miriam; Hou, Yuqing; Cortés, Claudio R.; Mans, Dorus A.; Huber, Celine; Boldt, Karsten; Patel, Mitali; van Reeuwijk, Jeroen; Plaza, Jean-Marc; van Beersum, Sylvia E. C.; Yap, Zhi Min; Letteboer, Stef J. F.; Taylor, S. Paige; Herridge, Warren; Johnson, Colin A.; Scambler, Peter J.; Ueffing, Marius; Kayserili, Hulya; Krakow, Deborah; King, Stephen M.; Beales, Philip L.; Al-Gazali, Lihadh; Wicking, Carol; Cormier-Daire, Valerie; Roepman, Ronald; Mitchison, Hannah M.; Witman, George B.; Al-Turki, Saeed; Anderson, Carl; Anney, Richard; Antony, Dinu; Asimit, Jennifer; Ayub, Mohammad; Barrett, Jeff; Barroso, Inês; Bentham, Jamie; Bhattacharya, Shoumo; Blackwood, Douglas; Bobrow, Martin; Bochukova, Elena; Bolton, Patrick; Boustred, Chris; Breen, Gerome; Brion, Marie-Jo; Brown, Andrew; Calissano, Mattia; Carss, Keren; Chatterjee, Krishna; Chen, Lu; Cirak, Sebhattin; Clapham, Peter; Clement, Gail; Coates, Guy; Collier, David; Cosgrove, Catherine; Cox, Tony; Craddock, Nick; Crooks, Lucy; Curran, Sarah; Daly, Allan; Danecek, Petr; Smith, George Davey; Day-Williams, Aaron; Day, Ian; Durbin, Richard; Edkins, Sarah; Ellis, Peter; Evans, David; Farooqi, I. Sadaf; Fatemifar, Ghazaleh; Fitzpatrick, David; Flicek, Paul; Floyd, Jamie; Foley, A. Reghan; Franklin, Chris; Futema, Marta; Gallagher, Louise; Gaunt, Tom; Geschwind, Daniel; Greenwood, Celia; Grozeva, Detelina; Guo, Xiaosen; Gurling, Hugh; Hart, Deborah; Hendricks, Audrey; Holmans, Peter; Huang, Jie; Humphries, Steve E.; Hurles, Matt; Hysi, Pirro; Jackson, David; Jamshidi, Yalda; Jewell, David; Chris, Joyce; Kaye, Jane; Keane, Thomas; Kemp, John; Kennedy, Karen; Kent, Alastair; Kolb-Kokocinski, Anja; Lachance, Genevieve; Langford, Cordelia; Lee, Irene; Li, Rui; Li, Yingrui; Ryan, Liu; Lönnqvist, Jouko; Lopes, Margarida; MacArthur, Daniel G.; Massimo, Mangino; Marchini, Jonathan; Maslen, John; McCarthy, Shane; McGuffin, Peter; McIntosh, Andrew; McKechanie, Andrew; McQuillin, Andrew; Memari, Yasin; Metrustry, Sarah; Min, Josine; Moayyeri, Alireza; Morris, James; Muddyman, Dawn; Muntoni, Francesco; Northstone, Kate; O'Donovan, Michael; O'Rahilly, Stephen; Onoufriadis, Alexandros; Oualkacha, Karim; Owen, Michael; Palotie, Aarno; Panoutsopoulou, Kalliope; Parker, Victoria; Parr, Jeremy; Paternoster, Lavinia; Paunio, Tiina; Payne, Felicity; Perry, John; Pietilainen, Olli; Plagnol, Vincent; Quail, Michael A.; Quaye, Lydia; Raymond, Lucy; Rehnström, Karola; Brent Richards, J.; Ring, Sue; Ritchie, Graham R S; Savage, David B.; Schoenmakers, Nadia; Semple, Robert K.; Serra, Eva; Shihab, Hashem; Shin, So-Youn; Skuse, David; Small, Kerrin; Smee, Carol; Soler, Artigas María; Soranzo, Nicole; Southam, Lorraine; Spector, Tim; St Pourcain, Beate; St. Clair, David; Stalker, Jim; Surdulescu, Gabriela; Suvisaari, Jaana; Tachmazidou, Ioanna; Tian, Jing; Timpson, Nic; Tobin, Martin; Valdes, Ana; van Kogelenberg, Margriet; Vijayarangakannan, Parthiban; Wain, Louise; Walter, Klaudia; Wang, Jun; Ward, Kirsten; Wheeler, Ellie; Whittall, Ros; Williams, Hywel; Williamson, Kathy; Wilson, Scott G.; Wong, Kim; Whyte, Tamieka; ChangJiang, Xu; Zeggini, Eleftheria; Zhang, Feng; Zheng, Hou-Feng

    2015-01-01

    The analysis of individuals with ciliary chondrodysplasias can shed light on sensitive mechanisms controlling ciliogenesis and cell signalling that are essential to embryonic development and survival. Here we identify TCTEX1D2 mutations causing Jeune asphyxiating thoracic dystrophy with partially penetrant inheritance. Loss of TCTEX1D2 impairs retrograde intraflagellar transport (IFT) in humans and the protist Chlamydomonas, accompanied by destabilization of the retrograde IFT dynein motor. We thus define TCTEX1D2 as an integral component of the evolutionarily conserved retrograde IFT machinery. In complex with several IFT dynein light chains, it is required for correct vertebrate skeletal formation but may be functionally redundant under certain conditions. PMID:26044572

  11. Treatment of epileptic encephalopathies.

    PubMed

    McTague, Amy; Cross, J Helen

    2013-03-01

    Epileptic encephalopathy is defined as a condition where the epileptic activity itself may contribute to the severe neurological and cognitive impairment seen, over and above that which would be expected from the underlying pathology alone. The epilepsy syndromes at high risk of this are a disparate group of conditions characterized by epileptic seizures that are difficult to treat and developmental delay. In this review, we discuss the ongoing debate regarding the significance of inter-ictal discharges and the impact of the seizures themselves on the cognitive delay or regression that is a common feature of these syndromes. The syndromes also differ in many ways and we provide a summary of the key features of the early-onset epileptic encephalopathies including Ohtahara and West syndromes in addition to later childhood-onset syndromes such as Lennox Gastaut and Doose syndromes. An understanding of the various severe epilepsy syndromes is vital to understanding the rationale for treatment. For example, the resolution of hypsarrhythmia in West syndrome is associated with an improvement in cognitive outcome and drives treatment choice, but the same cannot be applied to frequent inter-ictal discharges in Lennox Gastaut syndrome. We discuss the evidence base for treatment where it is available and describe current practice where it is not. For example, in West syndrome there is some evidence for preference of hormonal treatments over vigabatrin, although the choice and duration of hormonal treatment remains unclear. We describe the use of conventional and newer anti-epileptic medications in the various syndromes and discuss which medications should be avoided. Older possibly forgotten treatments such as sulthiame and potassium bromide also have a role in the severe epilepsies of childhood. We discuss hormonal treatment with particular focus on the treatment of West syndrome, continuous spike wave in slow wave sleep (CSWS)/electrical status epilepticus in slow wave

  12. Pregnancy & perinatal transmission update.

    PubMed

    Denison, R

    1998-09-01

    According to a June 1998 report from UNAIDS, the majority of children infected with HIV acquired it from their mothers during or near birth. One way to prevent perinatal transmission of HIV is to increase prevention efforts for women. Other ways to prevent perinatal transmission include using AZT treatment, avoiding breastfeeding, and choosing a C-section delivery instead of a vaginal delivery. One important study, called the Thai study, promoted a shorter course of AZT therapy that was less expensive, more accessible, and still prevented transmission in many cases. Several reasons are cited for why some women continue breastfeeding, despite the increased risk of transmitting HIV to their babies. An important factor in preventing perinatal transmission is the overall health of the mother, and her ability to maintain her health and treatment regimen while caring for a newborn.

  13. Neurological Abnormalities in Full-Term Asphyxiated Newborns and Salivary S100B Testing: The “Cooperative Multitask against Brain Injury of Neonates” (CoMBINe) International Study

    PubMed Central

    Gazzolo, Diego; Pluchinotta, Francesca; Bashir, Moataza; Aboulgar, Hanna; Said, Hala Mufeed; Iman, Iskander; Ivani, Giorgio; Conio, Alessandra; Tina, Lucia Gabriella; Nigro, Francesco; Li Volti, Giovanni; Galvano, Fabio; Michetti, Fabrizio; Di Iorio, Romolo; Marinoni, Emanuela; Zimmermann, Luc J.; Gavilanes, Antonio D. W.; Vles, Hans J. S.; Kornacka, Maria; Gruszfeld, Darek; Frulio, Rosanna; Sacchi, Renata; Ciotti, Sabina; Risso, Francesco M.; Sannia, Andrea; Florio, Pasquale

    2015-01-01

    Background Perinatal asphyxia (PA) is a leading cause of mortality and morbidity in newborns: its prognosis depends both on the severity of the asphyxia and on the immediate resuscitation to restore oxygen supply and blood circulation. Therefore, we investigated whether measurement of S100B, a consolidated marker of brain injury, in salivary fluid of PA newborns may constitute a useful tool for the early detection of asphyxia-related brain injury. Methods We conducted a cross-sectional study in 292 full-term newborns admitted to our NICUs, of whom 48 suffered PA and 244 healthy controls admitted at our NICUs. Saliva S100B levels measurement longitudinally after birth; routine laboratory variables, neurological patterns, cerebral ultrasound and, magnetic resonance imaging were performed. The primary end-point was the presence of neurological abnormalities at 12-months after birth. Results S100B salivary levels were significantly (P<0.001) higher in newborns with PA than in normal infants. When asphyxiated infants were subdivided according to a good (Group A; n = 15) or poor (Group B; n = 33) neurological outcome at 12-months, S100B was significantly higher at all monitoring time-points in Group B than in Group A or controls (P<0.001, for all). A cut-off >3.25 MoM S100B achieved a sensitivity of 100% (CI5-95%: 89.3%-100%) and a specificity of 100% (CI5-95%: 98.6%-100%) as a single marker for predicting the occurrence of abnormal neurological outcome (area under the ROC curve: 1.000; CI5-95%: 0.987-1.0). Conclusions S100B protein measurement in saliva, soon after birth, is a useful tool to identify which asphyxiated infants are at risk of neurological sequelae. PMID:25569796

  14. [Prevention of hepatic encephalopathy].

    PubMed

    Morillas, Rosa M; Sala, Marga; Planas, Ramon

    2014-06-06

    Hepatic encephalopathy (HE) is a frequent complication of cirrhosis which, in addition to producing a great social impact, deteriorates the quality of life of patients and is considered a sign of advanced liver disease and therefore a clinical indication for liver transplant evaluation. Patients who have had episodes of HE have a high risk of recurrence. Thus, after the HE episode resolves, it is recommended: control and prevention of precipitating factors (gastrointestinal bleeding, spontaneous bacterial peritonitis, use of diuretics with caution, avoid nervous system depressant medications), continued administration of non-absorbable disaccharides such as lactulose or lactitol, few or non-absorbable antibiotics such as rifaximin and assess the need for a liver transplant as the presence of a HE episode carries a poor prognosis in cirrhosis. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  15. "Symptomatic" infection-associated acute encephalopathy in children with underlying neurological disorders.

    PubMed

    Hirayama, Yoshimichi; Saito, Yoshiaki; Maegaki, Yoshihiro

    2017-03-01

    Development of infection-associated acute encephalopathy (AE) is precipitated by several factors, including viral agents, age, and genetic polymorphisms. In addition, children with prior underlying neurological disorders can also present with AE. We reviewed 55 children with AE who were referred to hospitals participating in the Status Epilepticus Study Group from 1988 to 2013. AE was classified into eight subtypes: acute encephalopathy with biphasic seizures and late reduced diffusion (AESD); hemiconvulsion-hemiplegia syndrome (HH); acute necrotizing encephalopathy; hemorrhagic shock and encephalopathy syndrome (HSES); clinically mild encephalitis/encephalopathy with a reversible splenial lesion; acute encephalitis with refractory, repetitive partial seizures; Reye-like syndrome; and unclassified. Of the 55 AE cases, 14 (25.4%) had underlying neurological disorders, including perinatal insults (n=6) and genetic syndrome and/or brain malformations (n=8). These preceding morbidities were relatively common in AESD (6/18, 33.3%), HH (3/9, 33.3%), and HSES (3/6, 50.0%). History of epilepsy or febrile seizures were frequent in HH cases (4/9, 44.4%), whereas they were rare in other AE subtypes. Among the AE subgroups, HH, HSES, and AESD frequently emerged in preceding etiologies with augmented neuronal excitability. These subgroups may have distinct pathomechanism from the "cytokine storm" mediated AEs during childhood. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  16. Feasibility and Safety of Therapeutic Hypothermia and Short Term Outcome in Neonates with Hypoxic Ischemic Encephalopathy.

    PubMed

    Purkayastha, Jayashree; Lewis, Leslie Edward; Bhat, Ramesh Y; Anusha, K M

    2016-02-01

    Therapeutic hypothermia is well known for neuroprotection in asphyxiated neonates with hypoxic ischemic encephalopathy. The authors aimed to study the feasibility and safety of therapeutic hypothermia and short term outcome in neonates with hypoxic ischemic encephalopathy (HIE). Total 31 neonates with moderate to severe HIE were enrolled in the study. Continuous temperature recording was noted in 31 neonates; 17 neonates were studied prospectively while 14 neonates were studied retrospectively. Rectal temperature was monitored in 31 neonates and maintained between 33 and 34 °C by switching off the warmer and using ice packs. Reusable ice packs were used which were inexpensive. Therapeutic hypothermia was maintained for 72 h and babies were then rewarmed 0.5 °C every hour. Therapeutic hypothermia was feasible and inexpensive. There was no major complication during the study. MRI was done in 17 neonates; 52 % were found to have normal MRI at the end of first week. Among the study neonates (n = 31) 64.5 % were neurologically normal at the time of discharge. To conclude, therapeutic hypothermia is feasible in a low resource setting and is a safe way of neuroprotection. Short term outcome was also favourable in these neonates.

  17. Gasoline sniffing and lead encephalopathy.

    PubMed Central

    Ross, C. A.

    1982-01-01

    Gasoline sniffing is endemic in northern Manitoba and perhaps throughout much of northern Canada. Its most serious complication is lead encephalopathy, which can be fatal. Most of the toxic effects are thought to be due to tetraethyl lead and its metabolites. The specific treatment is chelation therapy, for which a protocol has been developed at the Health Sciences Centre, Winnipeg. Lead encephalopathy, however, is a manifestation of social, cultural and psychologic malaise. PMID:7139470

  18. Suicide by asphyxiation with or without helium inhalation in the region of Amsterdam (2005-2014).

    PubMed

    van den Hondel, Karen E; Buster, Marcel; Reijnders, Udo J L

    2016-11-01

    Annually about 28% of the 5800 death of unnatural cause in the Netherlands are a result of suicide. In 2012 and 2013 a movie and a book were published about a "dignified end of life" which also described the suicide using the exit bag to establish asphyxia using helium. The purpose of this study is to investigate if the suicide methods changed since the publicity in 2013 about suicidal asphyxiation by using helium gas. This study especially focuses on suicide using the 'exit bag' with or without helium gas. In the period 2005 to 2014 all suicides in the region of Amsterdam-Amstelland and Zaanstreek-Waterland were analyzed and from these suicides cases using the exit bag were selected. The study shows a rising trend with the use of the helium (P > 0.01) and a decreasing trend for suicide by asphyxia using an exit bag (P < 0.05). The data does not show a sudden difference, but there seems to be a gradually change. The number of suicides using the helium method is rising in Amsterdam-Amstelland and Zaanstreek-Waterland, while suicides by asphyxiation without helium are decreasing. The specific publicity of books about suicides using helium may have influenced this transition. Copyright © 2016 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

  19. Metabolomic Profiling in Perinatal Asphyxia: A Promising New Field

    PubMed Central

    Denihan, Niamh M.; Boylan, Geraldine B.; Murray, Deirdre M.

    2015-01-01

    Metabolomics, the latest “omic” technology, is defined as the comprehensive study of all low molecular weight biochemicals, “metabolites” present in an organism. As a systems biology approach, metabolomics has huge potential to progress our understanding of perinatal asphyxia and neonatal hypoxic-ischaemic encephalopathy, by uniquely detecting rapid biochemical pathway alterations in response to the hypoxic environment. The study of metabolomic biomarkers in the immediate neonatal period is not a trivial task and requires a number of specific considerations, unique to this disease and population. Recruiting a clearly defined cohort requires standardised multicentre recruitment with broad inclusion criteria and the participation of a range of multidisciplinary staff. Minimally invasive biospecimen collection is a priority for biomarker discovery. Umbilical cord blood presents an ideal medium as large volumes can be easily extracted and stored and the sample is not confounded by postnatal disease progression. Pristine biobanking and phenotyping are essential to ensure the validity of metabolomic findings. This paper provides an overview of the current state of the art in the field of metabolomics in perinatal asphyxia and neonatal hypoxic-ischaemic encephalopathy. We detail the considerations required to ensure high quality sampling and analysis, to support scientific progression in this important field. PMID:25802843

  20. Spin on perinatal testicular torsion.

    PubMed

    Samnakay, Naeem; Tudehope, David; Walker, Rosslyn

    2006-11-01

    We describe a recent case of perinatal testicular torsion at our institution. The presentation, management and outcome of perinatal testicular torsion are quite different to testicular torsion in the general paediatric population. The literature describes a variety of management options for perinatal testicular torsion and these are briefly reviewed. In cases of unilateral perinatal testicular torsin, there is controversy over whether surgery to fix the contralateral testis is required, and if so, the appropriate timing for the surgery. A good understanding of the issues unique to perinatal torsion will facilitate appropriate counseling of parents of affected neonates.

  1. Fatal and near-fatal autoerotic asphyxial episodes in women. Characteristic features based on a review of nine cases.

    PubMed

    Byard, R W; Hucker, S J; Hazelwood, R R

    1993-03-01

    As asphyxial episodes during autoerotic activity are rarely reported in women, a review of eight fatal cases and one near-fatal case was conducted to delineate more clearly the characteristics of this syndrome in women. Six cases involved characteristic fatal autoerotic asphyxial activity. The remaining two fatal cases were atypical in that the apparatus that was used for sexual purposes was not intended to cause asphyxia in one case and did not directly cause asphyxial death in the second case. The final case was not fatal. Significantly, the majority of women did not use unusual clothing, props, or devices to augment their activity, for example, five were completely naked and only one was found with elaborate clothing and extra ligatures. Six of the fatal cases had objective evidence of sexual activity, three had used neck padding to prevent chafing, and eight had failed self-rescue mechanisms. Of note, the initial impression in four cases (44%) was homicide (two), attempted suicide (one), and accidental death during sexual activity with a partner (one). These results support the assertion that the manifestations of female autoerotic asphyxial activity reported to date may be initially misleading to investigators. Our purpose in presenting these findings, therefore, is to increase awareness of the more subtle features of this syndrome in women in an attempt to reduce the potential for underdiagnosis or confusion with nonaccidental death in future cases.

  2. Prospective cohort study for identification of underlying genetic causes in neonatal encephalopathy using whole-exome sequencing.

    PubMed

    Bruun, Theodora U J; DesRoches, Caro-Lyne; Wilson, Diane; Chau, Vann; Nakagawa, Tadashi; Yamasaki, Masahiro; Hasegawa, Shinya; Fukao, Toshiyuki; Marshall, Christian; Mercimek-Andrews, Saadet

    2017-08-17

    PurposeNeonatal encephalopathy, which is characterized by a decreased level of consciousness, occurs in 1-7/1,000 live-term births. In more than half of term newborns, there is no identifiable etiological factor. To identify underlying genetic defects, we applied whole-exome sequencing (WES) in term newborns with neonatal encephalopathy as a prospective cohort study.MethodsTerm newborns with neonatal encephalopathy and no history of perinatal asphyxia were included. WES was performed using patient and both parents' DNA.ResultsNineteen patients fulfilling inclusion criteria were enrolled. Five patients were excluded owing to withdrawal of consent, no parental DNA samples, or a genetic diagnosis prior to WES. Fourteen patients underwent WES. We confirmed a genetic diagnosis in five patients (36%): epileptic encephalopathy associated with autosomal dominant de novo variants in SCN2A (p.Met1545Val), KCNQ2 (p.Asp212Tyr), and GNAO1 (p.Gly40Arg); lipoic acid synthetase deficiency due to compound heterozygous variants in LIAS (p.Ala253Pro and p.His236Gln); and encephalopathy associated with an X-linked variant in CUL4B (p.Asn211Ser).ConclusionWES is helpful at arriving genetic diagnoses in neonatal encephalopathy and/or seizures and brain damage. It will increase our understanding and probably enable us to develop targeted neuroprotective treatment strategies.GENETICS in MEDICINE advance online publication, 17 August 2017; doi:10.1038/gim.2017.129.

  3. The transmissible spongiform encephalopathies of livestock

    USDA-ARS?s Scientific Manuscript database

    Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal protein misfolding neurodegenerative diseases. TSEs have been described in several species including bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep and goats, chronic wasting disease (CWD) in cervids, tr...

  4. Predicting neonatal morbidity after perinatal asphyxia: a scoring system.

    PubMed

    Portman, R J; Carter, B S; Gaylord, M S; Murphy, M G; Thieme, R E; Merenstein, G B

    1990-01-01

    Predicting immediate neonatal morbidity after perinatal asphyxia has been difficult. A review of asphyxiated neonates greater than or equal to 36 weeks' gestation admitted to The Children's Hospital Newborn Intensive Care Unit in 1983 was conducted to devise a scoring system that would rapidly predict organ dysfunction observed in the immediate neonatal period. Comparison of potential score components to morbidity by multiple regression analysis yielded significant association with abnormalities in fetal heart rate monitoring, the 5-minute Apgar score, and neonatal base deficit. A scoring system was devised whose sensitivity (93.8%) and specificity (81.3%) were more predictive than any of its individual components. Prospective analysis in a similar population in 1984 validated its ability to distinguish severe from moderate morbidity after asphyxia. Positive predictive value for the score in the combined study groups (n = 98) was 79% and the negative predictive value was 83%. The scoring system may offer a rapid and accurate prediction of organ dysfunction in the immediate neonatal period after asphyxia.

  5. Chronic traumatic encephalopathy.

    PubMed

    Yi, Juneyoung; Padalino, David J; Chin, Lawrence S; Montenegro, Philip; Cantu, Robert C

    2013-01-01

    Sports-related concussion has gained increased prominence, in part due to media coverage of several well-known athletes who have died from consequences of chronic traumatic encephalopathy (CTE). CTE was first described by Martland in 1928 as a syndrome seen in boxers who had experienced significant head trauma from repeated blows. The classic symptoms of impaired cognition, mood, behavior, and motor skills also have been reported in professional football players, and in 2005, the histopathological findings of CTE were first reported in a former National Football League (NFL) player. These finding were similar to Alzheimer's disease in some ways but differed in critical areas such as a predominance of tau protein deposition over amyloid. The pathophysiology is still unknown but involves a history of repeated concussive and subconcussive blows and then a lag period before CTE symptoms become evident. The involvement of excitotoxic amino acids and abnormal microglial activation remain speculative. Early identification and prevention of this disease by reducing repeated blows to the head has become a critical focus of current research.

  6. Canine congenital portosystemic encephalopathy.

    PubMed

    Maddison, J E

    1988-08-01

    The case records of 21 dogs with congenital portosystemic encephalopathy are reviewed. The disorder was most common in Australian cattledogs (blue heelers; 8 cases), Old English sheepdogs (3 cases) and Maltese terriers (3 cases). Extra-hepatic shunts occurred in small breeds, with the exception of 1 cattledog, while intra-hepatic shunts occurred in the medium to large breeds. The most common clinical pathology abnormalities were abnormal ammonia tolerance, mild to moderate increases in plasma alanine aminotransferase or alkaline phosphatase concentrations, decreased total serum protein concentrations, increased fasting ammonia concentrations and ammonium biurate crystalluria. Radiological examination revealed that all the dogs had a small liver. The kidneys were enlarged in 5 of 10 dogs in which kidney size could be estimated. Surgical ligation of an extra-hepatic shunt was successful in 2 of 4 dogs in which it was attempted. Medical management resulted in alleviation of clinical signs in 5 of 8 dogs. The period of successful treatment ranged from a few months to over a year.

  7. Transmissible encephalopathies in animals.

    PubMed Central

    Kimberlin, R H

    1990-01-01

    Scrapie in sheep and goats is the best known of the transmissible encephalopathies of animals. The combination of maternal transmission of infection and long incubation periods effectively maintains the infection in flocks. A single sheep gene (Sip) controls both experimental and natural scrapie and the discovery of allelic markers could enable the use of sire selection in the control of the natural disease. Studies of experimental rodent scrapie show that neuroinvasion occurs by spread of infection from visceral lymphoreticular tissues along nerve fibers to mid-thoracic cord. The slowness of scrapie is due to restrictions on replication and cell-to-cell spread of infection affecting neuroinvasion and subsequent neuropathogenesis. Probably both stages in mice are controlled by Sinc gene, the murine equivalent of Sip. The glycoprotein PrP may be the normal product of Sinc gene. Posttranslationally modified PrP forms the disease specific "scrapie associated fibrils" and may also be a constituent of the infectious agent. Scrapie-like diseases have been reported in mink and several species of ruminants including cattle. All of them may be caused by the recycling of scrapie infected sheep material in animal feed. The human health implications are discussed. PMID:2407328

  8. Sepsis-associated encephalopathy.

    PubMed

    Gofton, Teneille E; Young, G Bryan

    2012-10-01

    Sepsis-associated encephalopathy (SAE) is a diffuse brain dysfunction that occurs secondary to infection in the body without overt CNS infection. SAE is frequently encountered in critically ill patients in intensive care units, and in up to 70% of patients with severe systemic infection. The severity of SAE can range from mild delirium to deep coma. Seizures and myoclonus are infrequent and cranial nerves are almost always spared, but most severe cases have an associated critical illness neuromyopathy. Development of SAE probably involves a number of mechanisms that are not mutually exclusive and vary from patient to patient. Substantial neurological and psychological morbidities often occur in survivors. Mortality is almost always due to multiorgan failure rather than neurological complications, and is almost 70% in patients with severe SAE. Further research into the pathophysiology, management and prevention of SAE is needed. This Review discusses the epidemiology and clinical presentation of SAE. Recent evidence for SAE pathophysiology is outlined and a diagnostic approach to patients with this syndrome is presented. Lastly, prognosis and management of SAE is discussed.

  9. Sepsis Associated Encephalopathy.

    PubMed

    Chaudhry, Neera; Duggal, Ashish Kumar

    2014-01-01

    Sepsis associated encephalopathy (SAE) is a common but poorly understood neurological complication of sepsis. It is characterized by diffuse brain dysfunction secondary to infection elsewhere in the body without overt CNS infection. The pathophysiology of SAE is complex and multifactorial including a number of intertwined mechanisms such as vascular damage, endothelial activation, breakdown of the blood brain barrier, altered brain signaling, brain inflammation, and apoptosis. Clinical presentation of SAE may range from mild symptoms such as malaise and concentration deficits to deep coma. The evaluation of cognitive dysfunction is made difficult by the absence of any specific investigations or biomarkers and the common use of sedation in critically ill patients. SAE thus remains diagnosis of exclusion which can only be made after ruling out other causes of altered mentation in a febrile, critically ill patient by appropriate investigations. In spite of high mortality rate, management of SAE is limited to treatment of the underlying infection and symptomatic treatment for delirium and seizures. It is important to be aware of this condition because SAE may present in early stages of sepsis, even before the diagnostic criteria for sepsis can be met. This review discusses the diagnostic approach to patients with SAE along with its epidemiology, pathophysiology, clinical presentation, and differential diagnosis.

  10. Sepsis-associated encephalopathy.

    PubMed

    Cotena, Simona; Piazza, Ornella

    2012-01-01

    Sepsis-associated encephalopathy (SAE) is defined as a diffuse or multifocal cerebral dysfunction induced by the systemic response to the infection without clinical or laboratory evidence of direct brain infection. Its pathogenesis is multifactorial. SAE generally occurs early during severe sepsis and precedes multiple-organ failure. The most common clinical feature of SAE is the consciousness alteration which ranges from mildly reduced awareness to unresponsiveness and coma. Diagnosis of SAE is primarily clinical and depends on the exclusion of other possible causes of brain deterioration. Electroencephalography (EEG) is almost sensitive, but it is not specific for SAE. Computed Tomography (CT) head scan generally is negative in case of SAE, while Magnetic Resonance Imaging (MRI) can show brain abnormalities in case of SAE, but they are not specific for this condition. Somatosensitive Evoked Potentials (SEPs) are sensitive markers of developing cerebral dysfunction in sepsis. Cerebrospinal fluid (CBF) analysis is generally normal, a part an inconstant elevation of proteins concentration. S100B and NSE have been proposed like biomarkers for diagnosis of SAE, but the existing data are controversial. SAE is reversible even if survivors of severe sepsis have often long lasting or irreversible cognitive and behavioral sequel; however the presence of SAE can have a negative influence on survival. A specific therapy of SAE does not exist and the outcome depends on a prompt and appropriate treatment of sepsis as whole.

  11. Sepsis Associated Encephalopathy

    PubMed Central

    Chaudhry, Neera; Duggal, Ashish Kumar

    2014-01-01

    Sepsis associated encephalopathy (SAE) is a common but poorly understood neurological complication of sepsis. It is characterized by diffuse brain dysfunction secondary to infection elsewhere in the body without overt CNS infection. The pathophysiology of SAE is complex and multifactorial including a number of intertwined mechanisms such as vascular damage, endothelial activation, breakdown of the blood brain barrier, altered brain signaling, brain inflammation, and apoptosis. Clinical presentation of SAE may range from mild symptoms such as malaise and concentration deficits to deep coma. The evaluation of cognitive dysfunction is made difficult by the absence of any specific investigations or biomarkers and the common use of sedation in critically ill patients. SAE thus remains diagnosis of exclusion which can only be made after ruling out other causes of altered mentation in a febrile, critically ill patient by appropriate investigations. In spite of high mortality rate, management of SAE is limited to treatment of the underlying infection and symptomatic treatment for delirium and seizures. It is important to be aware of this condition because SAE may present in early stages of sepsis, even before the diagnostic criteria for sepsis can be met. This review discusses the diagnostic approach to patients with SAE along with its epidemiology, pathophysiology, clinical presentation, and differential diagnosis. PMID:26556425

  12. CT and MRI findings of cyclosporine-related encephalopathy and hypertensive encephalopathy.

    PubMed

    Yamamoto, Akira; Hayakawa, Katsumi; Houjyou, Makoto

    2002-05-01

    We present the MRI and CT findings of one child with cyclosporine-related encephalopathy, and one child with hypertensive encephalopathy following cyclosporine-related encephalopathy. The imaging findings were shown well on T2-weighted and fluid-attenuated inversion recovery (FLAIR) MR images. Cyclosporine-related encephalopathy was distributed predominantly in the posterior white matter. Hypertensive encephalopathy showed similar changes of CT attenuation, but with wider distribution. These two disorders seem to have the same pathogenesis.

  13. Atrial natriuretic factor in neonatal hypoxic-ischemic encephalopathy.

    PubMed

    Carbonell, X; Figueras, J; Salvia, M D; Esque, M T; Delgado, M P; Jimenez, R

    1993-01-01

    The influence of perinatal asphyxia in the secretion of atrial natriuretic factor (ANF) during the first 6 days of life, and its renal consequences are discussed. Comparison between 20 healthy term neonates and 19 with first--or second--degree hypoxic-ischemic encephalopathy (HIE) is made. Daily controls were performed on clinical and neurological examinations and administration of sodium and fluids. On the first and sixth days of life, 24 hours urine collection, natremia, natriuresis, fractionated excretion of sodium and creatinine clearance were determined. The ANF was performed at 1, 2, 3 and 6 days old, by R.I.A. The full term newborns with HIE showed a peak in ANF values on day two, as does the control group, thereafter maintaining higher levels, with a significant difference on day three and six. No correlation could be found between the ANF levels and the renal variables analyzed.

  14. Reflex gelastic–dacrystic seizures following hypoxic–ischaemic encephalopathy

    PubMed Central

    Verma, Rajesh; Praharaj, Heramba Narayan

    2013-01-01

    Reflex or stimulus-sensitive epilepsies are uncommon epileptic syndromes triggered by exogenous-specific sensory stimulus or endogenous various mental activities. Gelastic–dacrystic seizures are rare epileptic manifestations characterised by ictal laughter and crying. Gelastic–dacrystic seizures are commonly caused by hypothalamic hamartoma but rarely described due to cortical dysplasia, lesions of frontal and temporal lobes, tumours and vascular malformations. We report a young woman who presented with somatosensory-evoked gelastic–dacrystic seizures. This patient had a positive history of perinatal insult substantiated by MRI findings. Hypoxic–ischaemic encephalopathy as the cause of gelastic–dacrystic seizures has not been reported so far in the literature. PMID:23853086

  15. Sepsis associated encephalopathy (SAE): a review.

    PubMed

    Green, Rebecca; Scott, L Keith; Minagar, Alireza; Conrad, Steven

    2004-05-01

    Sepsis associated encephalopathy (SAE) is a poorly understood condition that is associated with severe sepsis and appears to have a negative influence on survival. The incidence of encephalopathy secondary to sepsis is unknown. Amino acid derangements, blood-brain barrier disruption, abnormal neurotransmitters, and direct CNS effect are possible causes of septic encephalopathy. Research has not defined the pathogenesis of SAE.

  16. The role of inflammation in perinatal brain injury

    PubMed Central

    Hagberg, Henrik; Mallard, Carina; Ferriero, Donna M.; Vannucci, Susan J.; Levison, Steven W.; Vexler, Zinaida S.; Gressens, Pierre

    2015-01-01

    Inflammation is increasingly recognized as being a critical contributor to both normal development and injury outcome in the immature brain. The focus of this Review is to highlight important differences in innate and adaptive immunity in immature versus adult brain, which support the notion that the consequences of inflammation will be entirely different depending on context and stage of CNS development. Perinatal brain injury can result from neonatal encephalopathy and perinatal arterial ischaemic stroke, usually at term, but also in preterm infants. Inflammation occurs before, during and after brain injury at term, and modulates vulnerability to and development of brain injury. Preterm birth, on the other hand, is often a result of exposure to inflammation at a very early developmental phase, which affects the brain not only during fetal life, but also over a protracted period of postnatal life in a neonatal intensive care setting, influencing critical phases of myelination and cortical plasticity. Neuroinflammation during the perinatal period can increase the risk of neurological and neuropsychiatric disease throughout childhood and adulthood, and is, therefore, of concern to the broader group of physicians who care for these individuals. PMID:25686754

  17. The prenatal detection of Jeune syndrome (asphyxiating thoracic dystrophy). Case report.

    PubMed

    Socolov, R; Rusu, Cristina; Socolov, Demetra; Iliev, G; Buţureanu, St; Covali, Roxana

    2006-01-01

    The authors present the case of a foetus with Jeune syndrome (asphyxiating thoracic dystrophy) in a woman with a previous deceased child with the same disease, and also with a normal sibling. The diagnosis was mentioned at 26 week of pregnancy, based on ultrasonographic findings: short proximal bones (under 3 percentiles), and a diminished thoracic circumference, (although greater than 10 percentiles for the gestational age). There was an interdisciplinary agreement for the therapeutic termination of the pregnancy, and the post-expulsion assessment confirmed the diagnosis. This case demonstrates a higher incidence of Jeune dystrophy than the one expected for an autosomal recessive disease, with 2 out of 3 children affected, instead of 25%. It also shows that the earliest change is the one regarding the shortened long bones, often difficult to notice before 20 weeks, fact which favors a detailed genetic sonogram done after this limit.

  18. [Risk factors for preterm encephalopathy].

    PubMed

    Kornacka, Maria K; Bokiniec, Renata; Bargiel, Agata

    2009-08-01

    Encephalopathy in a common neonatological sense is a term referring to a complex of clinical symptoms occurring in term infants in the first days of their life as a result of hypoxic-ischemic lesions. However, if we accept the encyclopedic definition of encephalopathy as a vast or multifocal brain lesions caused by a variety of factors, we may use the term to describe all patients with traumatic, hypoxic or toxic brain lesions, and therefore also newborns at different levels of maturity. Contrary to term newborns, in which case the hypoxic-ischemic encephalopathy are mostly intrauterine, for preterm infants there is a number of factors which destroy neural tissue postnatally The occurrence of those factors is often influenced by elements of essential intensive care. The article describes the most common biochemical disturbances and clinical causes.

  19. Electroencephalography of autoimmune limbic encephalopathy.

    PubMed

    Kaplan, Peter W; Sutter, Raoul

    2013-10-01

    There is an increasing recognition of autoimmune limbic encephalopathy with the hope for earlier diagnosis and expedited and improved treatment. Although antibody testing remains the definitive clinical diagnostic feature, the presentation of a rapid dementia, behavioral changes, and seizures leads to investigation using cerebral imaging, electroencephalography, and cerebrospinal fluid to confirm the diagnosis and also to exclude similar disorders. The electroencephalographer may be asked to comment on the types of electroencephalography abnormality and provide input toward the diagnosis of limbic encephalopathy. This article reviews the literature on limbic paraneoplastic and nonparaneoplastic encephalopathies, providing descriptions and examples of the electroencephalography findings. Typically, there are patterns of slow theta and delta activity and different patterns of temporal and frontal epileptic activity.

  20. Perinatal loss: a family perspective.

    PubMed

    Callister, Lynn Clark

    2006-01-01

    Perinatal loss is a profound experience for childbearing families. Examples of perinatal loss include miscarriage, ectopic pregnancy, stillbirth, neonatal death, and other losses. Perinatal loss engenders a unique kind of mourning since the child is so much a part of the parental identity. Societal expectations for mourning associated with perinatal loss are noticeably absent. Gender differences in response to such loss, as well as sibling and grandparent grief have been identified in the literature. Descriptive studies provide information on cultural responses to perinatal loss. Nursing interventions have been refined over the past two decades as research studies have been performed, in order to more fully promote health and healing in the face of perinatal loss. These include helping to create meaning through the sharing of the story of parental loss, the facilitation of sociocultural rituals associated with loss, the provision of tangible mementos, sensitive presence, and the validation of the loss. Outcome evaluations of such interventions are recommended.

  1. Molecular pathology of pulmonary surfactants and cytokines in drowning compared with other asphyxiation and fatal hypothermia.

    PubMed

    Miyazato, Takako; Ishikawa, Takaki; Michiue, Tomomi; Maeda, Hitoshi

    2012-07-01

    Drowning involves complex fatal factors, including asphyxiation and electrolyte/osmotic disturbances, as well as hypothermia in cold water. The present study investigated the molecular pathology of pulmonary injury due to drowning, using lung specimens from forensic autopsy cases of drowning (n = 21), acute mechanical asphyxia due to neck compression and smothering (n = 24), and hypothermia (cold exposure, n = 11), as well as those of injury (n = 23), intoxication (n = 13), fire fatality (n = 18), and acute cardiac death (n = 9) for comparison. TaqMan real-time reverse transcription polymerase chain reaction was used to quantify messenger RNA (mRNA) expressions of pulmonary surfactant-associated proteins A and D (SP-A and SP-D), tumor necrosis factor (TNF)-α, interleukin (IL)-1β, and IL-10. SP-A and SP-D mRNA levels were lower for drowning, mechanical asphyxiation, fire fatality, and acute cardiac deaths than for hypothermia and injury. TNF-α, IL-1β, and IL-10 mRNA levels were higher for drowning or for drowning and injury than for other groups; there was no significant difference between fire fatality, involving airway injury due to inhalation of hot/irritant gases, and other control groups. These observations suggest characteristic molecular biological patterns of pulmonary injury involving suppression of pulmonary surfactants and activation of early-phase mediators of inflammation in drowning, with high mRNA expression levels of pulmonary surfactants in fatal hypothermia; however, there was no significant difference among these markers in immunohistochemical detection, except for SP-A. These mRNA expressions can be used as markers of pulmonary injury to assist in investigations of the pathophysiology of drowning and fatal hypothermia in combination with other biochemical and biological markers.

  2. GNAO1 encephalopathy

    PubMed Central

    Danti, Federica Rachele; Galosi, Serena; Romani, Marta; Montomoli, Martino; Carss, Keren J.; Raymond, F. Lucy; Parrini, Elena; Bianchini, Claudia; McShane, Tony; Dale, Russell C.; Mohammad, Shekeeb S.; Shah, Ubaid; Mahant, Neil; Ng, Joanne; McTague, Amy; Samanta, Rajib; Vadlamani, Gayatri; Valente, Enza Maria; Leuzzi, Vincenzo; Kurian, Manju A.

    2017-01-01

    Objective: To describe better the motor phenotype, molecular genetic features, and clinical course of GNAO1-related disease. Methods: We reviewed clinical information, video recordings, and neuroimaging of a newly identified cohort of 7 patients with de novo missense and splice site GNAO1 mutations, detected by next-generation sequencing techniques. Results: Patients first presented in early childhood (median age of presentation 10 months, range 0–48 months), with a wide range of clinical symptoms ranging from severe motor and cognitive impairment with marked choreoathetosis, self-injurious behavior, and epileptic encephalopathy to a milder phenotype, featuring moderate developmental delay associated with complex stereotypies, mainly facial dyskinesia and mild epilepsy. Hyperkinetic movements were often exacerbated by specific triggers, such as voluntary movement, intercurrent illnesses, emotion, and high ambient temperature, leading to hospital admissions. Most patients were resistant to drug intervention, although tetrabenazine was effective in partially controlling dyskinesia for 2/7 patients. Emergency deep brain stimulation (DBS) was life saving in 1 patient, resulting in immediate clinical benefit with complete cessation of violent hyperkinetic movements. Five patients had well-controlled epilepsy and 1 had drug-resistant seizures. Structural brain abnormalities, including mild cerebral atrophy and corpus callosum dysgenesis, were evident in 5 patients. One patient had a diffuse astrocytoma (WHO grade II), surgically removed at age 16. Conclusions: Our findings support the causative role of GNAO1 mutations in an expanded spectrum of early-onset epilepsy and movement disorders, frequently exacerbated by specific triggers and at times associated with self-injurious behavior. Tetrabenazine and DBS were the most useful treatments for dyskinesia. PMID:28357411

  3. Inflammation in Epileptic Encephalopathies.

    PubMed

    Shandra, Oleksii; Moshé, Solomon L; Galanopoulou, Aristea S

    2017-01-01

    West syndrome (WS) is an infantile epileptic encephalopathy that manifests with infantile spasms (IS), hypsarrhythmia (in ~60% of infants), and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12%-15%), or of unknown origin. The current treatment options include hormonal treatment (adrenocorticotropic hormone and high-dose steroids) and the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS. There is a need to identify new therapeutic targets and more effective treatments for WS. Theories about the role of inflammatory pathways in the pathogenesis and treatment of WS have emerged, being supported by both clinical and preclinical data from animal models of WS. Ongoing advances in genetics have revealed numerous genes involved in the pathogenesis of WS, including genes directly or indirectly involved in inflammation. Inflammatory pathways also interact with other signaling pathways implicated in WS, such as the neuroendocrine pathway. Furthermore, seizures may also activate proinflammatory pathways raising the possibility that inflammation can be a consequence of seizures and epileptogenic processes. With this targeted review, we plan to discuss the evidence pro and against the following key questions. Does activation of inflammatory pathways in the brain cause epilepsy in WS and does it contribute to the associated comorbidities and progression? Can activation of certain inflammatory pathways be a compensatory or protective event? Are there interactions between inflammation and the neuroendocrine system that contribute to the pathogenesis of WS? Does activation of brain inflammatory signaling pathways contribute to the transition of WS to Lennox-Gastaut syndrome? Are there any lead candidates or unexplored targets for future therapy development for WS targeting inflammation? © 2017 Elsevier Inc. All rights reserved.

  4. [Multicenter program for the integrated care of newborns with perinatal hypoxic-ischemic insult (ARAHIP)].

    PubMed

    Arnáez, J; Vega, C; García-Alix, A; Gutiérrez, E P; Caserío, S; Jiménez, M P; Castañón, L; Esteban, I; Hortelano, M; Hernández, N; Serrano, M; Prada, T; Diego, P; Barbadillo, F

    2015-03-01

    Newborns with perinatal indicators of a potential hypoxic-ischemic event require an integrated care in order to control the aggravating factors of brain damage, and the early identification of candidates for hypothermia treatment. The application of a prospective, populational program that organizes and systematizes medical care during the first 6 hours of life to all newborns over 35 weeks gestational age born with indicators of a perinatal hypoxic-ischemic insult. The program includes 12 hospitals (91,217 m(2)); two level i centers, five level ii centers, and five level iii hospitals. The program establishes four protocols: a) detection of the newborn with a potential hypoxic-ischemic insult, b) surveillance of the neurological repercussions and other organ involvement, c) control and treatment of complications, d) procedures and monitoring during transport. From June 2011 to June 2013, 213 of 32325 newborns above 35 weeks gestational age met the criteria of a potential hypoxic-ischemic insult (7.4/1000), with 92% of them being cared for following the program specifications. Moderate-severe hypoxic-ischemic encephalopathy was diagnosed in 33 cases (1/1,000), and 31 out of the 33 received treatment with hypothermia (94%). The program for the Integrated Care of Newborns with Perinatal Hypoxic-Ischemic Insult has led to providing a comprehensive care to the newborns with a suspected perinatal hypoxic-ischemic insult. Aggravators of brain damage have been controlled, and cases of moderate-severe hypoxic-ischemic encephalopathy have been detected, allowing the start of hypothermia treatment within the first six hours of life. Populational programs are fundamental to reducing the mortality and morbidity of hypoxic-ischemic encephalopathy. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

  5. Restricted diffusion in the corpus callosum: A neuroradiological marker in hypoxic–ischemic encephalopathy

    PubMed Central

    Kale, Alok; Joshi, Priscilla; Kelkar, A B

    2016-01-01

    Background: Restricted diffusion within the splenium of the corpus callosum has been described by other authors in various conditions, however, restricted diffusion in the entire corpus callosum or isolated involvement of the splenium, genu, or body has been infrequently reported on magnetic resonance imaging (MRI) in neonatal hypoxic–ischemic encephalopathy. We report a series of cases showing different patterns of involvement. Methods and Materials: Perinatal imaging with MRI including diffusion-weighted imaging was performed in 40 neonates with hypoxic–ischemic encephalopathy, including 11 premature neonates. Sixteen out of 40 patients demonstrated restricted diffusion within the corpus callosum. Out of 16 patients, 9 showed restricted diffusion in the entire corpus callosum, 4 had isolated splenium involvement, 2 had body and splenium signal abnormality, and 1 showed diffusion restriction only in the genu. Conclusions: Changes in the corpus callosum were also associated with more severe clinical presentation of encephalopathy. Restricted diffusion within the corpus callosum in infants with hypoxic–ischemic encephalopathy is often associated with extensive brain injury and appears to be an early neuroradiologic marker of adverse neurologic outcome. PMID:28104944

  6. Could intra-alveolar hemosiderin deposition in adults be used as a marker for previous asphyxial episodes in cases of autoerotic death?

    PubMed

    Byard, Roger W; Masoumi, Homeyra; Haas, Elizabeth; Sage, Martin; Krous, Henry F

    2011-05-01

    Intra-alveolar hemorrhage and hemosiderin have been cited as possible markers of recent and remote asphyxial events. Little study has been undertaken of the potential significance of intra-alveolar hemosiderin in adults as a potential marker of previous sublethal asphyxial episodes. Ten cases of lethal sexual asphyxia (an entity known to be associated with repetitive sublethal asphyxial episodes) and 20 randomly selected, age- and sex-matched controls had sections of lung stained for hemosiderin. Subsequently, intra-alveolar, iron-containing macrophages were counted. All cases were men (ages 15-50 years; mean 31.8). No significant increase in hemosiderin was found in victims of sexual asphyxia, indicating that asphyxial episodes in sublethal sexual asphyxial activities may not be sufficiently intense or prolonged to cause intra-alveolar hemorrhage or that intra-alveolar hemorrhage in adults is a relatively nonspecific finding. These results do not support intra-alveolar hemosiderin deposition as a marker for previous sublethal asphyxial events in autoerotic asphyxia. © 2011 American Academy of Forensic Sciences.

  7. Hashimoto's encephalopathy cases: Chinese experience.

    PubMed

    Tang, Yi; Xing, Yi; Lin, Michael T; Zhang, Jin; Jia, Jianping

    2012-07-24

    Hashimoto's encephalopathy is a poorly understood syndrome consisting of heterogeneous neurological symptoms and high serum antithyroid antibody titers, typically responding to steroids. More clinical series studies are required to characterize the clinical, laboratory and imaging features, and outcomes, especially in the Chinese population. We analyzed the clinical, laboratory, and imaging features and outcomes of thirteen consecutive patients with Hashimoto's encephalopathy diagnosed in Xuan Wu Hospital, Beijing from 2005 to 2010 retrospectively. Cognitive impairment (84.6%) and psychiatric symptoms (38.5%) were the most frequent symptoms. Seizures (30.8%) and myoclonus (7.7%) were less common than previously described. Three (23.1%) patients showed abnormal signals in hippocampus or temporal lobe, which were believed related to their memory disorders or seizures. MRI changes showed resolution paralleling clinical improvement in one patient. Among eight patients who received steroid therapy, five patients recovered, one patient improved with residual deficits, and two patients relapsed or had no effect. Among five non-steroid treated patients, three patients experienced stable remission with antiepileptic drugs or general neurotrophic therapy, and two patients experienced continuous deterioration. Most patients with Hashimoto's encephalopathy showed good response to steroids. Some patients improved without steroid therapy. Considering its reversible course, we recommend that Hashimoto's encephalopathy should always be in the differential diagnosis while evaluating disorders of the central nervous system.

  8. Neonatal Encephalopathic Cerebral Injury in South India Assessed by Perinatal Magnetic Resonance Biomarkers and Early Childhood Neurodevelopmental Outcome

    PubMed Central

    Pauliah, Shreela S.; Bainbridge, Alan; Kurien, Justin; Sivasamy, Neeraja; Cowan, Frances M.; Balraj, Guhan; Ayer, Manjula; Satheesan, Kariyapilly; Ceebi, Sreejith; Wade, Angie; Swamy, Ravi; Padinjattel, Shaji; Hutchon, Betty; Vijayakumar, Madhava; Nair, Mohandas; Padinharath, Krishnakumar; Zhang, Hui; Cady, Ernest B.; Shankaran, Seetha; Thayyil, Sudhin

    2014-01-01

    Although brain injury after neonatal encephalopathy has been characterised well in high-income countries, little is known about such injury in low- and middle-income countries. Such injury accounts for an estimated 1 million neonatal deaths per year. We used magnetic resonance (MR) biomarkers to characterise perinatal brain injury, and examined early childhood outcomes in South India. Methods We recruited consecutive term or near term infants with evidence of perinatal asphyxia and a Thompson encephalopathy score ≥6 within 6 h of birth, over 6 months. We performed conventional MR imaging, diffusion tensor MR imaging and thalamic proton MR spectroscopy within 3 weeks of birth. We computed group-wise differences in white matter fractional anisotropy (FA) using tract based spatial statistics. We allocated Sarnat encephalopathy stage aged 3 days, and evaluated neurodevelopmental outcomes aged 3½ years using Bayley III. Results Of the 54 neonates recruited, Sarnat staging was mild in 30 (56%); moderate in 15 (28%) and severe in 6 (11%), with no encephalopathy in 3 (6%). Six infants died. Of the 48 survivors, 44 had images available for analysis. In these infants, imaging indicated perinatal rather than established antenatal origins to injury. Abnormalities were frequently observed in white matter (n = 40, 91%) and cortex (n = 31, 70%) while only 12 (27%) had abnormal basal ganglia/thalami. Reduced white matter FA was associated with Sarnat stage, deep grey nuclear injury, and MR spectroscopy N-acetylaspartate/choline, but not early Thompson scores. Outcome data were obtained in 44 infants (81%) with 38 (79%) survivors examined aged 3½ years; of these, 16 (42%) had adverse neurodevelopmental outcomes. Conclusions No infants had evidence for established brain lesions, suggesting potentially treatable perinatal origins. White matter injury was more common than deep brain nuclei injury. Our results support the need for rigorous evaluation of the efficacy of

  9. Neonatal and Perinatal Infections.

    PubMed

    Khan, Amira M; Morris, Shaun K; Bhutta, Zulfiqar A

    2017-08-01

    Lack of success in achieving considerable reductions in neonatal mortality is a contributory factor in failing to achieve Millennium Development Goal 4.2.6 million neonates still die each year, with preterm birth and infections the two leading causes. Maternal infections and environmental and infant factors influence acquisition of viral and bacterial infections in the perinatal and neonatal period. Scaling up evidence-based interventions addressing maternal risk factors and underlying causes could reduce neonatal infections by 84%. The emergence of new infections and increasing antimicrobial resistance present public health challenges that must be addressed to achieve substantial reductions in neonatal mortality. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Relationship between opioid therapy, tissue-damaging procedures, and brain metabolites as measured by proton MRS in asphyxiated term neonates.

    PubMed

    Angeles, Danilyn M; Ashwal, Stephen; Wycliffe, Nathaniel D; Ebner, Charlotte; Fayard, Elba; Sowers, Lawrence; Holshouser, Barbara A

    2007-05-01

    To examine the effects of opioid and tissue-damaging procedures (TDPs) [i.e. procedures performed in the neonatal intensive care unit (NICU) known to result in pain, stress, and tissue damage] on brain metabolites, we reviewed the medical records of 28 asphyxiated term neonates (eight opioid-treated, 20 non-opioid treated) who had undergone magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (MRS) within the first month of life as well as eight newborns with no clinical findings of asphyxial injury. We found that lower creatine (Cr), myoinositol (Ins), and N-acetylaspartate (NAA)/choline (Cho) (p < or = 0.03) and higher Cho/Cr and glutamate/glutamine (Glx) Cr (p < or = 0.02) correlated with increased TDP incidence in the first 2 d of life (DOL). We also found that occipital gray matter (OGM) NAA/Cr was decreased (p = 0.03) and lactate (Lac) was present in a significantly higher amount (40%; p = 0.03) in non-opioid-treated neonates compared with opioid-treated neonates. Compared with controls, untreated neonates showed larger changes in more metabolites in basal ganglia (BG), thalami (TH), and OGM with greater significance than treated neonates. Our data suggest that TDPs affect spectral metabolites and that opioids do not cause harm in asphyxiated term neonates exposed to repetitive TDPs in the first 2-4 DOL and may provide a degree of neuroprotection.

  11. Fatal Asphyxiation in Two Long-Finned Pilot Whales (Globicephala melas) Caused by Common Soles (Solea solea).

    PubMed

    IJsseldijk, Lonneke L; Leopold, Mardik F; Bravo Rebolledo, Elisa L; Deaville, Rob; Haelters, Jan; IJzer, Jooske; Jepson, Paul D; Gröne, Andrea

    2015-01-01

    Long-finned pilot whales (Globicephala melas) are rare visitors to the southern North Sea, but recently two individual strandings occurred on the Dutch coast. Both animals shared the same, unusual cause of death: asphyxiation from a common sole (Solea solea) stuck in their nasal cavity. This is a rare cause of death in cetaceans. Whilst asphyxiation has been reported in smaller odontocetes, there are no recent records of this occurring in Globicephala species. Here we report the stranding, necropsy and diet study results as well as discuss the unusual nature of this phenomenon. Flatfish are not a primary prey species for pilot whales and are rarely eaten by other cetaceans, such as harbour porpoises (Phocoena phocoena), in which there are several reports of asphyxiation due to airway obstruction by soles. This risk may be due to the fish's flexible bodies which can enter small cavities either actively in an attempt to escape or passively due to the whale 'coughing' or 'sneezing' to rid itself of the blockage of the trachea. It is also possible that the fish enter the airways whilst the whale is re-articulating the larynx after trying to ingest large, oddly shaped prey. It is unlikely that the soles entered the airways after the death of the whales and we believe therefore that they are responsible for the death of these animals.

  12. Fatal Asphyxiation in Two Long-Finned Pilot Whales (Globicephala melas) Caused by Common Soles (Solea solea)

    PubMed Central

    IJsseldijk, Lonneke L.; Leopold, Mardik F.; Bravo Rebolledo, Elisa L.; Deaville, Rob; Haelters, Jan; IJzer, Jooske; Jepson, Paul D.; Gröne, Andrea

    2015-01-01

    Long-finned pilot whales (Globicephala melas) are rare visitors to the southern North Sea, but recently two individual strandings occurred on the Dutch coast. Both animals shared the same, unusual cause of death: asphyxiation from a common sole (Solea solea) stuck in their nasal cavity. This is a rare cause of death in cetaceans. Whilst asphyxiation has been reported in smaller odontocetes, there are no recent records of this occurring in Globicephala species. Here we report the stranding, necropsy and diet study results as well as discuss the unusual nature of this phenomenon. Flatfish are not a primary prey species for pilot whales and are rarely eaten by other cetaceans, such as harbour porpoises (Phocoena phocoena), in which there are several reports of asphyxiation due to airway obstruction by soles. This risk may be due to the fish’s flexible bodies which can enter small cavities either actively in an attempt to escape or passively due to the whale ‘coughing’ or ‘sneezing’ to rid itself of the blockage of the trachea. It is also possible that the fish enter the airways whilst the whale is re-articulating the larynx after trying to ingest large, oddly shaped prey. It is unlikely that the soles entered the airways after the death of the whales and we believe therefore that they are responsible for the death of these animals. PMID:26580786

  13. How an extended perinatal audit may improve perinatal policy.

    PubMed

    Dehaene, Isabelle; Roelens, Kristien; Page, Geert

    2014-09-29

    Abstract Objective: A perinatal audit has the intention of quality of care improvement based on analysis of perinatal death, with our without analysis of maternal morbidity and/or mortality. Additional analysis of cases of intrapartum asphyxia could provide more insight into ways to improve quality of perinatal care. Methods: Analysis of cases of perinatal death and asphyxia in Jan Yperman Hospital, Ieper, Belgium, in 2012. Results: Three perinatal deaths occurred, none were preventable. Nineteen cases of proven metabolic acidosis have been identified. Three cases are considered possibly preventable, four cases are considered preventable. In three (possibly) preventable cases, foetal monitoring was absent during the active second stage of labour. In two preventable cases, intervention following a significant ST event in the second stage of labour was delayed. In one case intervention was delayed in the first stage of labour, while in another, indicated operative delivery in the second stage was not conducted. Conclusions: Integrating intrapartum asphyxia in the perinatal audit gives an opportunity to identify and eliminate weak points in the perinatal care chain, thereby optimizing quality of care. Lessons learned from our internal audit are the value of foetal monitoring and adequate action on significant ST events during second stage of labour.

  14. Postdatism -- a perinatal problem?

    PubMed

    Chhabra, S; Sood, S

    1990-01-01

    It has been traditionally accepted that maternal and fetal complications are at their lowest levels 37-42 weeks into gestation. 20% of pregnancies completed after 42 weeks gestation are thought to be affected by the postmaturity syndrome of uteroplacental insufficiency resulting in oligohydramnios, meconium passage, loss of fetal subcutaneous tissue, fetal asphyxia, and fetal death. Some workers, however, have also found that pregnancies completed between 40 and 42 weeks carry significant risk. The authors explored this question in a case-control study of 464 women seen at the Mahatma Gandhi Institute of Medical Sciences in Maharashtra, India. The cases of postdatism occurred in the absence of any other medical or obstetric problem. The operative delivery rate increased significantly among these patients compared to deliveries between 39 and 40 weeks. There was neither significant asphyxia nor perinatal loss in term completed normal patients. Asphyxia and perinatal mortality did, however, occur with postdatism. The authors note the likely role of oligohydramnios combined with placental dysfunction.

  15. Urinary Uric Acid/Creatinine Ratio - A Marker For Perinatal Asphyxia

    PubMed Central

    Patel, Kinjal Prahaladbhai; Makadia, Mayur Goradhanbhai; Patel, Vishwal Indravardan; Nilayangode, Haridas Neelakandan

    2017-01-01

    Background Perinatal hypoxia is one of the leading causes of perinatal mortality in developing countries. Both apgar score and arterial blood pH predict the neonatal mortality in asphyxia. Apgar score alone does not predict neurologic outcome and as it is influenced by various factors. This study was conducted to evaluate the utility and sensitivity of urinary uric acid to creatinine ratio (UA/Cr ratio) in asphyxia diagnosis, compared to invasive Arterial Blood Gas (ABG) analysis. Aim To assess the urinary uric acid/creatinine ratio as an additional marker for perinatal asphyxia compared with ABG analysis in apgar score monitoring. Materials and Methods The present case control study was conducted at a teaching hospital in Central Gujarat. Data of 40 healthy newborns and 40 asphyxiated newborns were collected. In absence of regional estimates, a sample of size 39 was required to attain a power of 80% at 5% alpha (type I error) considering a moderate effect size of 0.65. (UA/Cr) ratio was measured from the spot urine sample collected during 24-72 hours of birth. Statistical analysis was performed by Independent t-test, Pearson’s correlation coefficient (r) and Receiver Operating Characteristic (ROC) plots. Results The mean (UA/Cr ratio) (2.75±0.18 vs 1.78±0.23) is significantly higher in asphyxiated group than in the control group (p<0.0001). Urinary UA/Cr ratio had negative correlation with blood pH (r= -0.27, p=0.18), which was not significant (p>0.05). Urinary UA/Cr ratio with criterion of >2.3 had 100% sensitivity, 100% specificity with AUC of 1 (p<0.0001) had a better predictive value. Conclusions Apgar score is usually reduced in neonates with congenital anomalies and premature neonates. Hence, it is preferable that the clinical diagnosis of asphyxia by apgar scores be supported by other investigations so that early decision can be taken about the level of care the baby needs. pH, lactates and base deficits change with establishment of respiration

  16. Genetics Home Reference: STXBP1 encephalopathy with epilepsy

    MedlinePlus

    ... Conditions STXBP1 encephalopathy with epilepsy STXBP1 encephalopathy with epilepsy Printable PDF Open All Close All Enable Javascript ... the expand/collapse boxes. Description STXBP1 encephalopathy with epilepsy is a condition characterized by recurrent seizures (epilepsy), ...

  17. Treating perinatal asphyxia with theophylline at birth helps to reduce the severity of renal dysfunction in term neonates.

    PubMed

    Raina, Alok; Pandita, Aakash; Harish, Rekha; Yachha, Monika; Jamwal, Ashu

    2016-10-01

    Perinatal asphyxia is a common neonatal problem and contributes significantly to neonatal morbidity and mortality. This study was designed to determine whether theophylline could prevent or ameliorate renal dysfunction in term neonates with perinatal asphyxia. We randomised 159 severely asphyxiated term newborns to receive a single dose of 5 mg/kg intravenous theophylline (n = 78) or a placebo (n = 81) during the first hour of life. The infant's 24-hour fluid intake, urine volume, serum creatinine, creatinine clearance and sodium excretion were recorded during days one, three and five of life, starting 12 hours after the theophylline or placebo infusion. Neonates in the theophylline group had lower serum creatinine levels (0.83 ± 0.35 versus 1.47 ± 0.61; p = 0.00) and higher endogenous creatinine clearance (32.16 ± 16.34 versus 17.73 ± 7.92; p = 0.00) than the placebo group. Severe renal dysfunction, namely acute kidney injury, was present in 36 (15%) of the neonates in the theophylline group versus 117 (48%) in the placebo group (p < 0.01). A single dose of intravenous theophylline administered to term neonates with perinatal asphyxia within the first hour of life significantly decreased serum creatinine levels and significantly increased creatinine clearance. ©2016 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  18. Executive Functioning in Children and Adolescents With Perinatal HIV Infection.

    PubMed

    Nichols, Sharon L; Brummel, Sean S; Smith, Renee A; Garvie, Patricia A; Hunter, Scott J; Malee, Kathleen M; Kammerer, Betsy L; Wilkins, Megan L; Rutstein, Richard; Tassiopoulos, Katherine; Chernoff, Miriam C; Mellins, Claude A

    2015-09-01

    Perinatal HIV (PHIV) infection may place youth at risk for impairments in executive functioning (EF). We examined associations of EF with HIV infection, disease severity and other factors among youth with PHIV and perinatally HIV-exposed, uninfected youth (PHEU). Within the US-based Pediatric HIV/AIDS Cohort Study, 354 PHIV and 200 PHEU youth completed a standardized EF measure (Children's Color Trails Test, CCTT) and youth and/or caregivers completed a questionnaire measuring everyday EF (Behavior Rating Inventory of Executive Function, BRIEF). Covariates included HIV status, current and historical disease severity, demographic and caregiver variables and other cognitive measures. Analyses used linear and logistic regression and proportional odds models. No significant HIV status group differences were found on CCTT scores. Caregiver BRIEF ratings indicated significantly fewer problems for PHIV than PHEU youth. However, PHIV youth with past encephalopathy self-endorsed significantly greater metacognitive (ie, cognitive regulation) problems on the BRIEF and performed more slowly on the CCTT than PHEU youth. CCTT and caregiver BRIEF scores had significant associations with indicators of past and present disease severity. Both PHIV and PHEU had significantly worse scores than population means on CCTT and BRIEF; scores had significant associations with demographic covariates. Youth with PHIV show EF problems likely associated with risk factors other than HIV. However, cognitive slowing and self-reported metacognitive problems were evident in PHIV youth with a history of encephalopathy. Assessment and treatment of EF impairment may be important to identifying PHIV youth at particular risk for poor health and behavioral outcomes.

  19. Challenges in diagnosing hepatic encephalopathy.

    PubMed

    Weissenborn, K

    2015-02-01

    The term "hepatic encephalopathy" (HE) covers the neuropsychiatric syndrome associated with acute, chronic and acute-on-chronic liver disease (CLD). This paper deals with clinical features and diagnosis of HE in patients with liver cirrhosis and portal hypertension or porto-systemic shunts. The possible impact of concomitant disorders and the cirrhosis underlying liver disease upon brain function is described emphasizing the need of a detailed diagnostic work up of every individual case before diagnosing HE. Currently used methods for diagnosing minimal or covert hepatic encephalopathy are compared with regard to their sensitivity and specificity for diagnosing HE against the background of a multitude of concomitant disorders and diseases that could contribute to brain dysfunction.

  20. The posterior reversible encephalopathy syndrome.

    PubMed

    Sanjay, K Mandal; Partha, P Chakraborty

    2008-09-01

    The posterior/potentially reversible encephalopathy syndrome is a unique syndrome encountered commonly in hypertensive encephalopathy. A 13-year-old boy presented with of intermittent high grade fever, throbbing headache and non-projective vomiting for 5 days. The patient had a blood pressure of 120/80 mmHg but fundoscopy documented grade 3 hypertensive retinopathy. The patient improved symptomatically following conservative management. However, on the 5(th) post-admission day headache reappeared, and blood pressure measured at that time was 240/120 mmHg. Neuroimaging suggested white matter abnormalities. Search for the etiology of secondary hypertension led to the diagnosis of pheochromocytoma. Repeated MRI after successful surgical excision of the tumor patient showed reversal of white matter abnormalities. Reversible leucoencephalopathy due to pheochromocytoma have not been documented in literature previously.

  1. [Wernicke encephalopathy accompanying linitis plastica].

    PubMed

    Soós, Zsuzsanna; Salamon, Mónika; Oláh, Roland; Czégeni, Anna; Salamon, Ferenc; Folyovich, András; Winkler, Gábor

    2014-01-05

    Wernicke encephalopathy (or Wernicke-Korsakoff encephalopathy) is a rarely diagnosed neurological disorder, which is caused by vitamin B1 deficiency. In the classical form it is characterized by a typical triad (confusion, oculomotor disturbance and ataxia), however, in the majority of the cases only confusion is present. It can be frequently observed in subjects with chronic alcohol consumption, but it may accompany different pathological states of which end stage malignant diseases are the most importants, where confusion may have different backgrounds. The authors present the case of an old male patient with advanced gastric cancer recognised and treated vitamin B1 deficiency, and they draw attention to difficulties of the diagnosis of Wernicke's disease.

  2. Preterm Hypoxic–Ischemic Encephalopathy

    PubMed Central

    Gopagondanahalli, Krishna Revanna; Li, Jingang; Fahey, Michael C.; Hunt, Rod W.; Jenkin, Graham; Miller, Suzanne L.; Malhotra, Atul

    2016-01-01

    Hypoxic–ischemic encephalopathy (HIE) is a recognizable and defined clinical syndrome in term infants that results from a severe or prolonged hypoxic–ischemic episode before or during birth. However, in the preterm infant, defining hypoxic–ischemic injury (HII), its clinical course, monitoring, and outcomes remains complex. Few studies examine preterm HIE, and these are heterogeneous, with variable inclusion criteria and outcomes reported. We examine the available evidence that implies that the incidence of hypoxic–ischemic insult in preterm infants is probably higher than recognized and follows a more complex clinical course, with higher rates of adverse neurological outcomes, compared to term infants. This review aims to elucidate the causes and consequences of preterm hypoxia–ischemia, the subsequent clinical encephalopathy syndrome, diagnostic tools, and outcomes. Finally, we suggest a uniform definition for preterm HIE that may help in identifying infants most at risk of adverse outcomes and amenable to neuroprotective therapies. PMID:27812521

  3. Wernicke's encephalopathy following hyperemesis gravidarum

    PubMed Central

    Kantor, Sandeep; Prakash, Sadanandan; Chandwani, Juhi; Gokhale, Antara; Sarma, Kalpana; Albahrani, Maher J.

    2014-01-01

    Wernicke's encephalopathy (WE) is a potentially reversible yet serious neurological manifestation caused by vitamin B1(thiamine) deficiency. It is commonly associated with heavy alcohol consumption. Other clinical associations are with hyperemesis gravidarum (HG), starvation, and prolonged intravenous feeding. Most patients present with the triad of ocular signs, ataxia, and confusion. It can be associated with life-threatening complication like central pontine myelinolysis (CPM). We report two cases of WE following HG, with two different outcomes. PMID:24701066

  4. Encephalopathies: the emerging diabetic complications.

    PubMed

    Sima, Anders A F

    2010-12-01

    Diabetic encephalopathies are now accepted complications of diabetes. They appear to differ in type 1 and type 2 diabetes as to underlying mechanisms and the nature of resulting cognitive deficits. The increased incidence of Alzheimer's disease in type 2 diabetes is associated with insulin resistance, hyperinsulinemia and hyperglycemia, and commonly accompanying attributes such as hypercholesterolemia, hypertension and obesity. The relevance of these disorders as to the emergence of dementia and Alzheimer's disease is discussed based on epidemiological studies. The pathobiology of accumulation of β-amyloid and tau the hallmarks of Alzheimer's disease are discussed based on experimental data. Type 1 diabetic encephalopathy is likely to increase as a result of the global increase in the incidence of type 1 diabetes and its occurrence in increasingly younger patients. Alzheimer-like changes and dementia are not prominently increased in type 1 diabetes. Instead, the type 1 diabetic encephalopathy involves learning abilities, intelligence development and memory retrieval resulting in impaired school and professional performances. The major underlying component here appears to be insulin deficiency with downstream effects on the expression of neurotrophic factors, neurotransmitters, oxidative and apoptotic stressors resulting in defects in neuronal integrity, connectivity and loss commonly occurring in the still developing brain. Recent experimental data emphasize the role of impaired central insulin action and provide information as to potential therapies. Therefore, the underlying mechanisms resulting in diabetic encephalopathies are complex and appear to differ between the two types of diabetes. Major headway has been made in our understanding of their pathobiology; however, many questions remain to be clarified. In view of the increasing incidence of both type 1 and type 2 diabetes, intensified investigations are called for to expand our understanding of these

  5. Perinatal mortality in rural Tanzania.

    PubMed

    van Roosmalen, J

    1989-07-01

    Prolonged labour was the most frequent cause of perinatal death in a rural hospital in the south western highlands of Tanzania. After the introduction of an obstetric policy aiming to prevent prolonged labour by making use of the guidelines of the partogram, perinatal mortality was reduced from 71 to 39 per 1000 births. Baird's clinico-pathological classification is still considered a useful instrument for the discovery of avoidable factors in perinatal deaths. The concept of the partogram should be an integral part of the training of medical auxiliaries in the field of maternal and child health (MCH).

  6. Perinatal grief in Latino parents.

    PubMed

    Whitaker, Claudia; Kavanaugh, Karen; Klima, Carrie

    2010-01-01

    Extensive research exists that describes the meaning of perinatal loss to some parents, but the experience of loss from the perspective of Latino parents is not clearly understood. Additionally, current perinatal bereavement practices used often to facilitate memory making for parents (such as viewing or holding the baby, taking photographs, or collecting mementos) are based on research done primarily with non-Latino families. Are these common practices appropriate for this population? Because there is a paucity of research on this topic, this article describes what has been written over the past 30 years on the topic of grief and perinatal loss in Latino culture.

  7. Perinatal Grief in Latino Parents

    PubMed Central

    Whitaker, Claudia; Kavanaugh, Karen; Klima, Carrie

    2013-01-01

    Extensive research exists that describes the meaning of perinatal loss to some parents, but the experience of loss from the perspective of Latino parents is not clearly understood. Additionally, current perinatal bereavement practices used often to facilitate memory-making for parents (such as viewing or holding the baby, taking photographs, or collecting mementos) are based upon research done primarily with non-Latino families. Are these common practices appropriate for this population? Because there is a paucity of research on this topic, this article describes what has been written over the past 30 years on the topic of grief and perinatal loss in Latino culture. PMID:20975393

  8. The Nuss technique for Jeune asphyxiating thoracic dystrophy repair in siblings.

    PubMed

    Kikuchi, Noriaki; Kashiwa, Hideo; Ogino, Toshihoko; Kato, Mituhiro; Hayasaka, Kiyoshi

    2010-08-01

    Jeune syndrome, or asphyxiating thoracic dysplasia, is an autosomal recessive osteochondrodysplasia. Four forms of Jeune syndrome have been proposed: lethal, severe, mild, and latent. In the severe form, respiratory failure leads to death in early infancy. We present 2 cases of a mild variant of Jeune syndrome in a 14-year-old girl and her 9-year-old brother, who were referred to us because of characteristic concave deformities of bilateral middle-lower chest walls without cardiopulmonary distress or renal failure. In addition, both showed short statue (-2.1 and -2.5 SD), progressive retinal dystrophy, and metaphyseal dysplasia (cone-shaped metaphysis and metacarpal brachydactyly). The chest wall deformity was treated at the age of 9 years in the sister and at the age of 7 years in the brother. According to the Nuss procedure, 2 bent bars were inserted into the thoracic cavity from each lateral intercostal space at the midaxillary line and pulled out over the lower end of the sternum. The ends of inserted bars were fixed to soft tissue over the sternum with nonabsorbable sutures. Conjoined costal cartilage around the sternum restricts the number of bars that can be positioned. A single bar for deformity of each side could not achieve complete reconstruction, but the patients and their parents were satisfied with the results cosmetically.

  9. [Relation between PMI and FTIR spectral changes in asphyxiated rat's liver and spleen].

    PubMed

    Li, Shi-ying; Shao, Yu; Li, Zheng-dong; Zou, Dong-hua; Qin, Zhi-qiang; Chen, Yi-jiu; Huang, Ping

    2012-10-01

    Fourier transform infrared (FTIR) spectroscopy was applied to observe the postmortem degradation process in mechanical asphyxiated rat's liver and spleen for providing a new method of estimating PMI. Rats were sacrificed by mechanical asphyxia and cadavers were kept at (20 +/- 2) degrees C in a control chamber. The liver and spleen were sub-sampled from the same rat at intervals of 0-15 days postmortem and the data were measured by FTIR spectrometer. The different absorbance (A) ratios of peaks were calculated and the curve estimation analysis between absorbance ratios (x) and PMI (y) were performed to establish mathematical models by the statistical software. The band absorbance ratios showed increase, decrease and stable with PMI. The cubic model functions showed the strongest correlation coefficient. Compared with the spleen, the liver showed a higher correlation coefficient. The A1541/A1396 of liver showed the highest correlation coefficient (r=0.966). After 6-7 days postmortem, band absorbance ratios showed a steady period. FTIR spectroscopy can be a new and efficient method to estimate PMI within 7 days.

  10. Occupational asphyxiation by unknown compound(s): environmental and toxicological approach.

    PubMed

    Poli, D; Solarino, B; Di Vella, G; Tattoli, L; Strisciullo, G; Goldoni, M; Mutti, A; Gagliano-Candela, R

    2010-04-15

    During a routine truck-tank washing operation, five healthy workers were found motionless inside an empty tanker. Four of them died inside the tanker while the fifth died the following day in hospital. Since the true nature of the fatal compound(s) were not known, a rigorous environmental and toxicological approach supported by autopsy findings was essential to clarify the cause of death. Environmental results indicated that H(2)S fumes arising from the liquid sulfur previously shipped were responsible for the serial deaths, also confirmed by a simulation performed on two similar truck-tanks. These environmental findings were supported by toxicological analyses through the measurement of thiosulfate, one of the main H(2)S metabolites. Abnormal thiosulfate concentrations from 1.1 to 186.2 mg/kg were revealed in all post-mortem biological samples (blood, lung, liver, kidney, brain and fat). Finally, the cluster analysis performed on thiosulfate body distribution contributed to establishing the time of death according to the accident scene reconstruction. This report presents valuable findings in correctly identifying the cause of death in gas asphyxiation cases by unknown compound(s).

  11. One oxygen breath shortened the time to return of spontaneous circulation in severely asphyxiated piglets.

    PubMed

    Linner, Rikard; Cunha-Goncalves, Doris; Perez-de-Sa, Valeria

    2017-10-01

    Asphyxiated neonates should be resuscitated with air, but it remains unclear if oxygen supplementation is needed in ineffectively ventilated newborn infants. We studied the return of spontaneous circulation (ROSC) with oxygen or air in an experimental model of inadequate ventilation. Asphyxia was induced in 16 newborn piglets until their heart rate was <60 bpm or mean arterial pressure (MAP) <30 mmHg. During the first 10 minutes of resuscitation, they received one breath per minute of oxygen (n = 8) or air (n = 8). Tidal volume was 7.5 mL/kg. If MAP was <30 mmHg for 15 seconds, closed-chest cardiac massage (CCCM) was performed for 45 seconds. From 10 minutes onward, all piglets received normal ventilation with air. ROSC was defined as a heart rate >150 bpm, MAP >40 mmHg and no subsequent CCCM. Before resuscitation, the median arterial pH was 6.73. At 10 minutes, no piglets in the oxygen group needed CCCM, while all did in the air group (p < 0.001). The median time to ROSC was 60 seconds with oxygen and 845 seconds with air (p < 0.001). No brain tissue hyperoxia occurred. When ventilation was inadequate, one oxygen breath reduced time to ROSC in piglets with severe metabolic and respiratory acidosis. ©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  12. Mutations in the gene encoding IFT dynein complex component WDR34 cause Jeune asphyxiating thoracic dystrophy.

    PubMed

    Schmidts, Miriam; Vodopiutz, Julia; Christou-Savina, Sonia; Cortés, Claudio R; McInerney-Leo, Aideen M; Emes, Richard D; Arts, Heleen H; Tüysüz, Beyhan; D'Silva, Jason; Leo, Paul J; Giles, Tom C; Oud, Machteld M; Harris, Jessica A; Koopmans, Marije; Marshall, Mhairi; Elçioglu, Nursel; Kuechler, Alma; Bockenhauer, Detlef; Moore, Anthony T; Wilson, Louise C; Janecke, Andreas R; Hurles, Matthew E; Emmet, Warren; Gardiner, Brooke; Streubel, Berthold; Dopita, Belinda; Zankl, Andreas; Kayserili, Hülya; Scambler, Peter J; Brown, Matthew A; Beales, Philip L; Wicking, Carol; Duncan, Emma L; Mitchison, Hannah M

    2013-11-07

    Bidirectional (anterograde and retrograde) motor-based intraflagellar transport (IFT) governs cargo transport and delivery processes that are essential for primary cilia growth and maintenance and for hedgehog signaling functions. The IFT dynein-2 motor complex that regulates ciliary retrograde protein transport contains a heavy chain dynein ATPase/motor subunit, DYNC2H1, along with other less well functionally defined subunits. Deficiency of IFT proteins, including DYNC2H1, underlies a spectrum of skeletal ciliopathies. Here, by using exome sequencing and a targeted next-generation sequencing panel, we identified a total of 11 mutations in WDR34 in 9 families with the clinical diagnosis of Jeune syndrome (asphyxiating thoracic dystrophy). WDR34 encodes a WD40 repeat-containing protein orthologous to Chlamydomonas FAP133, a dynein intermediate chain associated with the retrograde intraflagellar transport motor. Three-dimensional protein modeling suggests that the identified mutations all affect residues critical for WDR34 protein-protein interactions. We find that WDR34 concentrates around the centrioles and basal bodies in mammalian cells, also showing axonemal staining. WDR34 coimmunoprecipitates with the dynein-1 light chain DYNLL1 in vitro, and mining of proteomics data suggests that WDR34 could represent a previously unrecognized link between the cytoplasmic dynein-1 and IFT dynein-2 motors. Together, these data show that WDR34 is critical for ciliary functions essential to normal development and survival, most probably as a previously unrecognized component of the mammalian dynein-IFT machinery.

  13. Metabolic Causes of Epileptic Encephalopathy

    PubMed Central

    Pearl, Phillip L.

    2013-01-01

    Epileptic encephalopathy can be induced by inborn metabolic defects that may be rare individually but in aggregate represent a substantial clinical portion of child neurology. These may present with various epilepsy phenotypes including refractory neonatal seizures, early myoclonic encephalopathy, early infantile epileptic encephalopathy, infantile spasms, and generalized epilepsies which in particular include myoclonic seizures. There are varying degrees of treatability, but the outcome if untreated can often be catastrophic. The importance of early recognition cannot be overemphasized. This paper provides an overview of inborn metabolic errors associated with persistent brain disturbances due to highly active clinical or electrographic ictal activity. Selected diseases are organized by the defective molecule or mechanism and categorized as small molecule disorders (involving amino and organic acids, fatty acids, neurotransmitters, urea cycle, vitamers and cofactors, and mitochondria) and large molecule disorders (including lysosomal storage disorders, peroxisomal disorders, glycosylation disorders, and leukodystrophies). Details including key clinical features, salient electrophysiological and neuroradiological findings, biochemical findings, and treatment options are summarized for prominent disorders in each category. PMID:23762547

  14. [Clinical characteristics of bilirubin encephalopathy in Chinese newborn infants-a national multicenter survey].

    PubMed

    2012-05-01

    Bilirubin encephalopathy continued to be reported worldwide. This multicenter study was conducted to explore the clinical characteristics, comorbidities and prognosis of bilirubin encephalopathy in China. The survey was conducted in 33 level III hospitals. Clinical charts of infants with diagnosis of bilirubin encephalopathy or kernicterus at discharge were reviewed. The data were collected by a detaild questionnaire and analyzed. From January to December in 2009, 348 cases of bilirubin encephalopathy were reported from 28 hospitals. The mean birth weight was (3112.4 ± 599.6) grams, mean gestational age was (38.3 ± 2.3) weeks; 291 (83.6%) cases were term infants, 40 (11.5%) cases were late-preterm infants, and 11 (3.2%) cases were early preterm infants. After admission, the highest bilirubin level was (478.1 ± 175.8) µmol/L. Of all the 348 cases, the age at admission was (7.3 ± 5.4) days; 247 (71.0%) cases were admitted before 7 days of age, 24 (6.9%) cases were admitted after 14 days of age. Most of the cases (86.2%) were complicated with other conditions, including bacterial infection (52.6%), ABO incompatibility (29.9%), and perinatal asphyxia (10.1%). A total of 131 infants (37.6%) underwent an exchange transfusion. Use of albumin, intravenous immunoglobulin was also common (68.7% and 44.0% respectively). A total of 52 cases were of minority ethnic groups, with significantly higher rate of G6PD deficiency than Han ethnicity cases. During the hospitalization and follow up, 36 infants died, and 125 infants (35.9%) were lost to follow up. Bilirubin encephalopathy is still not rare in China, the establishment of a population-based reporting system and prevention of kernicterus remain a high priority among public health institutions.

  15. Brainstem tegmental lesions in neonates with hypoxic-ischemic encephalopathy: Magnetic resonance diagnosis and clinical outcome

    PubMed Central

    Quattrocchi, Carlo Cosimo; Fariello, Giuseppe; Longo, Daniela

    2016-01-01

    Lesions of the brainstem have been reported in the clinical scenarios of hypoxic-ischemic encephalopathy (HIE), although the prevalence of these lesions is probably underestimated. Neuropathologic studies have demonstrated brainstem involvement in severely asphyxiated infants as an indicator of poor outcome. Among survivors to HIE, the most frequent clinical complaints that may be predicted by brainstem lesions include feeding problems, speech, language and communication problems and visual impairments. Clinical series, including vascular and metabolic etiologies, have found selective involvement of the brainstem with the demonstration of symmetric bilateral columnar lesions of the tegmentum. The role of brainstem lesions in HIE is currently a matter of debate, especially when tegmental lesions are present in the absence of supra-tentorial lesions. Differential diagnosis of tegmental lesions in neonates and infants include congenital metabolic syndromes and drug-related processes. Brainstem injury with the presence of supratentorial lesions is a predictor of poor outcome and high rates of mortality and morbidity. Further investigation will be conducted to identify specific sites of the brainstem that are vulnerable to hypoxic-ischemic and toxic-metabolic insults. PMID:26981220

  16. Comparison of early and late MRI in neonatal hypoxic-ischemic encephalopathy using three assessment methods.

    PubMed

    Charon, Valérie; Proisy, Maïa; Ferré, Jean-Christophe; Bruneau, Bertrand; Tréguier, Catherine; Beuchée, Alain; Chauvel, Jennifer; Rozel, Céline

    2015-12-01

    There is no consensus on the optimum timing of MRI in neonates with hypoxic-ischemic encephalopathy treated with hypothermia. Reliable early imaging assessment might help managing treatment. To assess non-random differences between early and late MRI that might influence intensive-care decisions. This single-center retrospective study included all asphyxiated term neonates eligible for hypothermia treatment November 2009-July 2012. MRI scans were systematically performed at day 4 (early MRI) and day 11 of life as part of routine protocol. Two experienced pediatric radiologists reviewed both scans according to three assessment methods: a pattern classification, a scoring system and a simplified classification. Agreement between early and late imaging findings was assessed using Cohen's kappa coefficients. Thirty-three neonates were included. Interobserver agreement was excellent. Early MRI detected all severe injuries. Agreement between early and late MRI was excellent for the simplified classification (κ = 0.82), good for the pattern classification (κ = 0.64), and good to excellent for 3 scores out of 4 in the scoring system (κ = 0.70-0.89). Early MRI may provide valuable information about brain injury to help parents and neonatologists in intensive-care decisions at the end of hypothermia treatment.

  17. Perinatal psychiatric disorders: an overview.

    PubMed

    Paschetta, Elena; Berrisford, Giles; Coccia, Floriana; Whitmore, Jennifer; Wood, Amanda G; Pretlove, Sam; Ismail, Khaled M K

    2014-06-01

    Perinatal mental illness has a significant implication on maternal health, birth outcomes, and the offspring's development. Prevalence estimates of perinatal psychiatric illnesses range widely, with substantial heterogeneity in different population studies, with a lower prevalence rate in high- rather than low- or middle-income countries. Because of the potential negative impact on maternal and child outcomes and the potential lability of these disorders, the perinatal period is a critical time to identify psychiatric illnesses. Thus, obstetricians and midwives play a crucial role in assessing women's mental health needs and to refer identified women promptly for multidisciplinary specialist assessment. However, there is still limited evidence on best practice assessment and management policies during pregnancy and postpartum. This review focuses on the prevalence of common perinatal mental disorders and antenatal screening policies to identify women at risk. The effect of these conditions and their management on pregnancy, fetal outcomes, and child development are discussed. Copyright © 2014 Mosby, Inc. All rights reserved.

  18. Neuroprotective treatment for perinatal asphyxia.

    PubMed

    Solevåg, Anne Lee; Nakstad, Britt

    2012-11-12

    Perinatal asphyxia can cause serious illness or death. By taking steps in the «latent phase», which occurs 6-24 hours after the hypoxic event, the neurological damage caused by perinatal asphyxia can be limited. We wish to present a selection of such measures that are either established treatment today or that appear promising. We searched in the Medline and Cochrane Library databases for options for treating perinatal asphyxia. An overwhelming number of potential treatments were identified. From among them we selected 44 indexed, peer-reviewed original articles in English on strategies for neuroprotective treatment after perinatal asphyxia. The treatments target different cellular mechanisms that cause neurological damage following perinatal asphyxia. In randomised clinical trials, only hypothermia treatment has improved the long-term outcome for newborns with perinatal asphyxia. Xenon gas, erythropoeitin and allopurinol are undergoing clinical testing. The efficacy of xenon gas, erythropoeitin and allopurinol in combination with the established treatment form of hypothermia must be studied more closely. Antioxidants, stem cell treatment and DNA repair mechanisms can pave the way for new opportunities in the future.

  19. Countrywide analysis of perinatal outcome.

    PubMed

    Stembera, Z; Kravka, A; Mandys, F

    1988-01-01

    The computer laboratory of the Research Institute for the Care of Mother and Child in Prague performs annually a countrywide analysis of perinatal outcome in order to obtain a background for the preparation of the optimal strategy for improving perinatal care in CSR in the future. The total as well as weight specific perinatal mortality rate further sub-divided into early neonatal death rate and late fetal death rate and differentiated according to the birthweight, was correlated with the incidence of different factors influencing the perinatal mortality rate both countrywide and for each of the eight provinces of CSR. This way a correlation was found between some of the mentioned perinatal outcomes and e.g. instrumental equipment of obstetrical departments and neonatal intensive care units, frequency of caesarean sections, or transport of LBW newborns in incubators or "in utero" etc. The results of this analysis have proved that there still remain in some provinces opportunity for further decrease in perinatal mortality due to the incomplete observance of the two intervention strategies "Risk approach" and "New technology" which were introduced in the whole country during the last 10 years.

  20. Rifaximin in the treatment of hepatic encephalopathy

    PubMed Central

    Iadevaia, Maddalena Diana; Prete, Anna Del; Cesaro, Claudia; Gaeta, Laura; Zulli, Claudio; Loguercio, Carmelina

    2011-01-01

    Hepatic encephalopathy is a challenging complication in patients with advanced liver disease. It can be defined as a neuropsychiatric syndrome caused by portosystemic venous shunting, ranging from minimal to overt hepatic encephalopathy or coma. Its pathophysiology is still unclear, although increased levels of ammonia play a key role. Diagnosis of hepatic encephalopathy is currently based on specific tests evaluating the neuropsychiatric state of patients and their quality of life; the severity of hepatic encephalopathy is measured by the West Haven criteria. Treatment of hepatic encephalopathy consists of pharmacological and corrective measures, as well as nutritional interventions. Rifaximin received approval for the treatment of hepatic encephalopathy in 2010 because of its few side effects and pharmacological benefits. The aim of this work is to review the use and efficacy of rifaximin both in acute and long-term management of hepatic encephalopathy. Treatment of overt hepatic encephalopathy involves management of the acute episode as well as maintenance of remission in those patients who have previously experienced an episode, in order to improve their quality of life. The positive effect of rifaximin in reducing health care costs is also discussed. PMID:24367227

  1. Diagnostic and prognostic factors for acute encephalopathy.

    PubMed

    Motojima, Yukiko; Nagura, Michiaki; Asano, Yoshitaka; Arakawa, Hiroshi; Takada, Eiko; Sakurai, Yoshio; Moriwaki, Koichi; Tamura, Masanori

    2016-11-01

    Acute encephalopathy has the possibility of sequelae. While early treatment is required to prevent the development of sequelae, differential diagnosis is of the utmost priority. The aim of this study was therefore to identify parameters that can facilitate early diagnosis and prediction of outcome of acute encephalopathy. We reviewed the medical charts of inpatients from 2005 to 2011 and identified 33 patients with febrile status epilepticus. Subjects were classified into an acute encephalopathy group (n = 20) and a febrile convulsion group (n = 13), and the parameters serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), lactate dehydrogenase (LDH), ammonia (NH3 ), cerebrospinal fluid (CSF) tau protein, and CSF interleukin-6 compared between them. Furthermore, the relationship between each parameter and prognosis was investigated in the encephalopathy group. Significant differences in serum AST, ALT, and LDH were observed between the febrile convulsion and acute encephalopathy group. Moreover, a significant difference in serum LDH was noted between the patients with and without developmental regression at the time of hospital discharge in the encephalopathy group. In particular, CSF tau protein was found to be highly likely to indicate progress, with CSF tau protein >1000 pg/dL associated with poor prognosis leading to developmental regression. Serum AST, ALT and LDH may be related to early diagnosis and prognosis, and should be carefully investigated in patients with encephalopathy. CSF tau protein could also be used as an indicator of poor prognosis in acute encephalopathy. © 2016 Japan Pediatric Society.

  2. Spongiform Encephalopathy in a Miniature Zebu

    PubMed Central

    Botteron, Catherine; Wenker, Christian; Café-Marçal, Valeria; Oevermann, Anna; Haase, Bianca; Leeb, Tosso; Heim, Dagmar; Zurbriggen, Andreas

    2006-01-01

    The first case of spongiform encephalopathy in a zebu (Bos indicus) was identified in a zoo in Switzerland. Although histopathologic and immunohistochemical analyses of the central nervous system indicated a diagnosis of bovine spongiform encephalopathy (BSE), molecular typing showed some features different from those of BSE in cattle (B. taurus). PMID:17326950

  3. Effect of co-medication on the pharmacokinetic parameters of phenobarbital in asphyxiated newborns.

    PubMed

    Šíma, M; Pokorná, P; Hronová, K; Slanař, O

    2015-01-01

    Phenobarbital is an anticonvulsive drug widely used in newborns with hypoxic-ischemic encephalopathy. The objective of our study was to describe possible effect of frequently co-administered medications (dopamine, dobutamine, norepinephrine, furosemide, phenytoin, and analgesics) on the phenobarbital pharmacokinetics in full term newborns with hypoxic-ischemic encephalopathy. Phenobarbital pharmacokinetic parameters (standardized intravenous loading dose was 10-20 mg/kg, maintenance dose 2-6 mg/kg/day) were computed using non-compartmental analysis. Co-medication was evaluated throughout the whole treatment period up to 5 days. Volume of distribution, clearance, and half-life median values (95 % CI) for phenobarbital in the whole study population (n=37) were 0.48 (0.41-0.56) l/kg, 0.0034 (0.0028-0.0040) l/h/kg, and 93.7 (88.1-99.2) h, respectively. Phenobarbital pharmacokinetic parameters were not significantly affected by vasoactive drugs (dopamine, dobutamine, and norepinephrine), furosemide, phenytoin, or analgesics. Furthermore, no dose-dependent alteration of phenobarbital pharmacokinetic parameters was noted for vasoactive medication at doses equivalent to cumulative vasoactive-inotropic score (area under the curve in a plot of vasoactive-inotropic score against time) 143.2-8473.6, furosemide at cumulative doses of 0.2-42.9 mg/kg, or phenytoin at cumulative doses of 10.3-46.2 mg/kg. Phenobarbital pharmacokinetics was not affected by investigated co-administered drugs used in newborns with hypoxic-ischemic encephalopathy in real clinical settings.

  4. Hashimoto's encephalopathy in children and adolescents.

    PubMed

    Erol, Ilknur; Saygi, Semra; Alehan, Füsun

    2011-12-01

    Hashimoto's encephalopathy is an underdiagnosed, steroid-responsive, progressive or relapsing encephalopathy associated with high titers of serum antithyroid antibodies. Although Hashimoto's encephalopathy is well documented in adults, it is rarely observed or studied in children and adolescents. We describe the clinical and laboratory findings of four children (aged 9-15 years) with Hashimoto's encephalopathy. The clinical features of two patients at presentation included epileptic seizures and confusion. The other presenting signs included breath-holding spells, behavioral problems, psychosis, and ataxia (one patient each). During their presentation, three patients were euthyroid, and one was hyperthyroid. All patients manifested increased antithyroid antibodies, and all improved with steroid treatment. Hashimoto's encephalopathy is rarely suspected at presentation. Therefore, greater awareness of its signs by clinicians is necessary for proper diagnoses.

  5. Different Respiratory Rates during Resuscitation in a Pediatric Animal Model of Asphyxial Cardiac Arrest.

    PubMed

    López, Jorge; Fernández, Sarah N; González, Rafael; Solana, María J; Urbano, Javier; López-Herce, Jesús

    2016-01-01

    Actual resuscitation guidelines recommend 10 respirations per minute (rpm) for advanced pediatric life support. This respiratory rate (RR) is much lower than what is physiological for children. The aim of this study is to compare changes in ventilation, oxygenation, haemodynamics and return of spontaneous circulation (ROSC) rates with three RR. An experimental model of asphyxial cardiac arrest (CA) in 46 piglets (around 9.5 kg) was performed. Resuscitation with three different RR (10, 20 and 30 rpm) was carried out. Haemodynamics and gasometrical data were obtained at 3, 9, 18 and 24 minutes after beginning of resuscitation. Measurements were compared between the three groups. No statistical differences were found in ROSC rate between the three RR (37.5%, 46.6% and 60% in the 10, 20 and 30 rpm group respectively P = 0.51). 20 and 30 rpm groups had lower PaCO2 values than 10 rpm group at 3 minutes (58 and 55 mmHg vs 75 mmHg P = 0.08). 30 rpm group had higher PaO2 (61 mmHg) at 3 minutes than 20 and 10 rpm groups (53 and 45 mmHg P = 0.05). No significant differences were found in haemodynamics or tissue perfusion between hyperventilated (PaCO2 <30 mmHg), normoventilated (30-50 mmHg) and hypoventilated (>50 mmHg) animals. PaO2 was significantly higher in hyperventilated (PaO2 153 mmHg) than in normoventilated (79 mmHg) and hypoventilated (47 mmHg) piglets (P<0.001). Our study confirms the hypothesis that higher RR achieves better oxygenation and ventilation without affecting haemodynamics. A higher RR is associated but not significantly with better ROSC rates.

  6. Different Respiratory Rates during Resuscitation in a Pediatric Animal Model of Asphyxial Cardiac Arrest

    PubMed Central

    López, Jorge; Fernández, Sarah N.; González, Rafael; Solana, María J.; Urbano, Javier; López-Herce, Jesús

    2016-01-01

    Aims Actual resuscitation guidelines recommend 10 respirations per minute (rpm) for advanced pediatric life support. This respiratory rate (RR) is much lower than what is physiological for children. The aim of this study is to compare changes in ventilation, oxygenation, haemodynamics and return of spontaneous circulation (ROSC) rates with three RR. Methods An experimental model of asphyxial cardiac arrest (CA) in 46 piglets (around 9.5 kg) was performed. Resuscitation with three different RR (10, 20 and 30 rpm) was carried out. Haemodynamics and gasometrical data were obtained at 3, 9, 18 and 24 minutes after beginning of resuscitation. Measurements were compared between the three groups. Results No statistical differences were found in ROSC rate between the three RR (37.5%, 46.6% and 60% in the 10, 20 and 30 rpm group respectively P = 0.51). 20 and 30 rpm groups had lower PaCO2 values than 10 rpm group at 3 minutes (58 and 55 mmHg vs 75 mmHg P = 0.08). 30 rpm group had higher PaO2 (61 mmHg) at 3 minutes than 20 and 10 rpm groups (53 and 45 mmHg P = 0.05). No significant differences were found in haemodynamics or tissue perfusion between hyperventilated (PaCO2 <30 mmHg), normoventilated (30–50 mmHg) and hypoventilated (>50 mmHg) animals. PaO2 was significantly higher in hyperventilated (PaO2 153 mmHg) than in normoventilated (79 mmHg) and hypoventilated (47 mmHg) piglets (P<0.001). Conclusions Our study confirms the hypothesis that higher RR achieves better oxygenation and ventilation without affecting haemodynamics. A higher RR is associated but not significantly with better ROSC rates. PMID:27618183

  7. Neonatal parechovirus leucoencephalitis- radiological pattern mimicking hypoxic-ischemic encephalopathy.

    PubMed

    Amarnath, C; Helen Mary, T; Periakarupan, A; Gopinathan, K; Philson, J

    2016-02-01

    Our objective is to study the MR imaging pattern in neonatal parechoviral leucoencephalitis, a rare cause of neonatal white matter abnormality and to differentiate it from hypoxic-ischemic encephalopathy which is the commonest cause of white matter change in neonates. We evaluated 25 neonates who presented with features of encephalopathy. Cranial ultrasound and MR imaging was done in all the cases. The pattern of white matter abnormality was analyzed in all cases. Neonatal leucoencephalitis caused by HPeV has a distinctive clinical presentation and has predilection for the white matter, causing diffusion restricting signal intensity changes involving the periventricular and subcortical white matter, in particular the frontal white matter, also the corpus callosum, internal capsule, external capsule and pyramidal tracts of the supratentorial brain and cerebral peduncle with relative sparing of occipital white matter, thalamus, basal ganglia and the infratentorial regions. Follow up imaging shows disappearance of the lesion without white matter loss. Whereas mild to moderate hypoxic-ischemic injury in a full-term neonate causes lesions in the watershed areas, and subcortical white matter predominantly involving the parietooccipital region and perirolandic region. Thalamus, brainstem, cerebellum, and deep gray matter structures are involved depending on the severity. White matter changes in the neonatal period are commonly associated with hypoxic-ischemic injuries and metabolic causes, less frequently, infection like parechovirus leucoencephalitis. HPeV infection must be considered in infants with specific pattern of white matter change but no convincing history of a perinatal hypoxic-ischemic insult, thus differentiating it from HIE. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  8. The EEG of tropical encephalopathies.

    PubMed

    Mallewa, Macpherson; Birbeck, Gretchen L

    2013-10-01

    In addition to encountering most of the conditions treated by clinicians in the West, clinicians in the tropics are faced with unique tropical encephalopathies. These are largely but not entirely infectious in nature. Despite the relatively low cost of EEG technology, it remains unavailable in many low-income tropical settings even at the tertiary care level. Where available, the EEG recordings and interpretation are often of unacceptable quality. Nonetheless, there are existing data on the EEG patterns seen in malaria and a number of tropical viral, bacterial, and parasitic infestations.

  9. [Wernicke encephalopathy in alcoholic patients].

    PubMed

    Chamorro Fernández, A J; Marcos Martín, M; Laso Guzmán, F J

    2011-10-01

    A 67-year old male was brought to the hospital by his family because he had been suffering from somnolence, bradypsychia and gait disturbance for one week. He lived alone, reported an ethanol intake higher than 100-120 g/day. His diet was limited in quality and amount. The physical examination showed stigmata of chronic liver disease. The neurological exam revealed right-side cerebellar tremor, bilateral dysmetria and gait ataxia as well as hyporeflexia in the lower limbs. He was diagnosed of Wernicke encephalopathy. How should this patient be evaluated and treated?

  10. Perinatal grief and mourning.

    PubMed

    Menke, J A; McClead, R E

    1990-01-01

    The grief and mourning that parents experience following a perinatal loss is as devastating as the loss of an older loved one. The pattern of mourning can be anticipated and interventions can be implemented. With proper help, the parents can pass through this catastrophic time in their lives with a minimum of scars. If the physician stops, reaches out, listens, and supports the parents, he or she can have a dramatic effect on the lives of these parents. In the same manner in which we started this paper, we close with a quotation from another parent who suffered a loss: Daughters may die, But why? For even daughters can't live with half a heart. Three days isn't much a life. But long enough to remember thin blue lips, uneven gasps in incubators, Racking breaths that cause a pain to those who watched. Long enough to remember I never held her Or felt her softness Or counted her toes. I didn't even know the color of her eyes. Dead paled hands not quite covered by the gown she Was to go home in. Moist earth smell. One small casket. And the tears. You see, I hold in my hand but souvenirs of an occasion. A sheet of paper filled with statistics, A certificate with smudged footprints, A tiny bracelet engraved "Girl, Smith." You say that you are sorry That you know how I feel. But you can't know because I don't feel. Not yet.

  11. Perinatal programming prevention measures.

    PubMed

    Larguía, A Miguel; González, María Aurelia; Dinerstein, Néstor Alejandro; Soto Conti, Constanza

    2015-01-01

    Over the past 10 years, there has been outstanding scientific progress related to perinatal programming and its epigenetic effects in health, and we can anticipate this trend will continue in the near future. We need to make use and apply these achievements to human neurodevelopment via prevention interventions. Based on the concept of the interaction between genome and ambiome, this chapter proposes low-cost easy-implementation preventive strategies for maternal and infant health institutions.Breastfeeding and human milk administration are the first preventive measures, as has been reviewed in the policy statement of the American Academy of Pediatrics. Another strategy is the Safe and Family-Centered Maternity Hospitals initiative that promotes and empowers the inclusion of the families and the respect for their rights, especially during pregnancy and birth. (This change of paradigm was approved and is recommended by both United Nations Children's Fund, UNICEF, and Pan American Health Organization, PAHO.) Then, there is also an important emphasis given to the sacred hour-which highlights the impact of bonding, attachment, and breastfeeding during the first hour of life-the pain prevention and treatment in newborns, the control of the "new morbidity" represented by late preterm infants, and finally, the importance of avoiding intrauterine and extrauterine growth restriction. (However, there are not yet clear recommendations about nutritional interventions in order to diminish the potential metabolic syndrome consequence in the adult.).

  12. [Perinatal obsessive-compulsive disorder].

    PubMed

    Mavrogiorgou, P; Illes, F; Juckel, G

    2011-09-01

    A perinatal obsessive-compulsive disorder (OCD) is defined as an illness exhibiting first symptoms in the context of pregnancy and the postpartal period. There are no valid data up to date concerning the incidence of OCD, which might be of multifactorial origin, in this period in which females are highly vulnerable for psychiatric diseases. From a clinical point of view, obsessions and compulsions are mainly related to the well-being of the foetus or newborn baby. Differential diagnosis of perinatal OCD including pregnancy psychosis and post-partum depression is often difficult. Concerning treatment, non-pharmacological approaches should be preferred. Administration of SSRIs should be strongly restricted. However, there are no controlled therapy studies in patients with perinatal OCD. Furthermore, current knowledge about these patients is still limited. The aim of this review article is the presentation of phenomenology, pathogenesis, differential diagnosis and treatment of perinatal OCD. The mental situation of the female patients can be improved and stabilised if early diagnosis of a perinatal OCD leads to early initiation of an adequate therapy. This will then enable a good and stable mother-child relationship to develop.

  13. Nutritional support in hepatic encephalopathy.

    PubMed

    Mizock, B A

    1999-03-01

    Hepatic encephalopathy (HE) is a syndrome of global cerebral dysfunction resulting from underlying liver disease or portal-systemic shunting. HE can present as one of four syndromes, depending on the rapidity of onset of hepatic failure and the presence or absence of preexisting liver disease. The precise pathogenesis is unknown but likely involves impaired hepatic detoxification of ammonia as well as alterations in brain transport and metabolism of amino acids and amines. The etiology of malnutrition in hepatic failure is multifactorial. Nutritional deficits may be clinically manifest as marasmus or kwashiorkor, or both. Nutritional support in HE is directed toward reducing morbidity related to underlying malnutrition and concurrent disease. However, reaching nutritional goals is often complicated by protein and carbohydrate intolerance. The use of protein restriction in HE is controversial. Modified formulas that are supplemented in branched chain amino acids may be of value in patients who exhibit protein intolerance with standard feeding solutions or in patients who present with advanced degrees of encephalopathy.

  14. Minimal hepatic encephalopathy: A review.

    PubMed

    Nardone, Raffaele; Taylor, Alexandra C; Höller, Yvonne; Brigo, Francesco; Lochner, Piergiorgio; Trinka, Eugen

    2016-10-01

    Minimal hepatic encephalopathy (MHE) is the earliest form of hepatic encephalopathy and can affect up to 80% of patients with liver cirrhosis. By definition, MHE is characterized by cognitive function impairment in the domains of attention, vigilance and integrative function, but obvious clinical manifestation are lacking. MHE has been shown to affect daily functioning, quality of life, driving and overall mortality. The diagnosis can be achieved through neuropsychological testing, recently developed computerized psychometric tests, such as the critical flicker frequency and the inhibitory control tests, as well as neurophysiological procedures. Event related potentials can reveal subtle changes in patients with normal neuropsychological performances. Spectral analysis of electroencephalography (EEG) and quantitative analysis of sleep EEG provide early markers of cerebral dysfunction in cirrhotic patients with MHE. Neuroimaging, in particular MRI, also increasingly reveals diffuse abnormalities in intrinsic brain activity and altered organization of functional connectivity networks. Medical treatment for MHE to date has been focused on reducing serum ammonia levels and includes non-absorbable disaccharides, probiotics or rifaximin. Liver transplantation may not reverse the cognitive deficits associated with MHE. We performed here an updated review on epidemiology, burden and quality of life, neuropsychological testing, neuroimaging, neurophysiology and therapy in subjects with MHE. Copyright © 2016 Elsevier Ireland Ltd and Japan Neuroscience Society. All rights reserved.

  15. Hashimoto's Encephalopathy Presenting with Acute Cognitive Dysfunction and Convulsion.

    PubMed

    Kang, Woo-Hyuk; Na, Ju-Young; Kim, Meyung-Kug; Yoo, Bong-Goo

    2013-12-01

    Hashimoto's encephalopathy is an immune-mediated disorder characterized by acute or subacute encephalopathy related to increased anti-thyroid antibodies. Clinical manifestations of Hashimoto's encephalopathy may include stroke-like episodes, altered consciousness, psychosis, myoclonus, abnormal movements, seizures, and cognitive dysfunction. Acute cognitive dysfunction with convulsion as initial clinical manifestations of Hashimoto's encephalopathy is very rare. We report a 65-year-old man who developed acute onset of cognitive decline and convulsion due to Hashimoto's encephalopathy.

  16. Defining encephalopathy in acute disseminated encephalomyelitis.

    PubMed

    Fridinger, S E; Alper, Gulay

    2014-06-01

    The International Pediatric Multiple Sclerosis Study Group requires the presence of encephalopathy to diagnose acute disseminated encephalomyelitis. Clinical characteristics of encephalopathy are inadequately delineated in the pediatric demyelinating literature. The authors' purpose was to better define encephalopathy in pediatric acute disseminated encephalomyelitis by describing the details of the mental status change. A retrospective chart review was conducted for 25 children diagnosed with acute disseminated encephalomyelitis according to the International Pediatric Multiple Sclerosis Study Group guidelines. Frequency of encephalopathy-defining features was determined. Clinical characteristics, cerebrospinal fluid findings, and electroencephalography (EEG) findings were compared between patients with different stages of encephalopathy. The authors found irritability (36%), sleepiness (52%), confusion (8%), obtundation (20%), and coma (16%) as encephalopathy-defining features in acute disseminated encephalomyelitis. Twenty-eight percent had seizures, and 65% demonstrated generalized slowing on EEG. Approximately half of the patients in this study were diagnosed with encephalopathy based on the presence of irritability and/or sleepiness only. Such features in young children are often subtle and transient and thus difficult to objectively determine.

  17. Temporal trends over the past two decades in asphyxial deaths in South Australia involving plastic bags or wrapping.

    PubMed

    Byard, Roger W; Simpson, Ellie; Gilbert, John D

    2006-01-01

    Asphyxial deaths utilising plastic bags or wrappings occurring over a 20-year period from March 1984 to February 2004 were reviewed at Forensic Science SA, Australia. A total of 45 cases were identified, with three occurring in infants and children (one accidental asphyxia; two homicides). Of the remaining 42 adults the male to female ratio was approximately 1:1 (23 and 19 cases, respectively), with all deaths attributed to suicide. The 42 adult cases represented 1.2% of the 3569 suicides autopsied at the centre over the time period of the study. The age ranges of the adult victims were 19-88 years (mean=47.1 years) for the males, and 32-89 years (mean=60.5 years) for the females. The adult female victims were significantly older than the males (p<0.001). A number of victims had histories of depression and had taken prescription medications. A significant difference was found in the temporal occurrence of the adult deaths, with six cases occurring between 1984 and 1989, nine between 1989 and 1994, 11 between 1994 and 1999, and 16 between 1999 and 2004 (p<0.001). Plastic bag asphyxial deaths were rare and in adults were due to suicide involving either older females or younger males. A significant increase in cases in South Australia in recent years was demonstrated, possibly related to publicity surrounding assisted suicides, and the ready availability of suicide manuals and information on suicide techniques from the internet.

  18. The "choking game": a new craze among Brazilian children and young people. Psychophysiological, behavioral and epidemiological characteristics of 'asphyxial games'.

    PubMed

    Guilheri, Juliana; Andronikof, Anne; Yazigi, Latife

    2017-03-01

    The 'choking game' is a risk-taking behavior that has spread quickly among children and young people, causing dependence, accidents and even death, including in Brazil. These activities are performed in order to experience fleeting euphoric sensations, attracting numerous participants through the thousands of videos posted on YouTube. The problem of 'asphyxial games' can be observed in the Brazilian digital media, although there is a lack of scientific studies. Through a systematic review of the literature and complementary material, this paper aims to address the 'asphyxial games', warning about the psychophysiological and behavioral effects of these practices, while also presenting international epidemiological data. Sharing this information in academic circles is extremely important given the need to acquire more knowledge on the topic, train professionals and propose preventive measures that raise awareness among children and young people of the potential danger of voluntary fainting. It is equally important to raise awareness among parents and teachers so they can identify the warning signs that children may be engaging in these practices. And finally, it is also necessary to request government support to control exposure to videos that encourage the behavior.

  19. Polynitroxyl albumin and albumin therapy after pediatric asphyxial cardiac arrest: effects on cerebral blood flow and neurologic outcome.

    PubMed

    Manole, Mioara D; Kochanek, Patrick M; Foley, Lesley M; Hitchens, T Kevin; Bayır, Hülya; Alexander, Henry; Garman, Robert; Ma, Li; Hsia, Carleton J C; Ho, Chien; Clark, Robert S B

    2012-03-01

    Postresuscitation cerebral blood flow (CBF) disturbances and generation of reactive oxygen species likely contribute to impaired neurologic outcome after pediatric cardiac arrest (CA). Hence, we determined the effects of the antioxidant colloid polynitroxyl albumin (PNA) versus albumin or normal saline (NS) on CBF and neurologic outcome after asphyxial CA in immature rats. We induced asphyxia for 9 minutes in male and female postnatal day 16 to 18 rats randomized to receive PNA, albumin, or NS at resuscitation from CA or sham surgery. Regional CBF was measured serially from 5 to 150 minutes after resuscitation by arterial spin-labeled magnetic resonance imaging. We assessed motor function (beam balance and inclined plane), spatial memory retention (water maze), and hippocampal neuronal survival. Polynitroxyl albumin reduced early hyperemia seen 5 minutes after CA. In contrast, albumin markedly increased and prolonged hyperemia. In the delayed period after resuscitation (90 to 150 minutes), CBF was comparable among groups. Both PNA- and albumin-treated rats performed better in the water maze versus NS after CA. This benefit was observed only in males. Hippocampal neuron survival was similar between injury groups. Treatment of immature rats with PNA or albumin resulted in divergent acute changes in CBF, but both improved spatial memory retention in males after asphyxial CA.

  20. Paroxysmal Amnesia Attacks due to Hashimoto's Encephalopathy

    PubMed Central

    Nar Senol, Pelin; Bican Demir, Aylin; Bora, Ibrahim; Bakar, Mustafa

    2016-01-01

    Hashimoto's encephalopathy is a rare disease which is thought to be autoimmune and steroid responsive. The syndrome is characterized by cognitive impairment, encephalopathy, psychiatric symptoms, and seizures associated with increased level of anti-thyroid antibodies. The exact pathophysiology underlying cerebral involvement is still lesser known. Although symptoms suggest a nonlesional encephalopathy in most of the cases, sometimes the clinical appearance can be subtle and may not respond to immunosuppressants or immunomodulatory agents. Here we report a case who presented with drowsiness and amnestic complaints associated with paroxysmal electroencephalography (EEG) abnormalities which could be treated only with an antiepileptic drug. PMID:27034679

  1. Wernicke encephalopathy in alcoholics with diabetic ketoacidosis.

    PubMed

    Chamorro, Antonio J; Marcos-Martin, Miguel; Martin-Polo, Jorge; Garcia-Diez, Luis Carlos; Luna, Guillermo

    2009-01-01

    Wernicke encephalopathy is caused by thiamine deficiency in the central nervous system, and is defined by the triad of confusional symptoms, ocular alterations and ataxia. Some other factors may also predispose alcoholic patients to this deficiency. We report two patients with hyperglicaemia and ketoacidosis due to diabetes mellitus decompensation and chronic alcoholism who developed Wernicke encephalopathy before their hospital admission. The outcome was successful after intravenous thiamine administration and insulinotherapy. The presence of Wernicke encephalopathy in alcoholics with diabetic ketoacidosis, suggests that metabolic decompensation is essential in the onset of the disease.

  2. Renal (uremic) encephalopathy in a goat.

    PubMed

    Radi, Z A; Thomsen, B V; Summers, B A

    2005-10-01

    Renal encephalopathy was diagnosed in a 2-year-old male boar goat with a history of chronic weight loss and ataxia. Histopathological examination of the brain revealed a striking myelin vacuolation distributed mainly in two patterns: (i) along the junction of the neocortex and corona radiata, and (ii) in the bundles of the internal capsule as it dissects through the basal nuclei. The kidneys had diffuse severe tubular and glomerular necrosis and degeneration. The neural lesions are consistent with renal (uremic) encephalopathy. To the authors' knowledge, this is the first report of renal encephalopathy in a goat.

  3. Perinatal outcomes in monoamniotic gestations.

    PubMed

    Roqué, H; Gillen-Goldstein, J; Funai, E; Young, B K; Lockwood, C J

    2003-06-01

    A comprehensive review of monoamniotic twin gestations reported between 1990 and 2002 was performed to estimate current perinatal mortality and morbidity rates, as well as the predictive value of an antenatal diagnosis of cord entanglement for poor obstetric outcomes. A Medline literature review using the search term 'monoamniotic' and limited to articles published in the English language between 1990 and 2002 was performed. A total of 133 continuing, non-conjoined twin monoamniotic pregnancies with delivery information were identified. Perinatal loss per 2-week interval was relatively constant at 2-4% from 15 to 32 weeks. However, of the 131 fetuses reaching 33 weeks, the percentage loss significantly increased to 11.0% at 33-35 weeks and 21.9% at 36-38 weeks compared to that at 30-32 weeks. Overall perinatal mortality was 23.3%. Of all losses, 61.2% involved both twins and 38.8% involved only one fetus. Cord entanglements were documented antenatally in 22.6% of reports. There was a statistically significant decrease in the average number of neonatal intensive care unit days for non-anomalous neonates (10.6 +/- 7.7 vs. 32.6 +/- 32.0), average gestational age at the time of delivery (30.4 +/- 7.6 vs. 32.6 +/- 4.1), as well as a decrease in the prevalence of total (8.3% vs. 27.7%) and non-anomalous (7.0% vs. 21.6%) perinatal mortality in pregnancies with an antenatal diagnosis of cord entanglement compared to those without the antenatal diagnosis of cord entanglement. The presence of fetal anomalies was associated with a 42.9% perinatal mortality rate. Contrary to previous reports, there is a significant increase in the incidence of perinatal loss beyond 32 weeks among monoamniotic twins, suggesting that delivery after corticosteroid therapy should be strongly considered at this gestational age.

  4. Flaxseed mitigates brain mass loss, improving motor hyperactivity and spatial memory, in a rodent model of neonatal hypoxic-ischemic encephalopathy.

    PubMed

    Mucci, Daniela de Barros; Fernandes, Flávia Spreafico; Souza, Amanda Dos Santos; Sardinha, Fátima Lúcia de Carvalho; Soares-Mota, Márcia; Tavares do Carmo, Maria das Graças

    2015-06-01

    Neonatal hypoxic-ischemic (HI) encephalopathy is a major cause of perinatal morbimortality. There is growing evidence that n-3 polyunsaturated fatty acids, especially docosahexaenoic acid (DHA), attenuate brain injury. This study aimed to investigate the possible neuroprotective effect of maternal intake of flaxseed, rich in DHA׳s precursor α-linolenic acid, in the young male offspring subjected to perinatal HI. Wistar rats were divided in six groups, according to maternal diet and offspring treatment at day 7: Control HI (CHI) and Flaxseed HI (FHI); Control Sham and Flaxseed Sham; Control Control and Flaxseed Control. Flaxseed diet increased offspring׳s hippocampal DHA content and lowered depressive behavior. CHI pups presented brain mass loss, motor hyperactivity and poor spatial memory, which were improved in FHI rats. Maternal flaxseed intake may prevent depressive symptoms in the offspring and promote neuroprotective effects, in the context of perinatal HI, improving brain injury and its cognitive and behavioral impairments.

  5. A new infectious encephalopathy syndrome, clinically mild encephalopathy associated with excitotoxicity (MEEX).

    PubMed

    Hirai, Nozomi; Yoshimaru, Daisuke; Moriyama, Yoko; Yasukawa, Kumi; Takanashi, Jun-Ichi

    2017-09-15

    Acute infectious encephalopathy is often observed in children in East Asia including Japan. More than 40% of the patients remain unclassified into specific syndromes. To investigate the underlying pathomechanisms in those with unclassified encephalopathy, we evaluated brain metabolism by MR spectroscopy. Among seven patients with acute encephalopathy admitted to our hospital from June 2016 to May 2017, three were classified into acute encephalopathy with biphasic seizures and late reduced diffusion (AESD). The other four showed consciousness disturbance lasting more than three days with no parenchymal lesion visible on MRI, which led to a diagnosis of unclassified encephalopathy. MR spectroscopy in these four patients, however, revealed an increase of glutamine with a normal N-acetyl aspartate level on days 5 to 8, which had normalized by follow-up studies on days 11 to 16. The four patients clinically recovered completely. Among 27 patients with encephalopathy, including the present seven patients, admitted to our hospital from January 2015 to March 2017, seven (26%) were classified into this type, which we propose is a new encephalopathy syndrome, clinically mild encephalopathy associated with excitotoxicity (MEEX). MEEX is the second most common subtype, following AESD (30%). This study suggests that excitotoxicity may be a common underlying pathomechanism of acute infectious encephalopathy, and prompt astrocytic neuroprotection from excitotoxicity may prevent progression of MEEX into AESD. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Perinatal brachial plexus palsy

    PubMed Central

    Andersen, John; Watt, Joe; Olson, Jaret; Van Aerde, John

    2006-01-01

    BACKGROUND Perinatal brachial plexus palsy (PBPP) is a flaccid paralysis of the arm at birth that affects different nerves of the brachial plexus supplied by C5 to T1 in 0.42 to 5.1 infants per 1000 live births. OBJECTIVES To identify antenatal factors associated with PBPP and possible preventive measures, and to review the natural history as compared with the outcome after primary or secondary surgical interventions. METHODS A literature search on randomized controlled trials, systematic reviews and meta-analyses on the prevention and treatment of PBPP was performed. EMBASE, Medline, CINAHL and the Cochrane Library were searched until June 2005. Key words for searches included ‘brachial plexus’, ‘brachial plexus neuropathy’, ‘brachial plexus injury’, ‘birth injury’ and ‘paralysis, obstetric’. RESULTS There were no prospective studies on the cause or prevention of PBPP. Whereas birth trauma is said to be the most common cause, there is some evidence that PBPP may occur before delivery. Shoulder dystocia and PBPP are largely unpredictable, although associations of PBPP with shoulder dystocia, infants who are large for gestational age, maternal diabetes and instrumental delivery have been reported. The various forms of PBPP, clinical findings and diagnostic measures are described. Recent evidence suggests that the natural history of PBPP is not all favourable, and residual deficits are estimated at 20% to 30%, in contrast with the previous optimistic view of full recovery in greater than 90% of affected children. There were no randomized controlled trials on nonoperative management. There was no conclusive evidence that primary surgical exploration of the brachial plexus supercedes conservative management for improved outcome. However, results from nonrandomized studies indicated that children with severe injuries do better with surgical repair. Secondary surgical reconstructions were inferior to primary intervention, but could still improve arm

  7. Bovine Spongiform Encephalopathy: Atypical Pros and Cons

    USDA-ARS?s Scientific Manuscript database

    Transmissible spongiform encephalopathies (TSEs) are fatal neurologic diseases that affect several mammalian species including human beings. Four animal TSE agents have been reported: scrapie of sheep and goats; chronic wasting disease (CWD) of deer, elk, and moose; transmissible mink encephalopath...

  8. Chronic traumatic encephalopathy: The unknown disease.

    PubMed

    Martínez-Pérez, R; Paredes, I; Munarriz, P M; Paredes, B; Alén, J F

    2017-04-01

    Chronic traumatic encephalopathy is a neurodegenerative disease produced by accumulated minor traumatic brain injuries; no definitive premortem diagnosis and no treatments are available for chronic traumatic encephalopathy. Risk factors associated with chronic traumatic encephalopathy include playing contact sports, presence of the apolipoprotein E4, and old age. Although it shares certain histopathological findings with Alzheimer disease, chronic traumatic encephalopathy has a more specific presentation (hyperphosphorylated tau protein deposited as neurofibrillary tangles, associated with neuropil threads and sometimes with beta-amyloid plaques). Its clinical presentation is insidious; patients show mild cognitive and emotional symptoms before progressing to parkinsonian motor signs and finally dementia. Results from new experimental diagnostic tools are promising, but these tools are not yet available. The mainstay of managing this disease is prevention and early detection of its first symptoms. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Hashimoto's encephalopathy: report of three cases.

    PubMed

    Chang, Jan-Shun; Chang, Tien-Chun

    2014-11-01

    Both severe thyrotoxicosis and hypothyroidism may affect brain function and cause a change in consciousness, as seen with a thyroid storm or myxedema coma. However, encephalopathy may also develop in patients with autoimmune thyroid diseases independent of actual thyroid function level, and this is known as Hashimoto's encephalopathy. Although most patients are found to have Hashimoto's thyroiditis, less frequently they have Graves' disease. Clinical manifestations include epilepsy, disturbance of consciousness, cognitive impairment, memory loss, myoclonus, hallucinations, stroke-like episodes, tremor, involuntary movements, language impairment, and gait impairment. Hashimoto's encephalopathy is a relatively rare disease. As a good response can be obtained with corticosteroid therapy, early diagnosis and treatment is very beneficial for patients. Here we report three patients with Hashimoto's encephalopathy with typical manifestations of hallucinations that were associated with hypothyroidism, hyperthyroidism, and euthyroid status, respectively. They all showed a dramatic response to methylprednisolone pulse therapy.

  10. Hashimoto's encephalopathy: a rare pediatric brain disease.

    PubMed

    Farrell, Ryan M; Foster, Michael B; Omoruyi, Adetokunbo O; Kingery, Suzanne E; Wintergerst, Kupper A

    2015-05-01

    We report a 9-year-old female who presented with new onset intractable seizure activity followed by a prolonged encephalopathic state. After ruling out common etiologies, Hashimoto's encephalopathy (HE) was considered, and antibody levels to thyroid peroxidase and thyroglobulin were both markedly elevated in her serum. She was euthyroid at the time of presentation. Upon treatment with high dose methylprednisolone, the patient demonstrated a significant improvement in her encephalopathy. The diagnosis of HE requires strong clinical suspicion with evidence of antithyroid antibodies, as well as an encephalopathy not explained by another etiology. While well documented in the adult literature, only a handful of pediatric cases have been described to date. Patients with HE have a nearly universal response to high dose glucocorticoids. HE should be considered in the differential diagnosis of any patient, adult or pediatric, who displays prolonged, unexplainable encephalopathy.

  11. [Cortexin effectiveness in circulatory encephalopathy].

    PubMed

    Khavinson, V Kh; Morozov, V G; Rybnikov, V Iu; Zakutskiĭ, N G

    1999-01-01

    A clinical trial of cortexin, a new peptide bioregulator of cerebral functions, in combined therapy of dyscirculatory encephalopathy (DE) stage I-II was made in 76 patients. They were divided into two groups: a control group of 31 patients on standard therapy and the study group of 45 patients on standard therapy with adjuvant cortexin delivered via nasal electrophoresis (NE). The effect was estimated by clinical symptoms, psychophysiological tests, computed EEG, quantitative parameters of rehabilitation. Cortexin NE produced a positive effect on psychoemotional state, neurological status, intellectual-mnestic and CNS functions. Adjuvant cortexin aroused efficiency of rehabilitation in DE stage I and II by 22.7%. The response of intellectual-mnestic and CNS functions was the highest. Cortexin improves attention, perception, memory, thinking, cortical neurodynamic processes. It is well tolerated and has no side effects. Cortexin is recommended as a drug of choice in combined treatment of patients with DE stage I-II.

  12. Suicide and Chronic Traumatic Encephalopathy.

    PubMed

    Iverson, Grant L

    2016-01-01

    For nearly 80 years, suicidality was not considered to be a core clinical feature of chronic traumatic encephalopathy (CTE). In recent years, suicide has been widely cited as being associated with CTE, and now depression has been proposed to be one of three core diagnostic features alongside cognitive impairment and anger control problems. This evolution of the clinical features has been reinforced by thousands of media stories reporting a connection between mental health problems in former athletes and military veterans, repetitive neurotrauma, and CTE. At present, the science underlying the causal assumption between repetitive neurotrauma, depression, suicide, and the neuropathology believed to be unique to CTE is inconclusive. Epidemiological evidence indicates that former National Football League players, for example, are at lower, not greater, risk for suicide than men in the general population. This article aims to discuss the critical issues and literature relating to these possible relationships.

  13. Metabolic encephalopathy in Egyptian children.

    PubMed

    Hindawy, A; Gouda, A; El-Ayyadi, A; Megahed, H; Bazaraa, H

    2007-01-01

    Fatty Acid Oxidation disorders represent an expanding group of inborn errors of metabolism. Clinical manifestations include episodic encephalopathy, hypoketotic hypoglycemia, Reye like episodes, hepatic, muscular, cardiac affection and sudden death. Analysis of urinary organic acids and plasma fatty acids of 44 clinically suspected patients by Gas Chromatography Mass spectrometry revealed 4 cases of Medium chain acyl-CoA dehydrogenase deficiency (MCADD), 3 cases of Very long chain acyl-CoA dehydrogenase deficiency, 9 cases of multiple defects of acyl-CoA dehydrogenation in addition to 3 patients with other metabolic disorders. Timely detection of these disorders including screening for MCADD can have a favorable impact on the outcome of these patients (Tab. 11, Fig. 3, Ref. 24) Full Text (Free, PDF).

  14. Hepatic Encephalopathy in Liver Cirrhosis.

    PubMed

    Djiambou-Nganjeu, Herbert

    2017-03-01

    Liver cirrhosis is a worldwide gastroenterological condition, characterized by a slow, progressive and irreversible replacement of liver cells by fibrous tissue (scar) that prevents liver function. This condition often leads to the development of other syndromes. Cardiac complications can be indicated through abnormal QTc interval and arrhythmias, thereby their analysis aids in the prevention of cardiovascular events. Most cirrhotic cases have abnormal laboratory values (bilirubin, albumin, AST, ALT, AST/ALT, INR) indicating the presence of concomitant infection, inflammation and coagulopathy. In this case report, the usage Halstead-Reitan and Child-Pugh score helped in the assessment of the status of deterioration of brain. The knowledge of liver cirrhosis aetiologies help to determine the predisposition to development of hepatic encephalopathy and cardiomyopathy. The different values of liver enzymes and other blood laboratory analyses indicated the level of liver damage and poor prognosis.

  15. Wernicke's Encephalopathy following Hyperemesis Gravidarum

    PubMed Central

    Kotha, V.K.; De Souza, A.

    2013-01-01

    Wernicke's encephalopathy (WE) due to causes other than chronic alcohol abuse is an uncommon and often misdiagnosed condition. In the setting of hyperemesis gravidarum, an acute deficiency of thiamine results from body stores being unable to meet increased metabolic demands. The condition produces typical clinical and radiological findings and when diagnosed early and treated promptly has a good prognosis. Magnetic resonance imaging (MRI) is sensitive and specific for diagnosis. We describe three patients with hyperemesis gravidarum who developed WE, and highlight a range of clinical and imaging features important for appropriate diagnosis. A high degree of clinical suspicion is essential. Treatment is often empirical pending results of investigation, and consists of parenteral repletion of thiamine stores. Reversal of MRI findings parallels clinical improvement. Neurologic outcomes are usually good, but half the pregnancies complicated by this condition do not produce healthy children. PMID:23859165

  16. Encephalopathy

    MedlinePlus

    ... progressive loss of memory and cognitive ability, subtle personality changes, inability to concentrate, lethargy, and progressive loss ... progressive loss of memory and cognitive ability, subtle personality changes, inability to concentrate, lethargy, and progressive loss ...

  17. Risk of Suicidal Ideation in Adolescents with Both Self-Asphyxial Risk-Taking Behavior and Non-Suicidal Self-Injury

    ERIC Educational Resources Information Center

    Brausch, Amy M.; Decker, Kristina M.; Hadley, Andrea G.

    2011-01-01

    This study examined adolescent participation in self-asphyxial risk-taking behaviors (SAB), sometimes known as the "choking game," and its relationship with other adolescent risk behaviors, including non-suicidal self-injury (NSSI). Researchers proposed that participation in SAB and NSSI would be associated with suicidal behavior, disordered…

  18. Cognitive Outcomes After Neonatal Encephalopathy

    PubMed Central

    Shankaran, Seetha; McDonald, Scott A.; Vohr, Betty R.; Hintz, Susan R.; Ehrenkranz, Richard A.; Tyson, Jon E.; Yolton, Kimberly; Das, Abhik; Bara, Rebecca; Hammond, Jane; Higgins, Rosemary D.

    2015-01-01

    OBJECTIVES: To describe the spectrum of cognitive outcomes of children with and without cerebral palsy (CP) after neonatal encephalopathy, evaluate the prognostic value of early developmental testing and report on school services and additional therapies. METHODS: The participants of this study are the school-aged survivors of the National Institute of Child Health and Human Development Neonatal Research Network randomized controlled trial of whole-body hypothermia. Children underwent neurologic examinations and neurodevelopmental and cognitive testing with the Bayley Scales of Infant Development–II at 18 to 22 months and the Wechsler intelligence scales and the Neuropsychological Assessment–Developmental Neuropsychological Assessment at 6 to 7 years. Parents were interviewed about functional status and receipt of school and support services. We explored predictors of cognitive outcome by using multiple regression models. RESULTS: Subnormal IQ scores were identified in more than a quarter of the children: 96% of survivors with CP had an IQ <70, 9% of children without CP had an IQ <70, and 31% had an IQ of 70 to 84. Children with a mental developmental index <70 at 18 months had, on average, an adjusted IQ at 6 to 7 years that was 42 points lower than that of those with a mental developmental index >84 (95% confidence interval, −49.3 to −35.0; P < .001). Twenty percent of children with normal IQ and 28% of those with IQ scores of 70 to 84 received special educational support services or were held back ≥1 grade level. CONCLUSIONS: Cognitive impairment remains an important concern for all children with neonatal encephalopathy. PMID:25713280

  19. Cognitive outcomes after neonatal encephalopathy.

    PubMed

    Pappas, Athina; Shankaran, Seetha; McDonald, Scott A; Vohr, Betty R; Hintz, Susan R; Ehrenkranz, Richard A; Tyson, Jon E; Yolton, Kimberly; Das, Abhik; Bara, Rebecca; Hammond, Jane; Higgins, Rosemary D

    2015-03-01

    To describe the spectrum of cognitive outcomes of children with and without cerebral palsy (CP) after neonatal encephalopathy, evaluate the prognostic value of early developmental testing and report on school services and additional therapies. The participants of this study are the school-aged survivors of the National Institute of Child Health and Human Development Neonatal Research Network randomized controlled trial of whole-body hypothermia. Children underwent neurologic examinations and neurodevelopmental and cognitive testing with the Bayley Scales of Infant Development-II at 18 to 22 months and the Wechsler intelligence scales and the Neuropsychological Assessment-Developmental Neuropsychological Assessment at 6 to 7 years. Parents were interviewed about functional status and receipt of school and support services. We explored predictors of cognitive outcome by using multiple regression models. Subnormal IQ scores were identified in more than a quarter of the children: 96% of survivors with CP had an IQ <70, 9% of children without CP had an IQ <70, and 31% had an IQ of 70 to 84. Children with a mental developmental index <70 at 18 months had, on average, an adjusted IQ at 6 to 7 years that was 42 points lower than that of those with a mental developmental index >84 (95% confidence interval, -49.3 to -35.0; P < .001). Twenty percent of children with normal IQ and 28% of those with IQ scores of 70 to 84 received special educational support services or were held back ≥1 grade level. Cognitive impairment remains an important concern for all children with neonatal encephalopathy. Copyright © 2015 by the American Academy of Pediatrics.

  20. Wernicke encephalopathy and ethanol-related syndromes.

    PubMed

    Kim, Tae Eun; Lee, Eun Ja; Young, Jeong Bo; Shin, Dong Jae; Kim, Ji Hoon

    2014-04-01

    Ethanol causes diverse neurologic conditions caused by acute and chronic brain damage. This review provides an overview of Wernicke encephalopathy and other ethanol-related brain changes, such as chronic brain atrophy, Marchiafava-Bignami disease, osmotic demyelination syndrome, chronic hepatic encephalopathy, and acute alcohol withdrawal. As clinical symptoms of this spectrum of diseases have nonspecific neurologic alterations, radiologists should have current radiologic information and understand the imaging findings pertaining to the pathophysiology to support diagnosis.

  1. Is major depressive disorder a metabolic encephalopathy?

    PubMed

    Harvey, Brian H

    2008-07-01

    Metabolic encephalopathy is an acute disturbance in cellular metabolism in the brain evoked by conditions of hypoxia, hypoglycaemia, oxidative stress and/or inflammation. It usually develops acutely or subacutely and is reversible if the systemic disorder is treated. If left untreated, however, metabolic encephalopathy may result in secondary structural damage to the brain. Most encephalopathies are present with neuropsychiatric symptoms, one in particular being depression. However, mood disorders are often co-morbid with cardiovascular, liver, kidney and endocrine disorders, while increasing evidence concurs that depression involves inflammatory and neurodegenerative processes. This would suggest that metabolic disturbances resembling encephalopathy may underscore the basic neuropathology of depression at a far deeper level than currently realized. Viewing depression as a form of encephalopathy, and exploiting knowledge gleaned from our understanding of the neurochemistry and treatment of metabolic encephalopathy, may assist in our understanding of the neurobiology of depression, but also in realizing new ideas in the pharmacotherapy of mood disorders. Copyright 2008 John Wiley & Sons, Ltd.

  2. Clinically mild infantile encephalopathy associated with excitotoxicity.

    PubMed

    Hirai, Nozomi; Yoshimaru, Daisuke; Moriyama, Yoko; Honda, Takafumi; Yasukawa, Kumi; Takanashi, Jun-Ichi

    2017-02-15

    Acute infectious encephalopathy is very frequently observed in children in East Asia including Japan. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most common subtype in Japan; however, more than 40% of the patients remain unclassified into specific syndromes. To investigate the underlying pathomechanism in those with unclassified acute encephalopathy, we evaluated brain metabolism by MR spectroscopy. Among 20 patients with acute encephalopathy admitted to our hospital during January 2015 to May 2016, 12 could not be classified into specific syndromes. MR spectroscopy was performed in 8 of these 12 patients with unclassified encephalopathy. MR spectroscopy showed an increase of glutamine with a normal N-acetyl aspartate level on days 3 to 8 in three of the 8 patients, which had normalized by follow-up studies. The three patients clinically recovered completely. This study suggests that excitotoxicity may be the underlying pathomechanism in some patients with unclassified mild encephalopathy. Copyright © 2016 Elsevier B.V. All rights reserved.

  3. Gemifloxacin-associated neurotoxicity presenting as encephalopathy.

    PubMed

    Barrett, Matthew J; Login, Ivan S

    2009-04-01

    To report a case of acute encephalopathy associated with ingestion of gemifloxacin, a fluoroquinolone. A 67-year-old woman presented to the emergency department with an acute alteration in mental status. Twenty-four hours earlier she had taken one 320-mg tablet of her husband's gemifloxacin prescription to treat symptoms of a mild upper respiratory infection. During her initial evaluation at our institution, the woman was dysphasic, unable to follow commands, and agitated, suggesting encephalopathy. A thorough diagnostic investigation did not reveal any structural, metabolic, or infectious etiology. Her mental status returned to normal within 2 days without any definitive treatment. Fluoroquinolone-associated neurotoxicity may manifest as encephalopathy, seizures, confusion, or toxic psychosis. To date, none of these adverse effects, specifically encephalopathy, has been reported with gemifloxacin. An objective causality assessment revealed that encephalopathy was probably associated with gemifloxacin use. Seizures, either convulsive or nonconvulsive, may have contributed to our patient's presentation, but she denied seizures prior to this event and did not suffer a seizure in the 18 months following her discharge. However, her second electroencephalograph revealed an underlying predisposition to seizures, which gemifloxacin may have unmasked. This report illustrates that severe central nervous system adverse effects associated with some fluoroquinolones may also occur with gemifloxacin. Gemifloxacin and other fluoroquinolones should be considered in the etiologic evaluation of patients with acute encephalopathy.

  4. End-Tidal CO2-Guided Chest Compression Delivery Improves Survival in a Neonatal Asphyxial Cardiac Arrest Model.

    PubMed

    Hamrick, Justin T; Hamrick, Jennifer L; Bhalala, Utpal; Armstrong, Jillian S; Lee, Jeong-Hoo; Kulikowicz, Ewa; Lee, Jennifer K; Kudchadkar, Sapna R; Koehler, Raymond C; Hunt, Elizabeth A; Shaffner, Donald H

    2017-08-16

    To determine whether end-tidal CO2-guided chest compression delivery improves survival over standard cardiopulmonary resuscitation after prolonged asphyxial arrest. Preclinical randomized controlled study. University animal research laboratory. 1-2-week-old swine. After undergoing a 20-minute asphyxial arrest, animals received either standard or end-tidal CO2-guided cardiopulmonary resuscitation. In the standard group, chest compression delivery was optimized by video and verbal feedback to maintain the rate, depth, and release within published guidelines. In the end-tidal CO2-guided group, chest compression rate and depth were adjusted to obtain a maximal end-tidal CO2 level without other feedback. Cardiopulmonary resuscitation included 10 minutes of basic life support followed by advanced life support for 10 minutes or until return of spontaneous circulation. Mean end-tidal CO2 at 10 minutes of cardiopulmonary resuscitation was 34 ± 8 torr in the end-tidal CO2 group (n = 14) and 19 ± 9 torr in the standard group (n = 14; p = 0.0001). The return of spontaneous circulation rate was 7 of 14 (50%) in the end-tidal CO2 group and 2 of 14 (14%) in the standard group (p = 0.04). The chest compression rate averaged 143 ± 10/min in the end-tidal CO2 group and 102 ± 2/min in the standard group (p < 0.0001). Neither asphyxia-related hypercarbia nor epinephrine administration confounded the use of end-tidal CO2-guided chest compression delivery. The response of the relaxation arterial pressure and cerebral perfusion pressure to the initial epinephrine administration was greater in the end-tidal CO2 group than in the standard group (p = 0.01 and p = 0.03, respectively). The prevalence of resuscitation-related injuries was similar between groups. End-tidal CO2-guided chest compression delivery is an effective resuscitation method that improves early survival after prolonged asphyxial arrest in this neonatal piglet model. Optimizing end-tidal CO2 levels during

  5. Epilepsy and West syndrome in neonates with hypoxic-ischemic encephalopathy.

    PubMed

    Inoue, Takeshi; Shimizu, Masaki; Hamano, Shin-Ichiro; Murakami, Nobuyuki; Nagai, Toshiro; Sakuta, Ryoichi

    2014-06-01

    Perinatal hypoxic-ischemic encephalopathy (HIE) has been linked to the development of late-onset seizures. The aim of the present study was to determine the incidence of epilepsy and West syndrome in children with perinatal HIE and identify factors associated with the development of postnatal seizure disorders. We retrospectively enrolled 208 term and late-preterm infants diagnosed with perinatal HIE from April 2000 to March 2009 at Saitama Children's Medical Center. Children with obvious multiple anomalies and known chromosomal abnormalities were excluded. A questionnaire was distributed to parents to determine seizure-related outcomes. Medical records were retrospectively reviewed and relevant clinical parameters were analyzed. In total, 162 questionnaires were answered (77.9%). Of the 162 subjects, 26 (16.0%) developed epilepsy, and eight subjects (4.9%) were diagnosed with West syndrome. Neonatal seizures occurred in 72 subjects (44.4%). The incidence of epilepsy and West syndrome was significantly higher in infants who experienced neonatal seizures than in those without seizure history. A total of 82 subjects were diagnosed with moderate (n = 52) or severe HIE (n = 30), of whom 57 subjects (69.5%) received therapeutic hypothermia. The incidence of epilepsy was significantly lower in these treated subjects. In addition, subjects with moderate or severe HIE were significantly more likely to develop late-onset epilepsy and West syndrome than those with mild HIE. The severity of perinatal HIE and neonatal seizures is a potential risk factor for the development of late-onset seizures. Therapeutic hypothermia may reduce the risk of the development of epilepsy in such cases. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

  6. Prenatal encephalopathies of unknown origin. Our 19-years experience. To what extent must genetic and biochemical studies be carried out?

    PubMed

    López Pisón, J; García Jiménez, M C; Lafuente Hidalgo, M; Pérez Delgado, R; Monge Galindo, L; Cabrerizo de Diago, R; Rebage Moisés, V; Peña Segura, J L; Baldellou Vázquez, A

    2011-10-01

    We examine those prenatal encephalopathies with clinical or neuroimaging data of encephalopathy before the birth. They affect a significant number of children seen by paediatric neurologists. They can be of disruptive origin (due to vascular problems, drugs, toxins or congenital infections), and genetically determined. We include cases of autism spectrum disorder and mental retardation with no history of perinatal of postnatal damages. We analysed our 19 year neuro-paediatric data base in search of prenatal encephalopathies and their diagnostic origin. We also analyse the studies made in the cases with a diagnosis of unknown origin. The 19 year period of study in the data base included 11,910 children, and 1596 (13.5%) were considered as prenatal encephalopathies; 1307 children (81.4%) had a diagnosis of unknown origin, despite many investigations being done in a large number of them. Most of the children included in this study suffer a rare disease, and whether they are identified or not, they increasingly require an early diagnosis. Peroxisomal, mitochondrial, lysosomal diseases, carbohydrate glycosylation deficiency syndrome and other inborn error of metabolism, congenital infections and genetic encephalopathies, can be clinically indistinguishable in early life and require specific studies to identify them. Early diagnosis requires strategies using step-wise systematic studies, giving priority to those diseases that could be treated, and in many cases using an individualised approach. We believe that the potential benefits of early diagnosis, including savings on further studies, genetic counselling and prenatal diagnosis, overcome the financial costs. Copyright © 2010 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  7. [Relationship between PMI and fourier transform infrared spectral changes in muscle of rats after death caused by mechanical asphyxial].

    PubMed

    Li, Shi-ying; Shao, Yu; Li, Zheng-dong; Liu, Ning-guo; Zou, Dong-hua; Qin, Zhi-qiang; Chen, Yi-jiu; Huang, Ping

    2012-06-01

    To observe the postmortem degradation process in rat myocardium and skeletal muscle using Fourier transform infrared (FTIR) spectroscopy and to provide a new method for estimating postmortem interval (PMI). Left ventricle and skeletal muscles of rats dying of mechanical asphyxiated were sampled at different PMIs. The changes of different chemical functional group in the myocardium and skeletal muscle samples were measured by FTIR spectroscopy. The different absorbance (A) ratios of peaks were calculated and the curve estimation analysis between absorbance ratios (x) and PMI (y) were performed to establish six mathematical models. FTIR spectral absorption peak of rat myocardium and skeletal muscle showed three changes: increase, decrease and stable. The cubic model function showed the strongest correlation coefficient. The A1080/A1396 ratio of skeletal muscle showed the strongest correlation coefficient (r = 0.832) with more accurate determination of PMI. FYIR spectroscopy can be potentially used as an effective method for estimating PMI in forensic practice using myocardium and skeletal muscle.

  8. Quantitative morphometry of granular 'dot-like' ubiquitin-immunoreactivity in the crus cerebri in asphyxiation and fire fatalities.

    PubMed

    Quan, Li; Ishikawa, Takaki; Michiue, Tomomi; Li, Dong-Ri; Zhao, Dong; Zhu, Bao-Li; Maeda, Hitoshi

    2005-03-01

    In the central nervous system (CNS), a variety of ubiquitinated structures have been reported, usually as pathological alterations of the brain related to degenerative diseases or aging. However, previous studies showed an increase in the ubiquitin (Ub)-immunoreactive intranuclear inclusion of the pigmented neurons of the substantia nigra in the midbrain in asphyxiation and fire fatalities in the adult subjects. The aim of the present study was to examine granular 'dot-like' Ub-immunoreactivity in the crus cerebri (cortico-spinal tracts) in related fatalities (over 35 years of age, n=169), including fatal asphyxiation (n=27), drownings (n=14), fire fatalities (n=60), and control groups (n=68). Dot-like Ub-immunoreactivity was clearly observed in the descending tract of the crus cerebri. Morphometric analysis of the positive granular area (dot-like Ub-area) showed a higher value in strangulation and fire fatalities and a lower value in hemorrhagic and head injury deaths, as was observed for the inclusion-type neuronal Ub-positivity. However, there was a difference between those markers: a low value was seen for the inclusion-type neuronal Ub-positivity in hanging and drownings, and a difference in the dot-like Ub-area was detected between fire fatalities with lower and higher COHb levels. Our findings suggested the possible usefulness of these markers for examination of CNS stress responses in traumas, at least in middle-aged and elderly victims and a partial difference in stress reaction between the cortico-spinal tracts and dopaminergic neurons.

  9. Cognitive impairments in Hashimoto's encephalopathy: a case-control study.

    PubMed

    Wang, Jianhong; Zhang, Jun; Xu, Lan; Shi, Yunbo; Wu, Xunyi; Guo, Qihao

    2013-01-01

    Hashimoto's encephalopathy is considered as a treatable dementia, but it is often misdiagnosed. We investigated cognitive impairment and the MRI pathology of Hashimoto's encephalopathy patients. The study comprised eight patients with Hashimoto's encephalopathy, 16 patients with mild Alzheimer's disease and 24 healthy subjects. A neuropsychological battery included assessments of memory, language, attention, executive function and visuospatial ability. Cranial MRI was obtained from all Hashimoto's encephalopathy patients. Hashimoto's encephalopathy and mild Alzheimer's disease showed cognitive impairments in episodic memory, attention, executive function and visuospatial ability, but naming ability was unaffected in Hashimoto's encephalopathy. The MRI of Hashimoto's encephalopathy showed leukoencephalopathy-like type or limbic encephalitis-like type; the lesions did not affect the temporal cortex which plays a role in naming ability. Except that the naming ability was retained, the impairments in cognitive functions for the Hashimoto's encephalopathy patients were similar to those of Alzheimer's disease patients. These results were consistent with the MRI findings.

  10. Holistic approach for automated background EEG assessment in asphyxiated full-term infants

    NASA Astrophysics Data System (ADS)

    Matic, Vladimir; Cherian, Perumpillichira J.; Koolen, Ninah; Naulaers, Gunnar; Swarte, Renate M.; Govaert, Paul; Van Huffel, Sabine; De Vos, Maarten

    2014-12-01

    Objective. To develop an automated algorithm to quantify background EEG abnormalities in full-term neonates with hypoxic ischemic encephalopathy. Approach. The algorithm classifies 1 h of continuous neonatal EEG (cEEG) into a mild, moderate or severe background abnormality grade. These classes are well established in the literature and a clinical neurophysiologist labeled 272 1 h cEEG epochs selected from 34 neonates. The algorithm is based on adaptive EEG segmentation and mapping of the segments into the so-called segments’ feature space. Three features are suggested and further processing is obtained using a discretized three-dimensional distribution of the segments’ features represented as a 3-way data tensor. Further classification has been achieved using recently developed tensor decomposition/classification methods that reduce the size of the model and extract a significant and discriminative set of features. Main results. Effective parameterization of cEEG data has been achieved resulting in high classification accuracy (89%) to grade background EEG abnormalities. Significance. For the first time, the algorithm for the background EEG assessment has been validated on an extensive dataset which contained major artifacts and epileptic seizures. The demonstrated high robustness, while processing real-case EEGs, suggests that the algorithm can be used as an assistive tool to monitor the severity of hypoxic insults in newborns.

  11. Social Support Following Perinatal Loss

    PubMed Central

    Kavanaugh, Karen; Trier, Darcie; Korzec, Michelle

    2005-01-01

    The purpose of this project was to examine parents' descriptions of the ways family and friends supported them after they had experienced a perinatal loss. For this project, a secondary analysis of data from two phenomenological studies on perinatal loss was performed. A combined total of 62 interview transcripts from 22 mothers and 9 fathers were examined. Data analysis included identifying all statements in the interview transcripts that pertained to the ways that family and friends supported parents. The modes of supportive behavior (emotional, advice/feedback, practical, financial, and socializing) in Vaux's theory of social support served as a useful framework for presenting the findings. Parents received emotional support most frequently. Findings from the current study provide data for health care professionals to use to provide guidance to family and friends of bereaved parents. PMID:17426820

  12. Estimating risks of perinatal death.

    PubMed

    Smith, Gordon C S

    2005-01-01

    The relative and absolute risks of perinatal death that are estimated from observational studies are used frequently in counseling about obstetric intervention. The statistical basis for these estimates therefore is crucial, but many studies are seriously flawed. In this review, a number of aspects of the approach to the estimation of the risk of perinatal death are addressed. Key factors in the analysis include (1) the definition of the cause of the death, (2) differentiation between antepartum and intrapartum events, (3) the use of the appropriate denominator for the given cause of death, (4) the assessment of the cumulative risk where appropriate, (5) the use of appropriate statistical tests, (6) the stratification of analysis of delivery-related deaths by gestational age, and (7) the specific features of multiple pregnancy, which include the correct determination of the timing of antepartum stillbirth and the use of paired statistical tests when outcomes are compared in relation to the birth order of twin pairs.

  13. Hashimoto's encephalopathy : epidemiology, pathogenesis and management.

    PubMed

    Mocellin, Ramon; Walterfang, Mark; Velakoulis, Dennis

    2007-01-01

    Hashimoto's encephalopathy is a term used to describe an encephalopathy of presumed autoimmune origin characterised by high titres of antithyroid peroxidase antibodies. In a similar fashion to autoimmune thyroid disease, Hashimoto's encephalopathy is more common in women than in men. It has been reported in paediatric, adult and elderly populations throughout the world. The clinical presentation may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms and myoclonus. Thyroid function is usually clinically and biochemically normal.Hashimoto's encephalopathy appears to be a rare disorder, but, as it is responsive to treatment with corticosteroids, it must be considered in cases of 'investigation negative encephalopathies'. Diagnosis is made in the first instance by excluding other toxic, metabolic and infectious causes of encephalopathy with neuroimaging and CSF examination. Neuroimaging findings are often not helpful in clarifying the diagnosis. Common differential diagnoses when these conditions are excluded are Creutzfeldt-Jakob disease, rapidly progressive dementias, and paraneoplastic and nonparaneoplastic limbic encephalitis. In the context of the typical clinical picture, high titres of antithyroid antibodies, in particular antithyroid peroxidase antibodies, are diagnostic. These antibodies, however, can be detected in elevated titres in the healthy general population. Treatment with corticosteroids is almost always successful, although relapse may occur if this treatment is ceased abruptly. Other forms of immunomodulation, such as intravenous immune-globulin and plasma exchange, may also be effective. Despite the link to autoimmune thyroid disease, the aetiology of Hashimoto's encephalopathy is unknown. It is likely that antithyroid antibodies are not pathogenic, but titres can be a marker of treatment response. Pathological findings can suggest an inflammatory process, but features

  14. Chronic traumatic encephalopathy: a review.

    PubMed

    Saulle, Michael; Greenwald, Brian D

    2012-01-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that is a long-term consequence of single or repetitive closed head injuries for which there is no treatment and no definitive pre-mortem diagnosis. It has been closely tied to athletes who participate in contact sports like boxing, American football, soccer, professional wrestling and hockey. Risk factors include head trauma, presence of ApoE3 or ApoE4 allele, military service, and old age. It is histologically identified by the presence of tau-immunoreactive NFTs and NTs with some cases having a TDP-43 proteinopathy or beta-amyloid plaques. It has an insidious clinical presentation that begins with cognitive and emotional disturbances and can progress to Parkinsonian symptoms. The exact mechanism for CTE has not been precisely defined however, research suggest it is due to an ongoing metabolic and immunologic cascade called immunoexcitiotoxicity. Prevention and education are currently the most compelling way to combat CTE and will be an emphasis of both physicians and athletes. Further research is needed to aid in pre-mortem diagnosis, therapies, and support for individuals and their families living with CTE.

  15. Chronic Traumatic Encephalopathy: A Review

    PubMed Central

    Saulle, Michael; Greenwald, Brian D.

    2012-01-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that is a long-term consequence of single or repetitive closed head injuries for which there is no treatment and no definitive pre-mortem diagnosis. It has been closely tied to athletes who participate in contact sports like boxing, American football, soccer, professional wrestling and hockey. Risk factors include head trauma, presence of ApoE3 or ApoE4 allele, military service, and old age. It is histologically identified by the presence of tau-immunoreactive NFTs and NTs with some cases having a TDP-43 proteinopathy or beta-amyloid plaques. It has an insidious clinical presentation that begins with cognitive and emotional disturbances and can progress to Parkinsonian symptoms. The exact mechanism for CTE has not been precisely defined however, research suggest it is due to an ongoing metabolic and immunologic cascade called immunoexcitiotoxicity. Prevention and education are currently the most compelling way to combat CTE and will be an emphasis of both physicians and athletes. Further research is needed to aid in pre-mortem diagnosis, therapies, and support for individuals and their families living with CTE. PMID:22567320

  16. Hepatic encephalopathy therapy: An overview.

    PubMed

    Riggio, Oliviero; Ridola, Lorenzo; Pasquale, Chiara

    2010-04-06

    Type-C hepatic encephalopathy (HE) is a severe complication of cirrhosis, which seriously affects quality of life and is strongly related to patient survival. Treatment based on a classical pharmacological approach that is aimed at reducing the production of gut-derived toxins, such as ammonia, is still under debate. Currently, results obtained from clinical trials do not support any specific treatment for HE and our competence in testing old and new treatment modalities by randomized controlled trials with appropriate clinically relevant end-points urgently needs to be improved. On the other hand, patients who are at risk for HE are now identifiable, based on studies on the natural history of the disease. Today, very few studies that are specifically aimed at establishing whether HE may be prevented are available or in progress. Recent studies have looked at non absorbable disaccharides or antibiotics and other treatment modalities, such as the modulation of intestinal flora. In the treatment of severe stage HE, artificial liver supports have been tested with initial positive results but more studies are needed.

  17. Chronic traumatic encephalopathy and athletes

    PubMed Central

    Mannix, Rebekah; Zafonte, Ross; Pascual-Leone, Alvaro

    2015-01-01

    Recent case reports have described athletes previously exposed to repetitive head trauma while participating in contact sports who later in life developed mood disorders, headaches, cognitive difficulties, suicidal ideation, difficulties with speech, and aggressive behavior. Postmortem discoveries show that some of these athletes have pathologic findings that are collectively termed chronic traumatic encephalopathy (CTE). Current hypotheses suggest that concussions or perhaps blows to the head that do not cause the signs and symptoms necessary for making the diagnosis of concussion, so-called subconcussive blows, cause both the clinical and pathologic findings. There are, however, some athletes who participate in contact sports who do not develop the findings ascribed to CTE. Furthermore, there are people who have headaches, mood disorders, cognitive difficulties, suicidal ideation, and other clinical problems who have neither been exposed to repeated head trauma nor possessed the pathologic postmortem findings of those currently diagnosed with CTE. The current lack of prospective data and properly designed case-control studies limits the current understanding of CTE, leading to debate about the causes of the neuropathologic findings and the clinical observations. Given the potential for referral and recall bias in available studies, it remains unclear whether or not the pathologic findings made postmortem cause the presumed neurobehavioral sequela and whether the presumed risk factors, such as sports activity, cerebral concussions, and subconcussive blows, are solely causative of the clinical signs and symptoms. This article discusses the current evidence and the associated limitations. PMID:26253448

  18. Chronic traumatic encephalopathy and athletes.

    PubMed

    Meehan, William; Mannix, Rebekah; Zafonte, Ross; Pascual-Leone, Alvaro

    2015-10-27

    Recent case reports have described athletes previously exposed to repetitive head trauma while participating in contact sports who later in life developed mood disorders, headaches, cognitive difficulties, suicidal ideation, difficulties with speech, and aggressive behavior. Postmortem discoveries show that some of these athletes have pathologic findings that are collectively termed chronic traumatic encephalopathy (CTE). Current hypotheses suggest that concussions or perhaps blows to the head that do not cause the signs and symptoms necessary for making the diagnosis of concussion, so-called subconcussive blows, cause both the clinical and pathologic findings. There are, however, some athletes who participate in contact sports who do not develop the findings ascribed to CTE. Furthermore, there are people who have headaches, mood disorders, cognitive difficulties, suicidal ideation, and other clinical problems who have neither been exposed to repeated head trauma nor possessed the pathologic postmortem findings of those currently diagnosed with CTE. The current lack of prospective data and properly designed case-control studies limits the current understanding of CTE, leading to debate about the causes of the neuropathologic findings and the clinical observations. Given the potential for referral and recall bias in available studies, it remains unclear whether or not the pathologic findings made postmortem cause the presumed neurobehavioral sequela and whether the presumed risk factors, such as sports activity, cerebral concussions, and subconcussive blows, are solely causative of the clinical signs and symptoms. This article discusses the current evidence and the associated limitations.

  19. Encephalopathy caused by lanthanum carbonate.

    PubMed

    Fraile, Pilar; Cacharro, Luis Maria; Garcia-Cosmes, Pedro; Rosado, Consolacion; Tabernero, Jose Matias

    2011-06-01

    Lanthanum carbonate is a nonaluminum, noncalcium phosphate-binding agent, which is widely used in patients with end-stage chronic kidney disease. Until now, no significant side-effects have been described for the clinical use of lanthanum carbonate, and there are no available clinical data regarding its tissue stores. Here we report the case of a 59-year-old patient who was admitted with confusional syndrome. The patient received 3750 mg of lanthanum carbonate daily. Examinations were carried out, and the etiology of the encephalopathy of the patient could not be singled out. The lanthanum carbonate levels in serum and cerebrospinal fluid were high, and the syndrome eased after the drug was removed. The results of our study confirm that, in our case, the lanthanum carbonate did cross the blood-brain barrier (BBB). Although lanthanum carbonate seems a safe drug with minimal absorption, this work reveals the problem derived from the increase of serum levels of lanthanum carbonate, and the possibility that it may cross the BBB. Further research is required on the possible pathologies that increase serum levels of lanthanum carbonate, as well as the risks and side-effects derived from its absorption.

  20. GABAergic transmission in hepatic encephalopathy.

    PubMed

    Sergeeva, Olga A

    2013-08-15

    Hepatic encephalopathy (HE)(1) is a neuropsychiatric disorder caused by chronic or acute liver failure. Nearly thirty years ago a hypothesis was formulated explaining the neuropathology of HE by increased GABAergic tone. Recent progress in the GABAA-receptor (GABAAR) molecular pharmacology and biochemistry as well as the physiology of GABAergic transmission provided better understanding of GABA's role in health and disease. A detailed analysis of neuronal populations and their GABAergic afferents affected in HE is still missing. The slow progress in understanding the pathology of GABAergic transmission in HE is due to the high complexity of brain circuitries controlled by multiple types of GABAergic interneurons and the large variety of GABAAR, which are differently affected by pathological conditions and not yet fully identified. The mechanisms of action of the GABAAR agonist taurine, allosteric positive modulators (inhibitory neurosteroids, anaesthetics, benzodiazepines and histamine) and inhibitors of the GABAAR (excitatory neurosteroids, Ro15-4513) are discussed with respect to HE pathophysiology. Perspectives for GABAergic drugs in the symptomatic treatment of HE are suggested.

  1. Opsoclonus as a manifestation of Hashimoto's encephalopathy.

    PubMed

    Salazar, R; Mehta, C; Zaher, N; Miller, D

    2012-10-01

    We present a 59-year-old male with early manifestation of opsoclonus associated with gait ataxia as a rare clinical presentation of Hashimoto's encephalopathy. Empiric use of intravenous immunoglobulin followed by intravenous high dose methylprednisolone was initiated with subsequent remittance of opsoclonus, encephalopathy, ataxia, and tremor. Extensive workup for infectious, autoimmune, and paraneoplastic etiologies were undertaken and all studies were negative. Thyroglobulin antibodies (312 U/mL) and thyroid peroxidase antibodies (457 U/mL) were elevated (normal <60 U/mL) with a euthyroid state (thyroid stimulating hormone 3.13 μIU/mL). Three months after intravenous steroid therapy, the concentrations of thyroglobulin and thyroid peroxidase antibodies were retested and found to have decreased considerably. Thus, with steroid therapy, the patient's opsoclonus and encephalopathy improved. We have presented a patient with a rare case of opsoclonus as the principal presenting feature of Hashimoto's encephalopathy that was incompletely responsive to intravenous immunoglobulin and resolved with corticosteroids. This report underscores the importance for clinical practitioners to maintain a high index of suspicion for Hashimoto's encephalopathy in cases of opsoclonus, especially when accompanied by an atypical presentation.

  2. Electroencephalography and Brain MRI Patterns in Encephalopathy.

    PubMed

    Wabulya, Angela; Lesser, Ronald P; Llinas, Rafael; Kaplan, Peter W

    2016-04-01

    Using electroencephalography (EEG) and histology in patients with diffuse encephalopathy, Gloor et al reported that paroxysmal synchronous discharges (PSDs) on EEG required combined cortical gray (CG) and "subcortical" gray (SCG) matter pathology, while polymorphic delta activity (PDA) occurred in patients with white matter pathology. In patients with encephalopathy, we compared EEG findings and magnetic resonance imaging (MRI) to determine if MRI reflected similar pathological EEG correlations. Retrospective case control study of 52 cases with EEG evidence of encephalopathy and 50 controls without evidence of encephalopathy. Review of clinical, EEG and MRI data acquired within 4 days of each other. The most common EEG finding in encephalopathy was background slowing, in 96.1%. We found PSDs in 0% of cases with the combination of CG and SCG abnormalities. Although 13.5% (n=7) had PSDs on EEG; 3 of these had CG and 4 had SCG abnormalities. A total of 73.1% (38/52) had white matter abnormalities-of these 28.9% (11/38) had PDA. PSDs were found with either CG or "SCG" MRI abnormalities and did not require a combination of the two. In agreement with Gloor et al, PDA occurred with white matter MRI abnormalities in the absence of gray matter abnormalities. © EEG and Clinical Neuroscience Society (ECNS) 2015.

  3. [Evidence-based management of perinatal depression].

    PubMed

    Chang, Mei-Yueh; Chen, Chung-Hey

    2008-04-01

    Perinatal depression, which may occur from pregnancy to one year after childbirth, is recognized by the World Health Organization as a significant health issue affecting women. Depression during the perinatal period can have enormous consequences, not only affecting the health of the woman herself but also influencing her interaction with her children and other family members. This article introduces several depression screening tools and evidence-based nonpharmacological managements of perinatal depression. There are some fairly valid and feasible screening methods, among which routinely screening perinatal women with EPDS (Edinburgh Perinatal Depression Scale) or BDI (Beck Depression Inventory) in the primary care setting is practicable. A survey of the limited literature available reveals that interpersonal psychotherapy, cognitive behavior therapy and listening to music provide quantifiable depression amelioration effects for perinatal women. More scientific research moderated by women's life experiences and preferences should be conducted, however, and applied to improve women's health.

  4. Discordance of Cognitive and Academic Achievement Outcomes in Youth with Perinatal HIV Exposure

    PubMed Central

    Garvie, Patricia A.; Zeldow, Bret; Malee, Kathleen; Nichols, Sharon L.; Smith, Renee A.; Wilkins, Megan L.; Williams, Paige L.

    2014-01-01

    Background To evaluate achievement in youth with perinatally acquired HIV (PHIV) compared to HIV-exposed uninfected peers (HEU), and to examine differential effects of HIV on cognition-achievement concordance. Methods Cognition and achievement were assessed using standardized measures. IQ-derived predicted achievement scores were subtracted from observed achievement scores to calculate discrepancy values. Linear regression models were used to compare achievement discrepancies between PHIV and HEU, adjusting for demographic covariates. Results Participants: 295 PHIV and 167 HEU youth; 71% black, 48% male, mean age 13.1 and 11.3 years, respectively. PHIV youth were relatively healthy (mean CD4%, 32%; viral load ≤400 copies/mL, 72%). PHIV and HEU youth had cognitive and achievement scores significantly below population norm means (p<0.001), but did not differ in cognition (mean FSIQ=86.7 vs. 89.4, respectively). In unadjusted models, HEU outperformed PHIV youth on Total Achievement (TA; mean=89.2 vs. 86.0, p=0.04) and Numerical Operations (NO; mean=88.8 vs. 82.9, p<0.001); no differences remained after adjustment. Mean observed-predicted achievement discrepancies reflected “underachievement”. History of encephalopathy predicted poorer achievement (p=0.039) and greater underachievement, even after adjustment. PHIV showed greater underachievement than HEU for NO (p<0.001) and TA (p=0.03), but these differences did not persist in adjusted models. Conclusions Both PHIV and HEU youth demonstrated lower achievement than normative samples, and underachieved relative to predicted achievement scores. Observed-predicted achievement discrepancies were associated with prior encephalopathy, older age and other non-HIV factors. PHIV youth with prior encephalopathy had significantly lower achievement and greater underachievement compared to PHIV without encephalopathy and HEU youth, even in adjusted models. PMID:25361033

  5. Discordance of cognitive and academic achievement outcomes in youth with perinatal HIV exposure.

    PubMed

    Garvie, Patricia A; Zeldow, Bret; Malee, Kathleen; Nichols, Sharon L; Smith, Renee A; Wilkins, Megan L; Williams, Paige L

    2014-09-01

    To evaluate achievement in youth with perinatally acquired HIV (PHIV) compared with HIV-exposed uninfected peers (HEU) and to examine differential effects of HIV on cognition-achievement concordance. Cognition and achievement were assessed using standardized measures. Intelligence quotient-derived predicted achievement scores were subtracted from observed achievement scores to calculate discrepancy values. Linear regression models were used to compare achievement discrepancies between PHIV and HEU, adjusting for demographic covariates. 295 PHIV and 167 HEU youth; 71% black, 48% male, mean age 13.1 and 11.3 years, respectively. PHIV youth were relatively healthy (mean CD4%, 32%; viral load ≤400 copies/mL, 72%). PHIV and HEU youth had cognitive and achievement scores significantly below population norm means (P < 0.001), but did not differ in cognition (mean full scale IQ = 86.7 vs. 89.4, respectively). In unadjusted models, HEU outperformed PHIV youth on total achievement (mean = 89.2 vs. 86.0, P = 0.04) and numerical operations (mean = 88.8 vs. 82.9, P < 0.001); no differences remained after adjustment. Mean observed-predicted achievement discrepancies reflected "underachievement". History of encephalopathy predicted poorer achievement (P = 0.039) and greater underachievement, even after adjustment. PHIV showed greater underachievement than HEU for numerical operations (P < 0.001) and total achievement (P = 0.03), but these differences did not persist in adjusted models. Both PHIV and HEU youth demonstrated lower achievement than normative samples and underachieved relative to predicted achievement scores. Observed-predicted achievement discrepancies were associated with prior encephalopathy, older age and other non-HIV factors. PHIV youth with prior encephalopathy had significantly lower achievement and greater underachievement compared with PHIV without encephalopathy and HEU youth, even in adjusted models.

  6. Near-infrared spectroscopy versus magnetic resonance imaging to study brain perfusion in newborns with hypoxic-ischemic encephalopathy treated with hypothermia.

    PubMed

    Wintermark, P; Hansen, A; Warfield, S K; Dukhovny, D; Soul, J S

    2014-01-15

    The measurement of brain perfusion may provide valuable information for assessment and treatment of newborns with hypoxic-ischemic encephalopathy (HIE). While arterial spin labeled perfusion (ASL) magnetic resonance imaging (MRI) provides noninvasive and direct measurements of regional cerebral blood flow (CBF) values, it is logistically challenging to obtain. Near-infrared spectroscopy (NIRS) might be an alternative, as it permits noninvasive and continuous monitoring of cerebral hemodynamics and oxygenation at the bedside. The purpose of this study is to determine the correlation between measurements of brain perfusion by NIRS and by MRI in term newborns with HIE treated with hypothermia. In this prospective cohort study, ASL-MRI and NIRS performed during hypothermia were used to assess brain perfusion in these newborns. Regional cerebral blood flow (CBF) values, measured from 1-2 MRI scans for each patient, were compared to mixed venous saturation values (SctO2) recorded by NIRS just before and after each MRI. Analysis included groupings into moderate versus severe HIE based on their initial background pattern of amplitude-integrated electroencephalogram. Twelve concomitant recordings were obtained of seven neonates. Strong correlation was found between SctO2 and CBF in asphyxiated newborns with severe HIE (r=0.88; p value=0.0085). Moreover, newborns with severe HIE had lower CBF (likely lower oxygen supply) and extracted less oxygen (likely lower oxygen demand or utilization) when comparing SctO2 and CBF to those with moderate HIE. NIRS is an effective bedside tool to monitor and understand brain perfusion changes in term asphyxiated newborns, which in conjunction with precise measurements of CBF obtained by MRI at particular times, may help tailor neuroprotective strategies in term newborns with HIE. Copyright © 2013 Elsevier Inc. All rights reserved.

  7. Near-Infrared Spectroscopy versus Magnetic Resonance Imaging To Study Brain Perfusion in Newborns with Hypoxic-Ischemic Encephalopathy Treated with Hypothermia

    PubMed Central

    Wintermark, P.; Hansen, A.; Warfield, SK.; Dukhovny, D.; Soul, JS.

    2014-01-01

    Background The measurement of brain perfusion may provide valuable information for assessment and treatment of newborns with hypoxic-ischemic encephalopathy (HIE). While arterial spin labeled perfusion (ASL) magnetic resonance imaging (MRI) provides noninvasive and direct measurements of regional cerebral blood flow (CBF) values, it is logistically challenging to obtain. Near-infrared spectroscopy (NIRS) might be an alternative, as it permits noninvasive and continuous monitoring of cerebral hemodynamics and oxygenation at the bedside. Objective The purpose of this study is to determine the correlation between measurements of brain perfusion by NIRS and by MRI in term newborns with HIE treated with hypothermia. Design/Methods In this prospective cohort study, ASL-MRI and NIRS performed during hypothermia were used to assess brain perfusion in these newborns. Regional cerebral blood flow values (CBF), measured from 1–2 MRI scans for each patient, were compared to mixed venous saturation values (SctO2) recorded by NIRS just before and after each MRI. Analysis included groupings into moderate versus severe HIE based on their initial background pattern of amplitude-integrated electroencephalogram. Results Twelve concomitant recordings were obtained of seven neonates. Strong correlation was found between SctO2 and CBF in asphyxiated newborns with severe HIE (r = 0.88; p value = 0.0085). Moreover, newborns with severe HIE had lower CBF (likely lower oxygen supply) and extracted less oxygen (likely lower oxygen demand or utilization) when comparing SctO2 and CBF to those with moderate HIE. Conclusions NIRS is an effective bedside tool to monitor and understand brain perfusion changes in term asphyxiated newborns, which in conjunction with precise measurements of CBF obtained by MRI at particular times, may help tailor neuroprotective strategies in term newborns with HIE. PMID:23631990

  8. [Prevention and treatment of energy failure in neonates with hypoxic-ischemic encephalopathy].

    PubMed

    Zou, Rong; Mu, De-Zhi

    2016-09-01

    Hypoxic-ischemic encephalopathy (HIE) in neonates is the brain injury caused by perinatal asphyxia or hypoxia and is a major cause of death in neonates and nervous system dysfunction in infants and young children. Although to a certain degree, mild hypothermia therapy reduces the mortality of infants with moderate to severe HIE, it cannot achieve the expected improvements in nervous system dysfunction. Hence, it is of vital importance to search for effective therapeutic methods for HIE. The search for more therapies and better preventive measures based on the pathogenesis of HIE has resulted in much research. As an important link in the course of HIE, energy failure greatly affects the development and progression of HIE. This article reviews the research advances in the treatment and prevention of energy failure in the course of HIE.

  9. Outcome After Therapeutic Hypothermia in Term Neonates with Encephalopathy and a Syndromic Diagnosis

    PubMed Central

    Mrelashvili, Anna; Bonifacio, Sonia L.; Rogers, Elizabeth E.; Shimotake, Thomas K.; Glass, Hannah C.

    2015-01-01

    The large randomized, controlled trials of therapeutic hypothermia for hypoxic-ischemic encephalopathy (HIE) excluded neonates with congenital disorders. The objective of this study was to report our experience using hypothermia in neonates with signs of HIE and a syndromic disorder or brain anomaly. Subjects were identified from a database of neonates admitted to the Neuro-Intensive Care Nursery at University of California, San Francisco. Of 169 patients fulfilling criteria for hypothermia, eight (5%) had a syndromic disorder, and were cooled as per guidelines for non-syndromic neonates. Perinatal characteristics of infants with and without syndromic disorder were not significantly different. Overall outcome was poor: 38% had evidence of acute HI injury, 3 subjects died, two survivors had low developmental quotient (DQ 25). The risk versus benefit of therapeutic hypothermia for HIE among neonates with congenital brain malformations or syndromic diagnoses is uncertain. PMID:25762585

  10. Perinatal injury of the central nervous system in Lithuania from 1997 to 2014

    PubMed Central

    Vytautas, Basys; Nijolė, Drazdienė; Nijolė, Vezbergienė; Jelena, Isakova

    2016-01-01

    Background. Perinatal CNS injuries are significant for the health of neonates and for child development at a later period. The aim of this study was to evaluate the dynamics of the frequency of perinatal CNS lesions (corresponding to ICD 10 code P91) over a 20-year period, using the data collected from the Lithuanian Medical Data of Births (Registry of Births). Material and methods. In total, data of 559,164 newborns were analyzed. Results. During the period from 1997 to 2014, the frequency of term newborns with perinatal CNS injury decreased almost two times, from 20.4/1000 live births in 1997 to 15.5/1000 live births in 2014, or from 3.12% (95% CI 2.95; 3.31) to 1.46% (95% CI 1.32; 1.61). In 18 years, the rate of infant mortality from perinatal CNS injury decreased by more than four times and in 2014 it was 0.3/1000 births; it accounts for 11% of neonatal mortality (2.6/1000 live births). The largest decrease of CNS injury was seen after a caesarean birth (from 13.7% in 1999 to 1.7% in 2014) and breech delivery (from 9.7% in 1999 to 0.8% in 2014). Analysis of the dynamics of perinatal CNS injury in preterm births in selected groups did not identify a significant positive shift during the period. When evaluating the level of childbirth services in different-level maternity hospitals, CNS injury is undoubtedly diminished in 2B-level maternity hospitals (regional). Also, positive dynamics was observed in the data of 2A-level maternity hospitals, while in 3-level maternity hospitals (university hospitals), which deal with the most complicated obstetrical pathology and preterm newborns, positive dynamics was not observed. It is estimated that the frequency of hypoxic-ischemic encephalopathy was 0.63/1000 live births in Lithuania in 1993. Conclusions. The frequency of perinatal CNS injury and its positive dynamics in over 18 years shows a progressive and scientifically-based perinatal health care organization in Lithuania.

  11. Clinical review of genetic epileptic encephalopathies

    PubMed Central

    Noh, Grace J.; Asher, Y. Jane Tavyev; Graham, John M.

    2012-01-01

    Seizures are a frequently encountered finding in patients seen for clinical genetics evaluations. The differential diagnosis for the cause of seizures is quite diverse and complex, and more than half of all epilepsies have been attributed to a genetic cause. Given the complexity of such evaluations, we highlight the more common causes of genetic epileptic encephalopathies and emphasize the usefulness of recent technological advances. The purpose of this review is to serve as a practical guide for clinical geneticists in the evaluation and counseling of patients with genetic epileptic encephalopathies. Common syndromes will be discussed, in addition to specific seizure phenotypes, many of which are refractory to anti-epileptic agents. Divided by etiology, we overview the more common causes of infantile epileptic encephalopathies, channelopathies, syndromic, metabolic, and chromosomal entities. For each condition, we will outline the diagnostic evaluation and discuss effective treatment strategies that should be considered. PMID:22342633

  12. Sepsis-associated encephalopathy: not just delirium

    PubMed Central

    Zampieri, Fernando Godinho; Park, Marcelo; Machado, Fabio Santana; Azevedo, Luciano Cesar Pontes

    2011-01-01

    Sepsis is a major cause of mortality and morbidity in intensive care units. Organ dysfunction is triggered by inflammatory insults and tissue hypoperfusion. The brain plays a pivotal role in sepsis, acting as both a mediator of the immune response and a target for the pathologic process. The measurement of brain dysfunction is difficult because there are no specific biomarkers of neuronal injury, and bedside evaluation of cognitive performance is difficult in an intensive care unit. Although sepsis-associated encephalopathy was described decades ago, it has only recently been subjected to scientific scrutiny and is not yet completely understood. The pathophysiology of sepsis-associated encephalopathy involves direct cellular damage to the brain, mitochondrial and endothelial dysfunction and disturbances in neurotransmission. This review describes the most recent findings in the pathophysiology, diagnosis, and management of sepsis-associated encephalopathy and focuses on its many presentations. PMID:22012058

  13. Surgical Treatment of Pediatric Epileptic Encephalopathies

    PubMed Central

    Fridley, J.; Reddy, G.; Curry, D.; Agadi, S.

    2013-01-01

    Pediatric epileptiform encephalopathies are a group of neurologically devastating disorders related to uncontrolled ictal and interictal epileptic activity, with a poor prognosis. Despite the number of pharmacological options for treatment of epilepsy, many of these patients are drug resistant. For these patients with uncontrolled epilepsy, motor and/or neuropsychological deterioration is common. To prevent these secondary consequences, surgery is often considered as either a curative or a palliative option. Magnetic resonance imaging to look for epileptic lesions that may be surgically treated is an essential part of the workup for these patients. Many surgical procedures for the treatment of epileptiform encephalopathies have been reported in the literature. In this paper the evidence for these procedures for the treatment of pediatric epileptiform encephalopathies is reviewed. PMID:24288601

  14. Wernicke's Encephalopathy Complicating Hyperemesis during Pregnancy

    PubMed Central

    Berdai, Mohamed Adnane; Labib, Smael; Harandou, Mustapha

    2016-01-01

    Wernicke's encephalopathy is caused by severe thiamine deficiency; it is mostly observed in alcoholic patients. We report the case of a 28-year-old woman, at 17 weeks of gestational age, with severe hyperemesis gravidarum. She presented with disturbance of consciousness, nystagmus, ophthalmoplegia, and ataxia. The resonance magnetic imagery showed bilaterally symmetrical hyperintensities of thalamus and periaqueductal area. The case was managed with very large doses of thiamine. The diagnosis of Wernicke's encephalopathy was confirmed later by a low thiamine serum level. The patient was discharged home on day 46 with mild ataxia and persistent nystagmus. Wernicke's encephalopathy is a rare complication of hyperemesis gravidarum. It should be diagnosed as early as possible to prevent long-term neurological sequela or death. Thiamine supplementation in pregnant women with prolonged vomiting should be initiated, especially before parenteral dextrose infusion. Early thiamine replacement will reduce maternal morbidity and fetal loss rate. PMID:26989522

  15. Experimental Interspecies Transmission Studies of the Transmissible Spongiform Encephalopathies to Cattle: Comparison to Bovine Spongiform Encephalopathy in Cattle

    USDA-ARS?s Scientific Manuscript database

    Prion diseases or transmissible spongiform encephalopathies (TSEs) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (TME); chronic wasting disease (CWD) of deer, elk and moose; and bovine spongiform encephalopathy (BSE) of cattle. Since the emergence of BSE and its pr...

  16. Concussion in Chronic Traumatic Encephalopathy

    PubMed Central

    Stein, Thor D.; Alvarez, Victor E.; McKee, Ann C.

    2015-01-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that occurs in association with repetitive mild traumatic brain injury. It is associated with a variety of clinical symptoms in multiple domains, and there is a distinct pattern of pathological changes. The abnormal tau pathology in CTE occurs uniquely in those regions of the brain that are likely most susceptible to stress concentration during trauma. CTE has been associated with a variety of types of repetitive head trauma, most frequently contact sports. In cases published to date, the mean length of exposure to repetitive head trauma was 15.4 years. The clinical symptoms of the disease began after a mean latency of 14.5 years with a mean age of death of 59.3 years. Most subjects had a reported history of concussions with a mean of 20.3. However, 16 % of published CTE subjects did not have a history of concussion suggesting that subconcussive hits are sufficient to lead to the development of CTE. Overall, the number of years of exposure, not the number of concussions, was significantly associated with worse tau pathology in CTE. This suggests that it is the chronic and repetitive nature of head trauma, irrespective of concussive symptoms, that is the most important driver of disease. CTE and exposure to repetitive head trauma is also associated with a variety of other neurodegenerations, including Alzheimer disease. In fact, amyloid β peptide deposition is altered and accelerated in CTE and is associated with worse disease. Here, we review the current exposure, clinical, and pathological associations of CTE. PMID:26260277

  17. Concussion in Chronic Traumatic Encephalopathy.

    PubMed

    Stein, Thor D; Alvarez, Victor E; McKee, Ann C

    2015-10-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disease that occurs in association with repetitive mild traumatic brain injury. It is associated with a variety of clinical symptoms in multiple domains, and there is a distinct pattern of pathological changes. The abnormal tau pathology in CTE occurs uniquely in those regions of the brain that are likely most susceptible to stress concentration during trauma. CTE has been associated with a variety of types of repetitive head trauma, most frequently contact sports. In cases published to date, the mean length of exposure to repetitive head trauma was 15.4 years. The clinical symptoms of the disease began after a mean latency of 14.5 years with a mean age of death of 59.3 years. Most subjects had a reported history of concussions with a mean of 20.3. However, 16 % of published CTE subjects did not have a history of concussion suggesting that subconcussive hits are sufficient to lead to the development of CTE. Overall, the number of years of exposure, not the number of concussions, was significantly associated with worse tau pathology in CTE. This suggests that it is the chronic and repetitive nature of head trauma, irrespective of concussive symptoms, that is the most important driver of disease. CTE and exposure to repetitive head trauma is also associated with a variety of other neurodegenerations, including Alzheimer disease. In fact, amyloid β peptide deposition is altered and accelerated in CTE and is associated with worse disease. Here, we review the current exposure, clinical, and pathological associations of CTE.

  18. Brain susceptibility to oxidative stress in the perinatal period.

    PubMed

    Perrone, Serafina; Tataranno, Luisa M; Stazzoni, Gemma; Ramenghi, Luca; Buonocore, Giuseppe

    2015-11-01

    Oxidative stress (OS) occurs at birth in all newborns as a consequence of the hyperoxic challenge due to the transition from the hypoxic intrauterine environment to extrauterine life. Free radical (FRs) sources such as inflammation, hyperoxia, hypoxia, ischaemia-reperfusion, neutrophil and macrophage activation, glutamate and free iron release, all increases the OS during the perinatal period. Newborns, and particularly preterm infants, have reduced antioxidant defences and are not able to counteract the harmful effects of FRs. Energy metabolism is central to life because cells cannot exist without an adequate supply of ATP. Due to its growth, the mammalian brain can be considered as a steady-state system in which ATP production matches ATP utilisation. The developing brain is particularly sensitive to any disturbances in energy generation, and even a short-term interruption can lead to long-lasting and irreversible damage. Whenever energy failure develops, brain damage can occur. Accumulating evidence indicates that OS is implicated in the pathogenesis of many neurological diseases, such as intraventricular haemorrhage, hypoxic-ischaemic encephalopathy and epilepsy.

  19. Epileptic Encephalopathies in Adults and Childhood

    PubMed Central

    Kural, Zekiye; Ozer, Ali Fahir

    2012-01-01

    Epileptic encephalopathies are motor-mental retardations or cognitive disorders secondary to epileptic seizures or epileptiform activities. Encephalopaties due to brain damage, medications, or systemic diseases are generally not in the scope of this definition, but they may rarely accompany the condition. Appropriate differential diagnosis of epileptic seizures as well as subclinical electroencephalographic discharges are crucial for management of seizures and epileptiform discharges and relative regression of cognitive deterioration in long-term followup. Proper antiepileptic drug, hormonal treatment, or i.v. immunoglobulin choice play major role in prognosis. In this paper, we evaluated the current treatment approaches by reviewing clinical electrophysiological characteristics of epileptic encephalopathies. PMID:23056934

  20. Cell culture models of transmissible spongiform encephalopathies.

    PubMed

    Béranger, F; Mangé, A; Solassol, J; Lehmann, S

    2001-11-30

    In this review, we describe the generation and use of cell culture models of transmissible spongiform encephalopathies, also known as prion diseases. These models include chronically prion-infected cell lines, as well as cultures expressing variable amounts of wild-type, mutated, or chimeric prion proteins. These cell lines have been widely used to investigate the biology of both the normal and the pathological isoform of the prion protein. They have also contributed to the comprehension of the pathogenic processes occurring in transmissible spongiform encephalopathies and in the development of new therapeutic approaches of these diseases.

  1. Minimal Hepatic Encephalopathy Impairs Quality of Life

    PubMed Central

    Agrawal, Swastik; Umapathy, Sridharan; Dhiman, Radha K.

    2015-01-01

    Minimal hepatic encephalopathy (MHE) is the mildest form of the spectrum of neurocognitive impairment in cirrhosis. It is a frequent occurrence in patients of cirrhosis and is detectable only by specialized neurocognitive testing. MHE is a clinically significant disorder which impairs daily functioning, driving performance, work capability and learning ability. It also predisposes to the development of overt hepatic encephalopathy, increased falls and increased mortality. This results in impaired quality of life for the patient as well as significant social and economic burden for health providers and care givers. Early detection and treatment of MHE with ammonia lowering therapy can reverse MHE and improve quality of life. PMID:26041957

  2. [Neuroprotection with hypothermia in the newborn with hypoxic-ischaemic encephalopathy. Standard guidelines for its clinical application].

    PubMed

    Blanco, D; García-Alix, A; Valverde, E; Tenorio, V; Vento, M; Cabañas, F

    2011-11-01

    Standardisation of hypothermia as a treatment for perinatal hypoxic-ischaemic encephalopathy is supported by current scientific evidence. The following document was prepared by the authors on request of the Spanish Society of Neonatology and is intended to be a guide for the proper implementation of this therapy. We discuss the difficulties that may arise when moving from the strict framework of clinical trials to clinical daily care: early recognition of clinical encephalopathy, inclusion and exclusion criteria, hypothermia during transport, type of hypothermia (selective head or systemic cooling) and side effects of therapy. The availability of hypothermia therapy has changed the prognosis of children with hypoxic-ischaemic encephalopathy and our choices of therapeutic support. In this sense, it is especially important to be aware of the changes in the predictive value of the neurological examination and the electroencephalographic recording in cooled infants. In order to improve neuroprotection with hypothermia we need earlier recognition of to recognise earlier the infants that may benefit from cooling. Biomarkers of brain injury could help us in the selection of these patients. Every single infant treated with hypothermia must be included in a follow up program in order to assess neurodevelopmental outcome.

  3. Animal-related fatalities--part II: characteristic autopsy findings and variable causes of death associated with envenomation, poisoning, anaphylaxis, asphyxiation, and sepsis.

    PubMed

    Bury, Danielle; Langlois, Neil; Byard, Roger W

    2012-03-01

    In addition to blunt and sharp trauma, animal-related fatalities may result from envenomation, poisoning, anaphylaxis, asphyxiation, and sepsis. Although the majority of envenomation deaths are caused by hornets, bees, and wasps, the mechanism of death is most often anaphylaxis. Envenomation resulting from the injection of a poison or toxin into a victim occurs with snakes, spiders, and scorpions on land. Marine animal envenomation may result from stings and bites from jellyfish, octopus, stonefish, cone fish, stingrays, and sea snakes. At autopsy, the findings may be extremely subtle, and so a history of exposure is required. Poisoning may also occur from ingesting certain fish, with three main forms of neurotoxin poisoning involving ciguatera, tetrodotoxin ingestion, and paralytic shellfish poisoning. Asphyxiation may follow upper airway occlusion or neck/chest compression by animals, and sepsis may follow bites. Autopsy analysis of cases requires extensive toxinological, toxicological, and biochemical analyses of body fluids. © 2011 American Academy of Forensic Sciences.

  4. The physical and psychological effects of HIV infection and its treatment on perinatally HIV-infected children.

    PubMed

    Vreeman, Rachel C; Scanlon, Michael L; McHenry, Megan S; Nyandiko, Winstone M

    2015-01-01

    As highly active antiretroviral therapy (HAART) transforms human immunodeficiency virus (HIV) into a manageable chronic disease, new challenges are emerging in treating children born with HIV, including a number of risks to their physical and psychological health due to HIV infection and its lifelong treatment. We conducted a literature review to evaluate the evidence on the physical and psychological effects of perinatal HIV (PHIV+) infection and its treatment in the era of HAART, including major chronic comorbidities. Perinatally infected children face concerning levels of treatment failure and drug resistance, which may hamper their long-term treatment and result in more significant comorbidities. Physical complications from PHIV+ infection and treatment potentially affect all major organ systems. Although treatment with antiretroviral (ARV) therapy has reduced incidence of severe neurocognitive diseases like HIV encephalopathy, perinatally infected children may experience less severe neurocognitive complications related to HIV disease and ARV neurotoxicity. Major metabolic complications include dyslipidaemia and insulin resistance, complications that are associated with both HIV infection and several ARV agents and may significantly affect cardiovascular disease risk with age. Bone abnormalities, particularly amongst children treated with tenofovir, are a concern for perinatally infected children who may be at higher risk for bone fractures and osteoporosis. In many studies, rates of anaemia are significantly higher for HIV-infected children. Renal failure is a significant complication and cause of death amongst perinatally infected children, while new data on sexual and reproductive health suggest that sexually transmitted infections and birth complications may be additional concerns for perinatally infected children in adolescence. Finally, perinatally infected children may face psychological challenges, including higher rates of mental health and behavioural

  5. The physical and psychological effects of HIV infection and its treatment on perinatally HIV-infected children

    PubMed Central

    Vreeman, Rachel C; Scanlon, Michael L; McHenry, Megan S; Nyandiko, Winstone M

    2015-01-01

    Introduction As highly active antiretroviral therapy (HAART) transforms human immunodeficiency virus (HIV) into a manageable chronic disease, new challenges are emerging in treating children born with HIV, including a number of risks to their physical and psychological health due to HIV infection and its lifelong treatment. Methods We conducted a literature review to evaluate the evidence on the physical and psychological effects of perinatal HIV (PHIV+) infection and its treatment in the era of HAART, including major chronic comorbidities. Results and discussion Perinatally infected children face concerning levels of treatment failure and drug resistance, which may hamper their long-term treatment and result in more significant comorbidities. Physical complications from PHIV+ infection and treatment potentially affect all major organ systems. Although treatment with antiretroviral (ARV) therapy has reduced incidence of severe neurocognitive diseases like HIV encephalopathy, perinatally infected children may experience less severe neurocognitive complications related to HIV disease and ARV neurotoxicity. Major metabolic complications include dyslipidaemia and insulin resistance, complications that are associated with both HIV infection and several ARV agents and may significantly affect cardiovascular disease risk with age. Bone abnormalities, particularly amongst children treated with tenofovir, are a concern for perinatally infected children who may be at higher risk for bone fractures and osteoporosis. In many studies, rates of anaemia are significantly higher for HIV-infected children. Renal failure is a significant complication and cause of death amongst perinatally infected children, while new data on sexual and reproductive health suggest that sexually transmitted infections and birth complications may be additional concerns for perinatally infected children in adolescence. Finally, perinatally infected children may face psychological challenges, including

  6. Metronidazole-induced encephalopathy in a patient with liver cirrhosis

    PubMed Central

    Cheong, Hyeong Cheol; Jeong, Taek Geun; Cho, Young Bum; Yang, Bong Joon; Kim, Tae Hyeon; Kim, Haak Cheoul

    2011-01-01

    Encephalopathy is a disorder characterized by altered brain function, which can be attributed to various causes. Encephalopathy associated with metronidazole administration occurs rarely and depends on the cumulative metronidazole dose, and most patients with this condition recover rapidly after discontinuation of therapy. Because metronidazole is metabolized in the liver and can be transported by the cerebrospinal fluid and cross the blood-brain barrier, it may induce encephalopathy even at a low cumulative dose in patients with hepatic dysfunction. We experienced a patient who showed ataxic gait and dysarthric speech after receiving metronidazole for the treatment of hepatic encephalopathy that was not controlled by the administration of lactulose. The patient was diagnosed as metronidazole-induced encephalopathy, and stopping drug administration resulted in a complete recovery from encephalopathy. This case shows that caution should be exercised when administering metronidazole because even a low dose can induce encephalopathy in patients with liver cirrhosis. PMID:21757988

  7. A Piglet Model of Neonatal Hypoxic-Ischemic Encephalopathy

    PubMed Central

    Kyng, Kasper J.; Skajaa, Torjus; Kerrn-Jespersen, Sigrid; Andreassen, Christer S.; Bennedsgaard, Kristine; Henriksen, Tine B.

    2015-01-01

    Birth asphyxia, which causes hypoxic-ischemic encephalopathy (HIE), accounts for 0.66 million deaths worldwide each year, about a quarter of the world’s 2.9 million neonatal deaths. Animal models of HIE have contributed to the understanding of the pathophysiology in HIE, and have highlighted the dynamic process that occur in brain injury due to perinatal asphyxia. Thus, animal studies have suggested a time-window for post-insult treatment strategies. Hypothermia has been tested as a treatment for HIE in pdiglet models and subsequently proven effective in clinical trials. Variations of the model have been applied in the study of adjunctive neuroprotective methods and piglet studies of xenon and melatonin have led to clinical phase I and II trials1,2. The piglet HIE model is further used for neonatal resuscitation- and hemodynamic studies as well as in investigations of cerebral hypoxia on a cellular level. However, it is a technically challenging model and variations in the protocol may result in either too mild or too severe brain injury. In this article, we demonstrate the technical procedures necessary for establishing a stable piglet model of neonatal HIE. First, the newborn piglet (< 24 hr old, median weight 1500 g) is anesthetized, intubated, and monitored in a setup comparable to that found in a neonatal intensive care unit. Global hypoxia-ischemia is induced by lowering the inspiratory oxygen fraction to achieve global hypoxia, ischemia through hypotension and a flat trace amplitude integrated EEG (aEEG) indicative of cerebral hypoxia. Survival is promoted by adjusting oxygenation according to the aEEG response and blood pressure. Brain injury is quantified by histopathology and magnetic resonance imaging after 72 hr. PMID:26068784

  8. Guidelines for Perinatal Care. Second Edition.

    ERIC Educational Resources Information Center

    American Coll. of Obstetricians and Gynecologists, Washington, DC.

    The basic concept emphasized in this book is that a coordinated, multidisciplinary approach within a regionalized system of perinatal care is a constant factor improving the quality of pregancy outcomes. This coordinated multidisciplinary approach has had an impact on perinatal care in three important areas: (1) improved and expanded understanding…

  9. Minimal Brain Dysfunction: Associations with Perinatal Complications.

    ERIC Educational Resources Information Center

    Nichols, Paul L.

    Examined with over 28,000 7-year-old children whose mothers registered for prenatal care was the relationship between perinatal complications and such characteristics as poor school achievement, hyperactivity, and neurological soft signs associated with the diagnosis of minimal brain dysfunction (MBD). Ten perinatal antecedents were studied:…

  10. Guidelines for Perinatal Care. Second Edition.

    ERIC Educational Resources Information Center

    American Coll. of Obstetricians and Gynecologists, Washington, DC.

    The basic concept emphasized in this book is that a coordinated, multidisciplinary approach within a regionalized system of perinatal care is a constant factor improving the quality of pregancy outcomes. This coordinated multidisciplinary approach has had an impact on perinatal care in three important areas: (1) improved and expanded understanding…

  11. BCAP31-associated encephalopathy and complex movement disorder mimicking mitochondrial encephalopathy.

    PubMed

    Albanyan, Saleh; Al Teneiji, Amal; Monfared, Nasim; Mercimek-Mahmutoglu, Saadet

    2017-06-01

    BCAP31, encoded by BCAP31, is involved in the export of transmembrane proteins from the endoplasmic reticulum. Pathogenic variants in BCAP31 results in global developmental delay, dystonia, deafness and dysmorphic features in males, called deafness, dystonia, and cerebral hypomyelination (DDCH) syndrome. We report a new patient with BCAP3-associated encephalopathy, DDCH syndrome, sensorineural hearing loss, generalized dystonia, and choreoathetosis. This 3.5-year-old boy had microcephaly and failure to thrive within the first 3 months of life. His brain MRI showed bilateral increased signal intensity in globus pallidus at age 3 months raising the suspicion of mitochondrial encephalopathy. His muscle biopsy revealed pleomorphic subsarcolemmal mitochondria collection in electron microscopy. Respiratory chain enzyme activities were normal in muscle. He was enrolled to a whole exome sequencing research study, which identified a hemizygous likely pathogenic truncating variant (c.533_536dup; p.Ser180AlafsX6) in BCAP31, inherited from his mother, who had sensorineural hearing loss and normal cognitive functions. We report a new patient with BCAP31-associated encephalopathy, DDCH syndrome, mimicking mitochondrial encephalopathy. We also report a heterozygous mother who has bilateral sensorineural hearing loss. This patient's clinical features, muscle histopathology, brain MRI features, and family history were suggestive of mitochondrial encephalopathy. Whole exome sequencing research study confirmed the diagnosis of BCAP31-associated encephalopathy, DDCH syndrome. © 2017 Wiley Periodicals, Inc.

  12. Value of retinal examination in hypertensive encephalopathy.

    PubMed

    Amraoui, F; van Montfrans, G A; van den Born, B J H

    2010-04-01

    The presence of grade III or IV hypertensive retinopathy (HRP) is considered to distinguish hypertensive urgencies from emergencies. However, case-reports suggest that these retinal changes may be lacking in patients with hypertensive encephalopathy. To assess the frequency of grade III and IV retinopathy in this hypertensive emergency, we conducted a retrospective cohort study. We retrieved 162 patients with malignant hypertension and 34 patients (17%) fulfilled the predefined criteria for hypertensive encephalopathy. Data on retinal examination were incomplete for 6 patients (18%), thus leaving 28 patients who were analysed for the presence or absence of grade III and IV HRP. In 9 (32%) patients with hypertensive encephalopathy, grade III or IV HRP was absent, 11 (39%) patients presented with grade III and 8 (29%) patients with grade IV retinopathy. Patients without retinal abnormalities were on average 13 years younger (P=0.05), more often black (P=0.02) and displayed lower blood pressure (BP) values (P=0.04 for systolic and diastolic BP). A substantial proportion of patients with hypertensive encephalopathy lack grade III or IV HRP. This suggests that the decision to admit these patients should not only rely on the presence of grade III and IV retinopathy alone, but should also include a careful neurological examination.

  13. Hypertensive encephalopathy complicating transplant renal artery stenosis.

    PubMed Central

    McGonigle, R. J.; Bewick, M.; Trafford, J. A.; Parsons, V.

    1984-01-01

    A 26-year-old female diabetic patient developed hypertensive encephalopathy with gross neurological abnormalities complicating renal artery stenosis of her transplant kidney. The elevated blood pressure was unresponsive to medical treatment. Surgical correction of the stenoses in the renal artery cured the hypertension and renal failure and led to the patient's complete recovery. Images Fig. 1 PMID:6377286

  14. Understanding Genotypes and Phenotypes in Epileptic Encephalopathies

    PubMed Central

    Helbig, Ingo; Tayoun, Abou Ahmad N.

    2016-01-01

    Epileptic encephalopathies are severe often intractable seizure disorders where epileptiform abnormalities contribute to a progressive disturbance in brain function. Often, epileptic encephalopathies start in childhood and are accompanied by developmental delay and various neurological and non-neurological comorbidities. In recent years, this concept has become virtually synonymous with a group of severe childhood epilepsies including West syndrome, Lennox-Gastaut syndrome, Dravet syndrome, and several other severe childhood epilepsies for which genetic factors are increasingly recognized. In the last 5 years, the field has seen a virtual explosion of gene discovery, raising the number of bona fide genes and possible candidate genes for epileptic encephalopathies to more than 70 genes, explaining 20-25% of all cases with severe early-onset epilepsies that had otherwise no identifiable causes. This review will focus on the phenotypic variability as a characteristic aspect of genetic epilepsies. For many genetic epilepsies, the phenotypic presentation can be broad, even in patients with identical genetic alterations. Furthermore, patients with different genetic etiologies can have seemingly similar clinical presentations, such as in Dravet syndrome. While most patients carry mutations in SCN1A, similar phenotypes can be seen in patients with mutations in PCDH19, CHD2, SCN8A, or in rare cases GABRA1 and STXBP1. In addition to the genotypic and phenotypic heterogeneity, both benign phenotypes and severe encephalopathies have been recognized in an increasing number of genetic epilepsies, raising the question whether these conditions represent a fluid continuum or distinct entities. PMID:27781027

  15. PRIONS AND THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

    EPA Science Inventory

    This book chapter is an invited, scholarly review of the mechanism(s) of TSEs for the 2nd edition of Metabolic Encephalopathies. Each chapter in the book assumes a professional knowledge of neuroscience and biochemistry, and the focus of the book is on the metabolic basis of dise...

  16. PRIONS AND THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

    EPA Science Inventory

    This book chapter is an invited, scholarly review of the mechanism(s) of TSEs for the 2nd edition of Metabolic Encephalopathies. Each chapter in the book assumes a professional knowledge of neuroscience and biochemistry, and the focus of the book is on the metabolic basis of dise...

  17. Encephalopathy in children with chronic renal failure.

    PubMed

    Baluarte, H J; Gruskin, A B; Hiner, L B; Foley, C M; Grover, W D

    1977-01-01

    The progressive encephalopathy observed in 5 children with chronic renal failure was clinically similar to the so-called dialysis encephalopathy of adults, except that it was not related to dialysis therapy. Renal osteodystrophy is more prevalent in children than in adults and often more severe. The attempt to control the crippling deformities of renal osteodystrophy in growing children with renal insufficiency has led to the use of large quantities of aluminum containing antacids. The encephalopathy observed in children with chronic renal failure may be related to the oral ingestion of aluminum containing compounds in the presence of persistent secondary hyperparathyroidism. We suggest that alternative methods for the adequate control of serum phosphorus levels should be sought and indications for parathyroidectomy in children reevaluated. During the past 18 mos we have lowered the dose of aluminum containing compounds to 50 to 100 mg/Kg/day in our patients with progressive renal failure and recommend parathyroidectomy. No new cases of the encephalopathy have occurred.

  18. Neuropsychological functioning in Wernicke's encephalopathy

    PubMed Central

    Behura, Sushree Sangita; Swain, Sarada Prasanna

    2015-01-01

    Context: Wernicke's encephalopathy (WE) is caused by thiamine (Vitamin B1) deficiency and most commonly found in chronic alcoholism and malnutrition. Clinically, the key features are mental status disturbances (global confusion), oculomotor abnormalities, and gait disturbances (ataxia). Apart from these clinical features, we can find deficits in neuropsychological functioning in patients with WE, which is more prominent after the improvement in the physical conditions. Neuropsychological functioning includes both basic cognitive processes (i.e., attention-concentration) as well as higher order cognitive processes (i.e., memory, executive functioning, reasoning), which is much vital for the maintenance of quality of life of an individual. However, unfortunately, in most of the cases, neuropsychological functioning is ignored by the clinicians. Materials and Methods: In this study four case reports of WE have been presented. The patients were taken from the outdoor department of Mental Health Institute, S.C.B. Medical College, Cuttack, Odisha. Neuropsychological functioning was measured by administration of PGIBBD and Quality of Life was measured by WHO-QOL BREF Odia Version. Discussion: As described in the literature, among the three cardinal signs (global confusion, ataxia, and ocular sings), the first two were present in all cases, but nystagmus was present in only two cases. Memory dysfunction was so disabling that the persons were unable to maintain a good Quality of Life and occupational impairment was prominent. There are disturbances in recent, remote memory, immediate recall, delayed recall, and attention and concentration, ultimately creating both physical and mental disability. PGI-BBD findings also suggest the overall impairment in neuropsychological functioning other than memory, that is, executive functioning, visual acuity, and depth perception. Findings of WHO-QOL BREF suggest the impairment of four domains of QOL in all the cases, but the severity

  19. Perinatal Safety: From Concept to Nursing Practice

    PubMed Central

    Kennedy, Holly Powell

    2010-01-01

    Communication and teamwork problems are leading causes of documented preventable adverse outcomes in perinatal care. An essential component of perinatal safety is the organizational culture in which clinicians work. Clinicians’ individual and collective authority to question the plan of care and take action to change the direction of a clinical situation in the patient’s best interest can be viewed as their “agency for safety.” However, collective agency for safety and commitment to support nurses in their advocacy role is missing in many perinatal care settings. This paper draws from Organizational Accident Theory, High Reliability Theory, and Symbolic Interactionism to describe the nurse’s role in maintaining safety during labor and birth in acute care settings, and suggests actions for supporting the perinatal nurse at individual, group, and systems levels to achieve maximum safety in perinatal care. PMID:20147827

  20. Perinatal Depression: An Update and Overview

    PubMed Central

    Stuart-Parrigon, Kaela

    2016-01-01

    Over the last 3 years there have been notable developments in screening and treatment of perinatal depression. Most importantly, the DSM-V has made only minor changes in the diagnostic criteria for perinatal depression as compared to the DSM-IV; “perinatal”, as opposed to “postpartum”, is a specifier for depression with a requirement that the depression onset occurs during pregnancy or the first 4 weeks postpartum. Advances in the treatment of perinatal depression have been made over the last 3 years, including both prevention and acute interventions. Additional support has emerged confirming the primary risk factors for perinatal depression: a personal or family history, low SES, and poor interpersonal support. There is general agreement that universal screening be conducted for all perinatal women, both by the woman’s obstetrician and the baby’s pediatrician. PMID:25034859

  1. A literature review on integrated perinatal care

    PubMed Central

    Rodríguez, Charo; des Rivières-Pigeon, Catherine

    2007-01-01

    Context The perinatal period is one during which health care services are in high demand. Like other health care sub-sectors, perinatal health care delivery has undergone significant changes in recent years, such as the integrative wave that has swept through the health care industry since the early 1990s. Purpose The present study aims at reviewing scholarly work on integrated perinatal care to provide support for policy decision-making. Results Researchers interested in integrated perinatal care have, by assessing the effectiveness of individual clinical practices and intervention programs, mainly addressed issues of continuity of care and clinical and professional integration. Conclusions Improvements in perinatal health care delivery appear related not to structurally integrated health care delivery systems, but to organizing modalities that aim to support woman-centred care and cooperative clinical practice. PMID:17786177

  2. Short-term outcome of magnesium sulfate infusion in perinatal asphyxia.

    PubMed

    Hossain, M M; Mannan, M A; Yeasmin, F; Shaha, C K; Rahman, M H; Shahidullah, M

    2013-10-01

    This randomized, single blind, controlled, clinical trial was done to see the effect of magnesium sulfate infusion in perinatal asphyxia. This study was conducted in the Department of Neonatology, Bangabandhu Sheikh Mujib Medical University and Dhaka Medical College Hospital from January, 2010 to October, 2010. Total 50 term neonates having postnatal age less than 12 hours with history of perinatal asphyxia and had history of hypoxic ischemic encephalopathy (moderate or severe) were included in this study. Patients were assigned randomly to receive either 3 doses of magnesium sulfate infusion at 250mg/kg per dose (0.5ml/kg per dose) 24 hours apart (experimental group) or 3 doses of normal saline infusion 24 hours apart (placebo-controlled group). Both groups also received supportive care according to the unit protocol for perinatal asphyxia. Baseline characteristics of 50 neonates had no differences in gestational age, birth weight, gender, mode and place of delivery, parity, ANC, liquor colour and hypoxic-ischemic encephalopathy (HIE) staging and mean age of intervention between the experimental and controlled groups. The mean serum magnesium at admission was 1.6±0.3mg/dl and 1.8±0.4mg/dl and after 48 hours was 3.9±0.6mg/dl and 1.9±0.2mg/dl in experimental group and in controlled group respectively. There was no significant difference or alteration in colour, heart rate, respiration, capillary filling time/blood pressure and oxygen saturation between the experimental and control groups. At discharge, 26% (5 of 19) of infants in the experimental group had neurological abnormalities, compared with 61% (11 of 18) of infants in the control group. At discharge experimental group were received more (78% vs. 44%) oral feedings by sucking compared with the controlled group. There is no significant difference in Electroencephalographic (EEG) abnormalities between groups. Good short-term outcomes at discharge were seen more (60% vs. 32%) in the experimental group

  3. Maternal arrhythmia and perinatal outcomes

    PubMed Central

    Henry, Dana; Gonzalez, Juan M; Harris, Ian, S.; Sparks, Teresa; Killion, Molly; Thiet, Mari-Paule; Bianco, Katherine

    2016-01-01

    Objective To determine if arrhythmia in the setting of maternal cardiac disease (MCD) affects perinatal outcomes. Study Design This is a retrospective cohort study of pregnant women with MCD who delivered from 2008 to 2013. Perinatal outcomes among women with an arrhythmia were compared to those without. Result Among 143 women; 36 (25%) had an arrhythmia. Those with an arrhythmia were more likely to have a spontaneous vaginal delivery (64% vs. 43%, p < 0.05) and required fewer operative vaginal births (8% vs. 27%, p=0.02). Pregnancies were more likely to be complicated by IUGR (17% vs. 5%, p < 0.05) although there were no differences in the rate of small for gestational age. The risk of IUGR remained increased after controlling for confounding (aOR 6.98, 95% CI 1.59–30.79, p=0.01). Two cases of placental abruption were identified among mothers with arrhythmia while none were identified in the controls (p < 0.05) Conclusion Patients with arrhythmias were more likely to have a spontaneous vaginal delivery. Our data suggests that these pregnancies were an increased risk for IUGR. PMID:27309629

  4. Measuring perinatal mental health risk.

    PubMed

    Johnson, M; Schmeid, V; Lupton, S J; Austin, M-P; Matthey, S M; Kemp, L; Meade, T; Yeo, A E

    2012-10-01

    The purpose of this review was to critically analyse existing tools to measure perinatal mental health risk and report on the psychometric properties of the various approaches using defined criteria. An initial literature search revealed 379 papers, from which 21 papers relating to ten instruments were included in the final review. A further four papers were identified from experts (one excluded) in the field. The psychometric properties of six multidimensional tools and/or criteria were assessed. None of the instruments met all of the requirements of the psychometric properties defined. Some had used large sample sizes but reported low positive predictive values (Antenatal Risk Questionnaire (ANRQ)) or insufficient information regarding their clinical performance (Antenatal Routine Psychosocial Assessment (ARPA)), while others had insufficient sample sizes (Antenatal Psychosocial Health Assessment Tool, Camberwell Assessment of Need-Mothers and Contextual Assessment of Maternity Experience). The ANRQ has fulfilled the requirements of this analysis more comprehensively than any other instrument examined based on the defined rating criteria. While it is desirable to recommend a tool for clinical practice, it is important that clinicians are made aware of their limitations. The ANRQ and ARPA represent multidimensional instruments commonly used within Australia, developed within large samples with either cutoff scores or numbers of risk factors related to service outcomes. Clinicians can use these tools, within the limitations presented here, to determine the need for further intervention or to refer women to mental health services. However, the effectiveness of routine perinatal psychosocial assessment continues to be debated, with further research required.

  5. Magnetic resonance imaging spectrum of perinatal hypoxic-ischemic brain injury.

    PubMed

    Varghese, Binoj; Xavier, Rose; Manoj, V C; Aneesh, M K; Priya, P S; Kumar, Ashok; Sreenivasan, V K

    2016-01-01

    Perinatal hypoxic-ischemic brain injury results in neonatal hypoxic-ischemic encephalopathy and serious long-term neurodevelopmental sequelae. Magnetic resonance imaging (MRI) of the brain is an ideal and safe imaging modality for suspected hypoxic-ischemic injury. The pattern of injury depends on brain maturity at the time of insult, severity of hypotension, and duration of insult. Time of imaging after the insult influences the imaging findings. Mild to moderate hypoperfusion results in germinal matrix hemorrhages and periventricular leukomalacia in preterm neonates and parasagittal watershed territory infarcts in full-term neonates. Severe insult preferentially damages the deep gray matter in both term and preterm infants. However, associated frequent perirolandic injury is seen in term neonates. MRI is useful in establishing the clinical diagnosis, assessing the severity of injury, and thereby prognosticating the outcome. Familiarity with imaging spectrum and insight into factors affecting the injury will enlighten the radiologist to provide an appropriate diagnosis.

  6. Magnetic resonance imaging spectrum of perinatal hypoxic-ischemic brain injury

    PubMed Central

    Varghese, Binoj; Xavier, Rose; Manoj, V C; Aneesh, M K; Priya, P S; Kumar, Ashok; Sreenivasan, V K

    2016-01-01

    Perinatal hypoxic–ischemic brain injury results in neonatal hypoxic–ischemic encephalopathy and serious long-term neurodevelopmental sequelae. Magnetic resonance imaging (MRI) of the brain is an ideal and safe imaging modality for suspected hypoxic–ischemic injury. The pattern of injury depends on brain maturity at the time of insult, severity of hypotension, and duration of insult. Time of imaging after the insult influences the imaging findings. Mild to moderate hypoperfusion results in germinal matrix hemorrhages and periventricular leukomalacia in preterm neonates and parasagittal watershed territory infarcts in full-term neonates. Severe insult preferentially damages the deep gray matter in both term and preterm infants. However, associated frequent perirolandic injury is seen in term neonates. MRI is useful in establishing the clinical diagnosis, assessing the severity of injury, and thereby prognosticating the outcome. Familiarity with imaging spectrum and insight into factors affecting the injury will enlighten the radiologist to provide an appropriate diagnosis. PMID:27857456

  7. Perinatal Mortality in the United States, 1950-81.

    ERIC Educational Resources Information Center

    Powell-Griner, Eve

    1986-01-01

    This report describes long-term trends in perinatal mortality in the United States in three basic parts: development of perinatal mortality measures, components of fetal and infant mortality, and trends and differentials in perinatal mortality. Perinatal deaths refer to the sum of spontaneous fetal deaths occurring after 20 weeks gestation plus…

  8. End-tidal CO2 Detection of an Audible Heart Rate During Neonatal Cardiopulmonary Resuscitation Following Asystole in Asphyxiated Piglets

    PubMed Central

    Chalak, Lina F.; Barber, Chad A.; Hynan, Linda; Garcia, Damian; Christie, Lucy; Wyckoff, Myra H.

    2011-01-01

    Even brief interruption of cardiac compressions significantly reduces critical coronary perfusion pressure during cardiopulmonary resuscitation (CPR). End-tidal CO2 (ETCO2) monitoring may provide a continuous non-invasive method of assessing return of spontaneous circulation (ROSC) without stopping to auscultate for heart rate (HR). However, the ETCO2 value that correlates with an audible HR is unknown. Our objective was to determine the threshold ETCO2 that is associated with ROSC following asphyxia-induced asystole. Neonatal swine (n=46) were progressively asphyxiated until asystole occurred. Resuscitation followed current neonatal guidelines with initial ventilation with 100% O2 followed by cardiac compressions followed by epinephrine for continued asystole. HR was auscultated every 30 sec and ETCO2 was continuously recorded. A receiver operator curve was generated using the calculated sensitivity and specificity for various ETCO2 values where a positive test was defined as the presence of HR >60 bpm by auscultation. An ETCO2 cut off value of 14 mmHg is the most sensitive ETCO2 value with the least false positives. When using ETCO2 to guide uninterrupted CPR in this model of asphyxia-induced asystole, auscultative confirmation of return of an adequate HR should be performed when ETCO2 ≥14 mmHg is achieved. Correlation during human neonatal CPR needs further investigation. PMID:21283051

  9. Comparison of Quantitative Characteristics of Early Post-resuscitation EEG Between Asphyxial and Ventricular Fibrillation Cardiac Arrest in Rats.

    PubMed

    Chen, Bihua; Chen, Gang; Dai, Chenxi; Wang, Pei; Zhang, Lei; Huang, Yuanyuan; Li, Yongqin

    2017-05-08

    Quantitative electroencephalogram (EEG) analysis has shown promising results in studying brain injury and functional recovery after cardiac arrest (CA). However, whether the quantitative characteristics of EEG, as potential indicators of neurological prognosis, are influenced by CA causes is unknown. The purpose of this study was designed to compare the quantitative characteristics of early post-resuscitation EEG between asphyxial CA (ACA) and ventricular fibrillation CA (VFCA) in rats. Thirty-two Sprague-Dawley rats of both sexes were randomized into either ACA or VFCA group. Cardiopulmonary resuscitation was initiated after 5-min untreated CA. Characteristics of early post-resuscitation EEG were compared, and the relationships between quantitative EEG features and neurological outcomes were investigated. Compared with VFCA, serum level of S100B, neurological deficit score and brain histopathologic damage score were dramatically higher in the ACA group. Quantitative measures of EEG, including onset time of EEG burst, time to normal trace, burst suppression ratio, and information quantity, were significantly lower for CA caused by asphyxia and correlated with the 96-h neurological outcome and survival. Characteristics of earlier post-resuscitation EEG differed between cardiac and respiratory causes. Quantitative measures of EEG not only predicted neurological outcome and survival, but also have the potential to stratify CA with different causes.

  10. Possible asphyxiation from carbon dioxide of a cross-country skier in eastern California: a deadly volcanic hazard.

    PubMed

    Hill, P M

    2000-01-01

    This report describes an incident in which exceedingly high levels of carbon dioxide may have contributed to the death of a skier in eastern California. A cross-country skier was found dead inside a large, mostly covered snow cave, 1 day after he was reported missing. The autopsy report suggests that the skier died of acute pulmonary edema consistent with asphyxiation; carbon dioxide measurements inside the hole in which he was found reached 70%. This area is known for having a high carbon dioxide flux attributed to degassing of a large body of magma (molten rock) 10 to 20 km beneath the ski area. The literature describes many incidents of fatal carbon dioxide exposures associated with volcanic systems in other parts of the world. We believe this case represents the first reported death associated with volcanically produced carbon dioxide in the United States. Disaster and wilderness medicine specialists should be aware of and plan for this potential health hazard associated with active volcanoes.

  11. The 2016 Lewis H. Wright Memorial Lecture: America's Doctor Anaesthetists (1862-1936)-Turning a Tide of Asphyxiating Waves.

    PubMed

    Bause, George S

    2017-01-01

    Laughing-gas showman G.Q. Colton franchised dental extraction under 100% nitrous oxide in many large American cities before popularizing the practice with French Imperial Court dentist T.W. Evans in France and then England. Chicago dentist Z. Rogers helped surgeon E. Andrews oxygenate nitrous oxide, with neither man changing significantly the clinical practices of others. London's F.W. Hewitt and Pittsburgh's S.J. Hayes oxygenated anesthetics with greater clinical impact. By 1920, E.I. McKesson had publicized his practice of secondary saturation with bursts of 100% nitrous oxide to relax musculature in anesthetized patients. In the banner year of 1936, (1) C.B. Courville published a paper about brain damage following hypoxic anesthetics, (2) pulse oximetry pioneer T. Aoyagi was born, and (3) a New York society nationalized into the American Society of Anesthetists, many of whose presidents would champion the adequate oxygenation and the monitoring of anesthetized patients. Many dental and medical doctors first promoted and then eventually opposed hypoxic anesthetics, finally turning the tide of asphyxiating waves.

  12. Continuous chest compression versus interrupted chest compression for cardiopulmonary resuscitation of non-asphyxial out-of-hospital cardiac arrest.

    PubMed

    Zhan, Lei; Yang, Li J; Huang, Yu; He, Qing; Liu, Guan J

    2017-03-27

    Out-of-hospital cardiac arrest (OHCA) is a major cause of death worldwide. Cardiac arrest can be subdivided into asphyxial and non asphyxial etiologies. An asphyxia arrest is caused by lack of oxygen in the blood and occurs in drowning and choking victims and in other circumstances. A non asphyxial arrest is usually a loss of functioning cardiac electrical activity. Cardiopulmonary resuscitation (CPR) is a well-established treatment for cardiac arrest. Conventional CPR includes both chest compressions and 'rescue breathing' such as mouth-to-mouth breathing. Rescue breathing is delivered between chest compressions using a fixed ratio, such as two breaths to 30 compressions or can be delivered asynchronously without interrupting chest compression. Studies show that applying continuous chest compressions is critical for survival and interrupting them for rescue breathing might increase risk of death. Continuous chest compression CPR may be performed with or without rescue breathing. To assess the effects of continuous chest compression CPR (with or without rescue breathing) versus conventional CPR plus rescue breathing (interrupted chest compression with pauses for breaths) of non-asphyxial OHCA. We searched the Cochrane Central Register of Controlled Trials (CENTRAL; Issue 1 2017); MEDLINE (Ovid) (from 1985 to February 2017); Embase (1985 to February 2017); Web of Science (1985 to February 2017). We searched ongoing trials databases including controlledtrials.com and clinicaltrials.gov. We did not impose any language or publication restrictions. We included randomized and quasi-randomized studies in adults and children suffering non-asphyxial OHCA due to any cause. Studies compared the effects of continuous chest compression CPR (with or without rescue breathing) with interrupted CPR plus rescue breathing provided by rescuers (bystanders or professional CPR providers). Two authors extracted the data and summarized the effects as risk ratios (RRs), adjusted risk

  13. Perinatal Complications and Aging Indicators by Midlife

    PubMed Central

    Caspi, Avshalom; Ambler, Antony; Belsky, Daniel W.; Chapple, Simon; Cohen, Harvey Jay; Israel, Salomon; Poulton, Richie; Ramrakha, Sandhya; Rivera, Christine D.; Sugden, Karen; Williams, Benjamin; Wolke, Dieter; Moffitt, Terrie E.

    2014-01-01

    BACKGROUND: Perinatal complications predict increased risk for morbidity and early mortality. Evidence of perinatal programming of adult mortality raises the question of what mechanisms embed this long-term effect. We tested a hypothesis related to the theory of developmental origins of health and disease: that perinatal complications assessed at birth predict indicators of accelerated aging by midlife. METHODS: Perinatal complications, including both maternal and neonatal complications, were assessed in the Dunedin Multidisciplinary Health and Development Study cohort (N = 1037), a 38-year, prospective longitudinal study of a representative birth cohort. Two aging indicators were assessed at age 38 years, objectively by leukocyte telomere length (TL) and subjectively by perceived facial age. RESULTS: Perinatal complications predicted both leukocyte TL (β = −0.101; 95% confidence interval, −0.169 to −0.033; P = .004) and perceived age (β = 0.097; 95% confidence interval, 0.029 to 0.165; P = .005) by midlife. We repeated analyses with controls for measures of family history and social risk that could predispose to perinatal complications and accelerated aging, and for measures of poor health taken in between birth and the age-38 follow-up. These covariates attenuated, but did not fully explain the associations observed between perinatal complications and aging indicators. CONCLUSIONS: Our findings provide support for early-life developmental programming by linking newborns’ perinatal complications to accelerated aging at midlife. We observed indications of accelerated aging “inside,” as measured by leukocyte TL, an indicator of cellular aging, and “outside,” as measured by perceived age, an indicator of declining tissue integrity. A better understanding of mechanisms underlying perinatal programming of adult aging is needed. PMID:25349321

  14. Qualifying and quantifying minimal hepatic encephalopathy.

    PubMed

    Morgan, Marsha Y; Amodio, Piero; Cook, Nicola A; Jackson, Clive D; Kircheis, Gerald; Lauridsen, Mette M; Montagnese, Sara; Schiff, Sami; Weissenborn, Karin

    2016-12-01

    Minimal hepatic encephalopathy is the term applied to the neuropsychiatric status of patients with cirrhosis who are unimpaired on clinical examination but show alterations in neuropsychological tests exploring psychomotor speed/executive function and/or in neurophysiological variables. There is no gold standard for the diagnosis of this syndrome. As these patients have, by definition, no recognizable clinical features of brain dysfunction, the primary prerequisite for the diagnosis is careful exclusion of clinical symptoms and signs. A large number of psychometric tests/test systems have been evaluated in this patient group. Of these the best known and validated is the Portal Systemic Hepatic Encephalopathy Score (PHES) derived from a test battery of five paper and pencil tests; normative reference data are available in several countries. The electroencephalogram (EEG) has been used to diagnose hepatic encephalopathy since the 1950s but, once popular, the technology is not as accessible now as it once was. The performance characteristics of the EEG are critically dependent on the type of analysis undertaken; spectral analysis has better performance characteristics than visual analysis; evolving analytical techniques may provide better diagnostic information while the advent of portable wireless headsets may facilitate more widespread use. A large number of other diagnostic tools have been validated for the diagnosis of minimal hepatic encephalopathy including Critical Flicker Frequency, the Inhibitory Control Test, the Stroop test, the Scan package and the Continuous Reaction Time; each has its pros and cons; strengths and weaknesses; protagonists and detractors. Recent AASLD/EASL Practice Guidelines suggest that the diagnosis of minimal hepatic encephalopathy should be based on the PHES test together with one of the validated alternative techniques or the EEG. Minimal hepatic encephalopathy has a detrimental effect on the well-being of patients and their care

  15. 76 FR 38667 - Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-07-01

    ... HUMAN SERVICES Food and Drug Administration Transmissible Spongiform Encephalopathies Advisory Committee... be open to the public. Name of Committee: Transmissible Spongiform Encephalopathies Advisory... available at the following link. Transmissible Spongiform Encephalopathies Advisory Committee http://fda...

  16. [Role of the perinatal sexologist in the interdisciplinary perinatal health care team in Canada].

    PubMed

    de Pierrepont, C; Polomeno, V

    2014-01-01

    Interdisciplinary health care teams are models of health care that are the way of the future. In this model, the sexologist has a unique and important role, particularly in perinatal health care where sexuality is a central component of health. Perinatal sexuality is a newly emerging discipline in which the perinatal sexologist has a double role to play: 1) to train other perinatal health professionals in sexuality; and 2) to educate and to intervene with future and new parenting couples by answering their multiple intimate and sexual questions and concerns during the transition to parenthood. Copyright © 2014. Published by Elsevier SAS.

  17. Hashimoto's encephalopathy: A rare proteiform disorder.

    PubMed

    Montagna, Giacomo; Imperiali, Mauro; Agazzi, Pamela; D'Aurizio, Federica; Tozzoli, Renato; Feldt-Rasmussen, Ulla; Giovanella, Luca

    2016-05-01

    Hashimoto's encephalopathy (HE) is a rare not well understood, progressive and relapsing multiform disease, characterized by seizures, movement disorders, subacute cognitive dysfunction, psychiatric symptoms and responsiveness to steroid therapy. The disorder is generally associated with thyroid diseases and the most common feature is the presence of anti-thyroperoxidase antibodies (TPOAb). Patients are usually euthyroid or mildly hypothyroid at presentation. All age groups can be affected. The pathophysiology is still unclear, especially the link between elevated serum TPOAb and the encephalopathy. Most reported cases occurred in women and girls. Unspecific symptoms, non-pathognomonic laboratory neurophysiology and neuroimaging features make its diagnosis a real challenge for clinicians. The case of a 16 year old boy, with a clinical picture of HE associated with hypothyroidism, demonstrating an excellent response to high dose steroids is presented together with a systematic review of the literature.

  18. Pathophysiology of septic encephalopathy - an unsolved puzzle

    PubMed Central

    2010-01-01

    The exact cellular and molecular mechanisms of sepsis-induced encephalopathy remain elusive. The breakdown of the blood-brain barrier (BBB) is considered a focal point in the development of sepsis-induced brain damage. Contributing factors for the compromise of the BBB include cytokines and chemokines, activation of the complement cascade, phagocyte-derived toxic mediators, and bacterial products. To date, we are far from fully understanding the neuropathology that develops as a secondary remote organ injury as a consequence of sepsis. However, recent studies suggest that bacterial proteins may readily cross the functional BBB and trigger an inflammatory response in the subarachnoid space, in absence of a bacterial invasion. A better understanding of the pathophysiological events leading to septic encephalopathy appears crucial to advance the clinical care for this vulnerable patient population. PMID:20565858

  19. Value of plasmapheresis in hepatic encephalopathy.

    PubMed

    Riviello, J J; Halligan, G E; Dunn, S P; Widzer, S J; Foley, C M; Breningstall, G N; Grover, W D

    1990-01-01

    Plasmapheresis is used for treating the complications of liver failure. We performed plasmapheresis on 6 children with hepatic encephalopathy resulting from acute hepatic failure and prospectively assessed its effects on neurologic and electrophysiologic (electroencephalography and evoked potentials) function. Clinical improvement was observed in 3 of 6 patients; changes in the serum ammonia value or the results of initial electrophysiologic tests did not predict the patient response. Two patients underwent transplantation after neurologic improvement was produced by plasmapheresis; however, despite plasmapheresis, 4 patients progressed to brain death. Our data demonstrate that plasmapheresis may transiently improve the encephalopathy of acute hepatic failure but is not curative alone. Therefore, plasmapheresis may be a useful adjunct in the treatment of liver failure, potentially improving the pretransplantation status of the patient.

  20. Duloxetine-related posterior reversible encephalopathy syndrome

    PubMed Central

    Zappella, Nathalie; Perier, François; Pico, Fernando; Palette, Catherine; Muret, Alexandre; Merceron, Sybille; Girbovan, Andrei; Marquion, Fabien; Legriel, Stephane

    2016-01-01

    Abstract Background: Posterior reversible encephalopathy syndrome (PRES) has well-established links with several drugs. Whether a link also exists with serotonin–norepinephrine reuptake inhibitor such as duloxetine is unclear. Methods: We report on a patient who developed PRES with a coma and myoclonus related to hypertensive encephalopathy a few days after starting duloxetine treatment. Magnetic resonance imaging was performed and catecholamine metabolites assayed. Results: The patient achieved a full recovery after aggressive antihypertensive therapy and intravenous anticonvulsant therapy. Conclusions: The clinical history, blood and urinary catecholamine and serotonin levels, and response to treatment strongly suggest that PRES was induced by duloxetine. Duloxetine should be added to the list of causes of PRES. PMID:27537580

  1. [Metabolic encephalopathy secondary to vitamin D intoxication].

    PubMed

    Herrera Martínez, Aura; Viñals Torràs, Montserrat; Muñoz Jiménez, Ma Concepción; Arenas de Larriva, Antonio Pablo; Molina Puerta, Ma José; Manzano García, Gregorio; Gálvez Moreno, Ma Ángeles; Calañas-Continente, Alfonso

    2014-10-25

    The association between vitamin D deficiency and increased risk of, among others, cardiovascular and autoimmune diseases has lead in the last years to an enhanced interest in the usage of supplements to achieve the normalization of plasmatic values at 25(OH) D. Apparently this search for normalization is resulting in an higher incidence on vitamin D intoxication. We present the case of an 81 years old woman with metabolic encephalopathy and renal failure secondary to iatrogenic vitamin D intoxication. Calcium and vitamin D oral supplements were prescribed after an osteoporotic vertebral fracture. The patient improved clinically as well as analytically after receiving treatment with diuretics and hydration. We emphasize the importance of discarding hypercalcemia as a cause of metabolic encephalopathy; moreover we highly recommend keeping vitamin D intoxication in mind as an uncommon although always possible etiology of reversible hypercalcemia and renal failure.

  2. Head stereotypies in STXBP1 encephalopathy.

    PubMed

    Kim, Young Ok; Korff, Christian M; Villaluz, Mel Michel G; Suls, Arvid; Weckhuysen, Sarah; De Jonghe, Peter; Scheffer, Ingrid E

    2013-08-01

    STXBP1 encephalopathy is associated with a range of movement disorders. We observed head stereotypies in three patients. These comprised a slow (<1Hz), high-amplitude, horizontal, 'figure-of-eight' pattern, beginning at age 4-6 years and resulting in neck muscle hypertrophy, in two males; a faster (2-3Hz), side-to-side, 'no' movement, starting at the age of 9 years 6 months was observed in one female. Upper limb and truncal stereotypies and vocalization occurred intermittently with the head movements. The stereotypies increased with excitement but settled with concentration and sleep. Head and upper limb stereotypies are valuable clinical clues to the diagnosis of STXBP1 encephalopathy in patients with profound impairments. © 2013 Mac Keith Press.

  3. Treatment of portal systemic encephalopathy: standard and new treatments.

    PubMed

    Marín, E; Uribe, M

    1990-07-01

    The management of hepatic encephalopathy should be considered accordingly with the precipitating factor and the type of encephalopathy. Ideally the therapeutic approach must be useful for both acute and chronic forms of encephalopathy. Current treatment of hepatic encephalopathy consists of certain well-established measures attempting to identify and treat the precipitating factors, and to reduce the intestinal nitrogenous compounds formation and absorption by dietary restriction or bowel-cleansing with catartics or antibiotics such as neomycin, metronidazol, etc. This review describes briefly several therapeutic modalities.

  4. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion.

    PubMed

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-09-01

    Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities. We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion. Healthcare providers should be aware of the dangers of baking soda misuse and the associated adverse effects.

  5. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion

    PubMed Central

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-01-01

    Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities. We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion. Healthcare providers should be aware of the dangers of baking soda misuse and the associated adverse effects. PMID:27625729

  6. Transmissible Spongiform Encephalopathy and Meat Safety

    NASA Astrophysics Data System (ADS)

    Ward, Hester J. T.; Knight, Richard S. G.

    Prion diseases or transmissible spongiform encephalopathies (TSEs) comprise a wide-ranging group of neurodegenerative diseases found in animals and humans. They have diverse causes and geographical distributions, but have similar pathological features, transmissibility and, are ultimately, fatal. Central to all TSEs is the presence of an abnormal form of a normal host protein, namely the prion protein. Because of their potential transmissibility, these diseases have wide public health ramifications.

  7. Medium chain triglycerides and hepatic encephalopathy

    PubMed Central

    Morgan, M. Hilary; Bolton, C. H.; Morris, J. S.; Read, A. E.

    1974-01-01

    The oral administration of short (C6) and medium (C8 and (C10) chain triglycerides produced no clinical or electroencephalographic changes in patients with cirrhosis of the liver. Arterial ammonia levels were also monitored in these patients and showed no significant change after medium chain triglycerides. It was concluded that medium chain triglycerides, known to be of potential value in the treatment of malabsorption in patients with cirrhosis, are not clinically contraindicated, even in patients with evidence of hepatic encephalopathy. PMID:4841275

  8. Management of Hepatic Encephalopathy in the Hospital

    PubMed Central

    Leise, Michael D.; Poterucha, John J.; Kamath, Patrick S.; Kim, W. Ray

    2014-01-01

    Hepatic encephalopathy (HE) develops in about 50% of patients with cirrhosis and is one of the features of decompensated cirrhosis. The inpatient incidence of HE is approximately 23,000/year and management of these patients is common for internists and subspecialists. Treatment of the hospitalized patient with HE has changed in recent years. Treatment entails two phases, induction and maintenance of remission. Most cases of significant hepatic encephalopathy are precipitated by infection, gastrointestinal bleeding, medications or other culprits. All patients should be evaluated for secondary triggers of HE and treatment should be initiated with a non-absorbable disaccharide (i.e. lactulose) in most cases. Rifaximin (off-label) can be added in patients not responding to lactulose. Neomycin is a less preferable alternative to rifaximin, due to its side effect profile. Other therapies including zinc, LOLA, and branch chain amino acids can be considered for patients not responding to a disaccharide and non-absorbable antibiotic. Large portosystemic shunts may be embolized in patients with medically refractory recurrent or severe HE with otherwise well compensated cirrhosis. Molecular Adsorbent Recirculating System is now available for patients with severe hepatic encephalopathy who do not respond to medical therapy. It is critically important that patients hospitalized with significant hepatic encephalopathy continue a maintenance medication(s) at the time of dismissal to prevent further episodes. Patients with a 1st time episode of HE can be placed on lactulose and careful instruction should be provided to patient and caregiver about titration of dose to achieve 3 bowel movements per day. Patients with recurrent HE episodes despite lactulose benefit from the addition of rifaximin which decreases the frequency of recurrent HE episodes and related hospitalizations. Lastly, patients and their families should be counselled about the risk of motor vehicle accidents which

  9. Vitamin-Responsive Epileptic Encephalopathies in Children

    PubMed Central

    Agadi, Satish; Quach, Michael M.

    2013-01-01

    Untreated epileptic encephalopathies in children may potentially have disastrous outcomes. Treatment with antiepileptic drugs (AEDs) often may not control the seizures, and even if they do, this measure is only symptomatic and not specific. It is especially valuable to identify potential underlying conditions that have specific treatments. Only a few conditions have definitive treatments that can potentially modify the natural course of disease. In this paper, we discuss the few such conditions that are responsive to vitamin or vitamin derivatives. PMID:23984056

  10. Urinary gas chromatography mass spectrometry metabolomics in asphyxiated newborns undergoing hypothermia: from the birth to the first month of life

    PubMed Central

    Noto, Antonio; Pomero, Giulia; Barberini, Luigi; Fattuoni, Claudia; Palmas, Francesco; Dalmazzo, Cristina; Delogu, Antonio; Dessì, Angelica; Fanos, Vassilios; Gancia, Paolo

    2016-01-01

    Background Perinatal asphyxia is a severe clinical condition affecting around four million newborns worldwide. It consists of an impaired gas exchange leading to three biochemical components: hypoxemia, hypercapnia and metabolic acidosis. Methods The aim of this longitudinal experimental study was to identify the urine metabolome of newborns with perinatal asphyxia and to follow changes in urine metabolic profile over time. Twelve babies with perinatal asphyxia were included in this study; three babies died on the eighth day of life. Total-body cooling for 72 hours was carried out in all the newborns. Urine samples were collected in each baby at birth, after 48 hours during hypothermia, after the end of the therapeutic treatment (72 hours), after 1 week of life, and finally after 1 month of life. Urine metabolome at birth was considered the reference against which to compare metabolic profiles in subsequent samples. Quantitative metabolic profiling in urine samples was measured by gas chromatography mass spectrometry (GC-MS). The statistical approach was conducted by using the multivariate analysis by means of principal component analysis (PCA) and orthogonal partial least square discriminant analysis (OPLS-DA). Pathway analysis was also performed. Results The most important metabolites depicting each time collection point were identified and compared each other. At birth before starting therapeutic hypothermia (TH), urine metabolic profiles of the three babies died after 7 days of life were closely comparable each other and significantly different from those in survivors. Conclusions In conclusion, a plethora of data have been extracted by comparing the urine metabolome at birth with those observed at each time point collection. The modifications over time in metabolites composition and concentration, mainly originated from the depletion of cellular energy and homeostasis, seems to constitute a fingerprint of perinatal asphyxia. PMID:27942508

  11. Reversible obstructive hydrocephalus from hypertensive encephalopathy.

    PubMed

    Kumar, Abhay; Keyrouz, Salah G; Willie, Jon T; Dhar, Rajat

    2012-06-01

    Diffuse edema involving the posterior fossa may be seen with hypertensive encephalopathy and has rarely been reported to cause hydrocephalus. We present three such cases and review the literature to better delineate this uniquely reversible syndrome. Case reports and review of literature. Three patients with hypertensive encephalopathy presented to our institutions with clinical and radiographic features of obstructive hydrocephalus associated with brainstem and cerebellar edema. This required transient external drainage of cerebrospinal fluid (CSF) in two of the three patients. However, with recognition of this unusual syndrome and aggressive management of elevated blood pressure, both edema and hydrocephalus resolved. All patients made complete recoveries and did not require permanent CSF shunting. A review of the literature yielded 15 additional case reports describing reversible obstructive hydrocephalus related to hypertensive encephalopathy. All had mean arterial pressures above 130 mmHg and presented primarily with altered mental status. While half required ventriculostomy, only one required shunting. Excluding a patient who died from sepsis, all recovered neurologically once blood pressure was controlled. It is imperative to recognize such cases where hypertension causes edema within the posterior fossa resulting in secondary hydrocephalus. Focusing management on lowering blood pressure avoids unnecessary or prolonged CSF diversion.

  12. Stimulus induced bursts in severe postanoxic encephalopathy.

    PubMed

    Tjepkema-Cloostermans, Marleen C; Wijers, Elisabeth T; van Putten, Michel J A M

    2016-11-01

    To report on a distinct effect of auditory and sensory stimuli on the EEG in comatose patients with severe postanoxic encephalopathy. In two comatose patients admitted to the Intensive Care Unit (ICU) with severe postanoxic encephalopathy and burst-suppression EEG, we studied the effect of external stimuli (sound and touch) on the occurrence of bursts. In patient A bursts could be induced by either auditory or sensory stimuli. In patient B bursts could only be induced by touching different facial regions (forehead, nose and chin). When stimuli were presented with relatively long intervals, bursts persistently followed the stimuli, while stimuli with short intervals (<1s) did not induce bursts. In both patients bursts were not accompanied by myoclonia. Both patients deceased. Bursts in patients with a severe postanoxic encephalopathy can be induced by external stimuli, resulting in stimulus-dependent burst-suppression. Stimulus induced bursts should not be interpreted as prognostic favourable EEG reactivity. Copyright © 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  13. Inflammatory Macrophages Promotes Development of Diabetic Encephalopathy.

    PubMed

    Wang, Beiyun; Miao, Ya; Zhao, Zhe; Zhong, Yuan

    2015-01-01

    Diabetes and Alzheimer's disease are often associated with each other, whereas the relationship between two diseases is ill-defined. Although hyperglycemia during diabetes is a major cause of encephalopathy, diabetes may also cause chronic inflammatory complications including peripheral neuropathy. Hence the role and the characteristics of inflammatory macrophages in the development of diabetic encephalopathy need to be clarified. Diabetes were induced in mice by i.p. injection of streptozotocin (STZ). Two weeks after STZ injection and confirmation of development of diabetes, inflammatory macrophages were eliminated by i.p. injection of 20µg saporin-conjugated antibody against a macrophage surface marker CD11b (saporin-CD11b) twice per week, while a STZ-treated group received injection of rat IgG of same frequency as a control. The effects of macrophage depletion on brain degradation markers, brain malondialdehyde (MDA), catalase, superoxidase anion-positive cells and nitric oxide (NO) were measured. Saporin-CD11b significantly reduced inflammatory macrophages in brain, without affecting mouse blood glucose, serum insulin, glucose responses and beta cell mass. However, reduced brain macrophages significantly inhibited the STZ-induced decreases in brain MDA, catalase and superoxidase anion-positive cells, and the STZ-induced decreases in brain NO. Inflammatory macrophages may promote development of diabetic encephalopathy. © 2015 S. Karger AG, Basel.

  14. Extending the KCNQ2 encephalopathy spectrum

    PubMed Central

    Weckhuysen, Sarah; Ivanovic, Vanja; Hendrickx, Rik; Van Coster, Rudy; Hjalgrim, Helle; Møller, Rikke S.; Grønborg, Sabine; Schoonjans, An-Sofie; Ceulemans, Berten; Heavin, Sinead B.; Eltze, Christin; Horvath, Rita; Casara, Gianluca; Pisano, Tiziana; Giordano, Lucio; Rostasy, Kevin; Haberlandt, Edda; Albrecht, Beate; Bevot, Andrea; Benkel, Ira; Syrbe, Steffan; Sheidley, Beth; Guerrini, Renzo; Poduri, Annapurna; Lemke, Johannes R.; Mandelstam, Simone; Scheffer, Ingrid; Angriman, Marco; Striano, Pasquale; Marini, Carla; Suls, Arvid

    2013-01-01

    Objectives: To determine the frequency of KCNQ2 mutations in patients with neonatal epileptic encephalopathy (NEE), and to expand the phenotypic spectrum of KCNQ2 epileptic encephalopathy. Methods: Eighty-four patients with unexplained NEE were screened for KCNQ2 mutations using classic Sanger sequencing. Clinical data of 6 additional patients with KCNQ2 mutations detected by gene panel were collected. Detailed phenotyping was performed with particular attention to seizure frequency, cognitive outcome, and video-EEG. Results: In the cohort, we identified 9 different heterozygous de novo KCNQ2 missense mutations in 11 of 84 patients (13%). Two of 6 missense mutations detected by gene panel were recurrent and present in patients of the cohort. Seizures at onset typically consisted of tonic posturing often associated with focal clonic jerking, and were accompanied by apnea with desaturation. One patient diagnosed by gene panel had seizure onset at the age of 5 months. Based on seizure frequency at onset and cognitive outcome, we delineated 3 clinical subgroups, expanding the spectrum of KCNQ2 encephalopathy to patients with moderate intellectual disability and/or infrequent seizures at onset. Recurrent mutations lead to relatively homogenous phenotypes. One patient responded favorably to retigabine; 5 patients had a good response to carbamazepine. In 6 patients, seizures with bradycardia were recorded. One patient died of probable sudden unexpected death in epilepsy. Conclusion: KCNQ2 mutations cause approximately 13% of unexplained NEE. Patients present with a wide spectrum of severity and, although rare, infantile epilepsy onset is possible. PMID:24107868

  15. Acetyl-L-carnitine in hepatic encephalopathy.

    PubMed

    Malaguarnera, Michele

    2013-06-01

    Hepatic encephalopathy is a common complication of hepatic cirrhosis. The clinical diagnosis is based on two concurrent types of symptoms: impaired mental status and impaired neuromotor function. Impaired mental status is characterized by deterioration in mental status with psychomotor dysfunction, impaired memory, and increased reaction time, sensory abnormalities, poor concentration, disorientation and coma. Impaired neuromotor function include hyperreflexia, rigidity, myoclonus and asterixis. The pathogenesis of hepatic encephalopathy has not been clearly defined. The general consensus is that elevated levels of ammonia and an inflammatory response work in synergy to cause astrocyte to swell and fluid to accumulate in the brain which is thought to explain the symptoms of hepatic encephalopathy. Acetyl-L-carnitine, the short-chain ester of carnitine is endogenously produced within mitochondria and peroxisomes and is involved in the transport of acetyl-moieties across the membranes of these organelles. Acetyl-L-carnitine administration has shown the recovery of neuropsychological activities related to attention/concentration, visual scanning and tracking, psychomotor speed and mental flexibility, language short-term memory, attention, and computing ability. In fact, Acetyl-L-carnitine induces ureagenesis leading to decreased blood and brain ammonia levels. Acetyl-L-carnitine treatment decreases the severity of mental and physical fatigue, depression cognitive impairment and improves health-related quality of life. The aim of this review was to provide an explanation on the possible toxic effects of ammonia in HE and evaluate the potential clinical benefits of ALC.

  16. Perinatal depression: implications for child mental health

    PubMed Central

    2010-01-01

    Perinatal depression is common and primary care holds a crucial role for detecting, treating or, if necessary, providing referrals to mental health care for affected women. Family doctors should be aware of risk factors for peripartum depression, including previous history of depression, life events and interpersonal conflict. Perinatal depression has been associated with many poor outcomes, including maternal, child and family unit challenges. Infants and young children of perinatally depressed mothers are more likely to have a difficult temperament, as well as cognitive and emotional delays. The primary care setting is uniquely poised to be the screening and treatment site for perinatal depression; however, several obstacles, both at patient and systems level, have been identified that interfere with women's treatment engagement. Current published treatment guidelines favour psychotherapy above medicines as first line treatment for mild to moderate perinatal depression, while pharmacotherapy is first choice for severe depression, often in combination with psychosocial or integrative approaches. Among mothers who decide to stop taking their antidepressants despite ongoing depression during the perinatal period, the majority suffer from relapsing symptoms. If depression continues post‐partum, there is an increased risk of poor mother–infant attachment, delayed cognitive and linguistic skills in the infant, impaired emotional development and risk for behavioural problems in later life. Complex, comprehensive and multilevel algorithms are warranted to treat perinatal depression. Primary care doctors are best suited to initiate, carry out and evaluate the effectiveness of such interventions designed to prevent adverse outcomes of maternal perinatal depression on mother and child wellbeing. PMID:22477948

  17. Fetal akinesia and multiple perinatal fractures.

    PubMed

    Chen, H; Blackburn, W R; Wertelecki, W

    1995-02-13

    Two newborn infants with fetal akinesia sequence were noted to have multiple perinatal fractures of the long bones. The radiographic manifestations are characterized by gracile ribs, thin long bones, and multiple diaphyseal fractures. Consistent histopathologic changes of bone are irregular with focal areas of extreme diaphyseal thinning, thin and long marrow spicules, and with or without callous formation at fracture sites. Pathogenic mechanisms of bone fractures in fetal akinesia sequence and the differential diagnoses of congenital/perinatal bone fractures are discussed.

  18. Genetic and perinatal effects of abused substances

    SciTech Connect

    Brande, M.C.; Zimmerman, A.M.

    1987-01-01

    This book provides an overview of the effects of several abused drugs, including opiates, cannabinoids, alcohol, nicotine, and cocaine, with special emphasis on the actions of these substances at the molecular and cellular levels. The first half deals with genetic effects, including molecular genetics, biochemical genetics, pharmacogenetics, cytogenetics, and genetic toxicity. The second half focuses on perinatal effects and covers: drug abuse during pregnancy; biochemical aspects of marihuana on male reproduction; and long-term behavioral and neuroendocrine effects of perinatal alcohol exposure.

  19. Posterior reversible encephalopathy syndrome in alcoholic hepatitis: Hepatic encephalopathy a common theme

    PubMed Central

    John, Elizabeth S; Sedhom, Ramy; Dalal, Ishita; Sharma, Ranita

    2017-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiologic diagnosis that has become more widely recognized and reported over the past few decades. As such, there are a number of known risk factors that contribute to the development of this syndrome, including volatile blood pressures, renal failure, cytotoxic drugs, autoimmune disorders, pre-eclampsia, and eclampsia. This report documents the first reported case of PRES in a patient with severe alcoholic hepatitis with hepatic encephalopathy and delves into a molecular pathophysiology of the syndrome. PMID:28127211

  20. Posterior reversible encephalopathy syndrome in alcoholic hepatitis: Hepatic encephalopathy a common theme.

    PubMed

    John, Elizabeth S; Sedhom, Ramy; Dalal, Ishita; Sharma, Ranita

    2017-01-14

    Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiologic diagnosis that has become more widely recognized and reported over the past few decades. As such, there are a number of known risk factors that contribute to the development of this syndrome, including volatile blood pressures, renal failure, cytotoxic drugs, autoimmune disorders, pre-eclampsia, and eclampsia. This report documents the first reported case of PRES in a patient with severe alcoholic hepatitis with hepatic encephalopathy and delves into a molecular pathophysiology of the syndrome.

  1. Naturalization of immigrants and perinatal mortality

    PubMed Central

    Englert, Yvon; Buekens, Pierre

    2013-01-01

    Background: Differences in neonatal mortality among immigrants have been documented in Belgium and elsewhere, and these disparities are poorly understood. Our objective was to compare perinatal mortality rates in immigrant mothers according to citizenship status. Methods: This was a population-based study using 2008 data from the Belgian birth register data pertaining to regions of Brussels and Wallonia. Odds ratio (OR) and 95% confidence intervals (95% CIs) for perinatal mortality according to naturalization status were calculated by logistic regression analyses adjusting for parents’ medical and social characteristics. Results: Four hundred and thirty-seven perinatal deaths were registered among 60 881 births (7.2‰). Perinatal mortality rate varied according to the origin of the mother and her naturalization status: among immigrants, non-naturalized immigrants had a higher incidence of perinatal mortality (10.3‰) than their naturalized counterparts (6.1‰) with an adjusted OR of 2.2, 95% CI (1.1–4.5). Conclusion: In a country with a high frequency of naturalization, and universal access to health care, naturalized immigrant mothers experience less perinatal mortality than their not naturalized counterparts. PMID:22490473

  2. Recent advances of perinatal medicine in China.

    PubMed

    Zhou, Z

    1995-05-01

    Perinatal medicine has been practiced for only 30 years. The basis for such medicine is perinatal health care and the main theme is systemic monitoring and management of high-risk pregnancies. China has offered such practice since 1979, with the perinatal health care system derived from the former health care system for pregnant women. The rate of maternal mortality in China had fallen to 94.7/100,000 by 1989, while the perinatal mortality rate was 51.8/1000 as of 1986. Comparable rates for 1993 in the Shanghai area were 19.95/100,000 and 10.6/1000, respectively. A group of Baby Friendly Hospitals was formally approved by the Ministry of Health and the WHO-UNICEF joint committee. In Shanghai, 11 such maternity hospitals received this status in 1993, and 27 more in 1994. Recently, the social model of perinatal health care, as proposed by WHO-EURO, has been adopted in Shanghai, providing the mother the rights and freedom to choose appropriate health care management on her own. It is gaining emphasis in Shanghai that both medical and social models are mandatory in perinatal health care.

  3. Assessment of phospholipid synthesis related biomarkers for perinatal asphyxia: a piglet study

    PubMed Central

    Sánchez-Illana, Ángel; Solberg, Rønnaug; Lliso, Isabel; Pankratov, Leonid; Quintás, Guillermo; Saugstad, Ola Didrik; Vento, Máximo; Kuligowski, Julia

    2017-01-01

    The prompt and reliable identification of infants at risk of hypoxic-ischemic encephalopathy secondary to perinatal asphyxia in the first critical hours is important for clinical decision-making and yet still remains a challenge. This work strives for the evaluation of a panel of metabolic biomarkers that have been associated with the hypoxic-ischemic insult in the perinatal period. Plasma and urine samples from a consolidated newborn piglet model of hypoxia and withdrawn before and at different time points after a hypoxic insult were analyzed and compared to a control group. Time-dependent metabolic biomarker profiles were studied and observed patterns were similar to those of lactate levels, which are currently considered the gold standard for assessing hypoxia. Class prediction performance could be improved by the use of a combination of the whole panel of determined metabolites in plasma as compared to lactate values. Using a multivariate model including lactate together with the studied metabolic biomarkers allowed to improve the prediction performance of duration of hypoxia time, which correlates with the degree of brain damage. The present study evidences the usefulness of choline and related metabolites for improving the early assessment of the severity of the hypoxic insult. PMID:28071721

  4. Effect of Phenobarbital on Nitric Oxide Level in Term Newborn Infants with Perinatal Asphyxia

    PubMed Central

    Khoshdel, Abolfazl; Noormohammadi, Hajar; Kheiri, Soleiman; Reisi, Roya; Nourbakhsh, Seyed Mohammad-Kazem; Panahandeh, Gholam Reza; Heidarian, Esfandiar

    2016-01-01

    Objectives Perinatal asphyxia (PA) is very significant in perinatal medicine due to the involvement of the central nervous system. This study was conducted to investigate the biochemical, clinical, and paraclinical changes associated with phenobarbital administration in neonates with PA. Methods In this prospective, case-control study, 30 neonates with PA in two groups of 15 each (case and control) were investigated. The case group received 20 mg/kg intravenous phenobarbital within six hours of birth, and the control group did not receive phenobarbital. Serum concentrations of nitric oxide (NO) were measured at enrollment and one week after birth in the two groups. Clinical, electroencephalography, and magnetic resonance imaging findings of the two groups were compared. Results At enrollment, the two groups did not differ in clinical severity, seizure incidence, or NO concentration. After one week, NO concentration was significantly lower in the case group (p < 0.050), but there was no significant difference in other variables between the two groups. Conclusions Early administration of phenobarbital in term neonates with PA could protect them against encephalopathy. PMID:27602186

  5. A clinically relevant model of perinatal global ischemic brain damage in rats.

    PubMed

    Yang, Ting; Zhuang, Lei; Terrando, Niccolò; Wu, Xinmin; Jonhson, Mark R; Maze, Mervyn; Ma, Daqing

    2011-04-06

    We have designed a clinically relevant model of perinatal asphyxia providing intrapartum hypoxia in rats. On gestation day 22 SD rats were anesthetized and the uterine horns were exteriorized and placed in a water bath at 37°C for up to 20min. After this, pups were delivered from the uterus and manually stimulated to initiate breathing in an incubator at 37°C for 1 h in air. Brains were harvested and stained with cresyl violet, caspase-3, and TUNEL to detect morphological and apoptotic changes on postnatal days (PND) 1, 3, and 7. Separate cohorts were maintained until PND 50 and tested for learning and memory using Morris water maze (WM). Survival rate was decreased with longer hypoxic time, and 100% mortality was noted when hypoxia time was beyond 18min. Apoptosis was increased with the duration of hypoxia with neuronal loss and cell shrinkage in the CA1 of hippocampus. The time taken for the juveniles to locate the hidden platform during WM was increased in animals subjected to hypoxia. These data demonstrate that perinatal ischemic injury leads to neuronal death in the hippocampus and long-lasting cognitive dysfunction. This model mimics hypoxic ischemic encephalopathy in humans and may be appropriate for investigating therapeutic interventions. Copyright © 2011 Elsevier B.V. All rights reserved.

  6. A founder CEP120 mutation in Jeune asphyxiating thoracic dystrophy expands the role of centriolar proteins in skeletal ciliopathies

    PubMed Central

    Shaheen, Ranad; Schmidts, Miriam; Faqeih, Eissa; Hashem, Amal; Lausch, Ekkehart; Holder, Isabel; Superti-Furga, Andrea; Mitchison, Hannah M.; Almoisheer, Agaadir; Alamro, Rana; Alshiddi, Tarfa; Alzahrani, Fatma; Beales, Philip L.; Alkuraya, Fowzan S.

    2015-01-01

    Jeune asphyxiating thoracic dystrophy (JATD) is a skeletal dysplasia characterized by a small thoracic cage and a range of skeletal and extra-skeletal anomalies. JATD is genetically heterogeneous with at least nine genes identified, all encoding ciliary proteins, hence the classification of JATD as a skeletal ciliopathy. Consistent with the observation that the heterogeneous molecular basis of JATD has not been fully determined yet, we have identified two consanguineous Saudi families segregating JATD who share a single identical ancestral homozygous haplotype among the affected members. Whole-exome sequencing revealed a single novel variant within the disease haplotype in CEP120, which encodes a core centriolar protein. Subsequent targeted sequencing of CEP120 in Saudi and European JATD cohorts identified two additional families with the same missense mutation. Combining the four families in linkage analysis confirmed a significant genome-wide linkage signal at the CEP120 locus. This missense change alters a highly conserved amino acid within CEP120 (p.Ala199Pro). In addition, we show marked reduction of cilia and abnormal number of centrioles in fibroblasts from one affected individual. Inhibition of the CEP120 ortholog in zebrafish produced pleiotropic phenotypes characteristic of cilia defects including abnormal body curvature, hydrocephalus, otolith defects and abnormal renal, head and craniofacial development. We also demonstrate that in CEP120 morphants, cilia are shortened in the neural tube and disorganized in the pronephros. These results are consistent with aberrant CEP120 being implicated in the pathogenesis of JATD and expand the role of centriolar proteins in skeletal ciliopathies. PMID:25361962

  7. Cerebral Hemodynamics in Asphyxiated Newborns Undergoing Hypothermia Therapy: Pilot Findings Using a Multiple-Time-Scale Analysis

    PubMed Central

    Chalak, Lina F; Tian, Fenghua; Tarumi, Takashi; Zhang, Rong

    2015-01-01

    Background Improved quantitative assessment of cerebral hemodynamics in newborns might enable us to optimize cerebral perfusion. Our objective was to develop an approach to assess cerebral hemodynamics across multiple time scales during the first 72 hours of life in newborns during hypothermia therapy. Methods Spontaneous oscillations in mean arterial pressure (MAP) and regional cerebral tissue oxygen saturation (SctO2) were analyzed using a moving window correlation (MWC) method with time scales ranging from 0.15 to 8 hours in this pilot methodology study. Abnormal neurodevelopmental outcome was defined by Bayley III scores and/or cerebral palsy by 24 months of age using receiver operating curve (ROC). Results Multiple-time-scale correlations between MAP and SctO2 oscillations were tested in 10 asphyxiated newborns undergoing hypothermia therapy. Large non induced fluctuations in the blood pressure were observed during cooling in all five infants with abnormal outcomes. Notably, these infants had two distinct patterns of correlation: a positive in-phase correlation at the short time scales (15 min), and/or a negative anti-phase correlations observed at long time scales (4 hrs.). Both the in-phase (AUC 0.6, [95% CI 0.2–0.95]) and anti-phase correlations (AUC 0.75, [95% CI 0.4–0.95]) appeared to be related to an abnormal outcome. Conclusions Our observations suggest that the time scale is an important factor that needs to be standardized in the assessment of neonatal cerebral hemodynamics. PMID:26858217

  8. Three cases of suprachoroidal hemorrhage associated with chest compression or asphyxiation and detected using postmortem computed tomography.

    PubMed

    Oshima, Toru; Yoshikawa, Hiroshi; Ohtani, Maki; Mimasaka, Sohtaro

    2015-05-01

    We report 3 cases of suprachoroidal hemorrhage (SCH) found to be triggered by increased intrathoracic pressure and detected using postmortem computed tomography (PMCT). Case 1 was a man aged in his 50s who was found dead at a landslide site. The autopsy showed clogging of the upper respiratory tract with soil debris from the landslide. The cause of death was determined to be asphyxia. PMCT showed SCH in both eyes, which was believed to be caused by chest compression or choking on the soil debris from the landslide. Case 2 was a woman aged in her 60s who was found dead in the sea. The autopsy revealed injuries primarily to her chest. We concluded that the cause of death was drowning. PMCT showed SCH in her right eye that was believed to be caused by chest compression. Case 3 was a woman aged in her 80s who was buried in a snowdrift and potentially died from hypothermia. PMCT showed SCH in both eyes, which was considered to be from an increase in intrathoracic pressure that might have been caused by the burial in the snow. Histological findings showed serous retinal detachment associated with retinal pigment epithelium damage due to SCH, which indicated that she was alive for several hours after the onset of SCH. The increase in intrathoracic pressure caused by dyspnea or chest compression was considered responsible for the onset of SCH in all of the present cases. PMCT might assist with the differential diagnosis of traumatic asphyxiation by SCH. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  9. Prevention of perinatal HIV transmission: the Perinatal HIV Hotline perspective.

    PubMed

    Waldura, Jess Fogler

    2011-01-01

    Among the most frequently asked questions by callers to the National Perinatal HIV Hotline are those on the use of hormonal contraception in women receiving antiretroviral therapy. Estradiol levels are reduced by ritonavir-boosted protease inhibitors (PIs), nelfinavir, and nevirapine and increased by non-ritonavir-boosted PIs (except nelfinavir), efavirenz, and etravirine. Oral contraceptives do not affect antiretroviral drug levels, and several options are available for hormonal contraception that can compensate for or avoid the effects of antiretroviral drugs on estrogen levels. Other common questions on the hotline involve interpretation and management issues that arise from indeterminate Western blot test results early and late in pregnancy and from positive rapid test results during labor. Many questions focus on appropriate selection of antiretroviral drugs in pregnancy and the need to change regimens to reduce risk of birth defects in the child. This articlesummarizes a presentation by Jess Fogler Waldura, MD, at the 13th Annual Clinical Conference for the Ryan White HIV/AIDS Program held in August 2010 in Washington, DC.

  10. A quantitative analysis of optimal treatment capacity for perinatal asphyxia.

    PubMed

    Geva, Alon; Gray, James

    2012-01-01

    In centers electing to offer therapeutic hypothermia for treating hypoxic-ischemic encephalopathy (HIE), determining the optimal number of cooling devices is not straightforward. The authors used computer-based modeling to determine the level of service as a function of local HIE caseload and number of cooling devices available. The authors used discrete event simulation to create a model that varied the number of HIE cases and number of cooling devices available. Outcomes of interest were percentage of HIE-affected infants not cooled, number of infants not cooled, and percentage of time that all cooling devices were in use. With 1 cooling device, even the smallest perinatal center did not achieve a cooling rate of 99% of eligible infants. In contrast, 2 devices ensured 99% service in centers treating as many as 20 infants annually. In centers averaging no more than 1 HIE infant monthly, the addition of a third cooling device did not result in a substantial reduction in the number of infants who would not be cooled. Centers electing to offer therapeutic hypothermia with only a single cooling device are at significant risk of being unable to provide treatment to eligible infants, whereas 2 devices appear to suffice for most institutions treating as many as 20 annual HIE cases. Three devices would rarely be needed given current caseloads seen at individual institutions. The quantitative nature of this analysis allows decision makers to determine the number of devices necessary to ensure adequate availability of therapeutic hypothermia given the HIE caseload of a particular institution.

  11. Oxygen and oxidative stress in the perinatal period.

    PubMed

    Torres-Cuevas, Isabel; Parra-Llorca, Anna; Sánchez-Illana, Angel; Nuñez-Ramiro, Antonio; Kuligowski, Julia; Cháfer-Pericás, Consuelo; Cernada, María; Escobar, Justo; Vento, Máximo

    2017-08-01

    Fetal life evolves in a hypoxic environment. Changes in the oxygen content in utero caused by conditions such as pre-eclampsia or type I diabetes or by oxygen supplementation to the mother lead to increased free radical production and correlate with perinatal outcomes. In the fetal-to-neonatal transition asphyxia is characterized by intermittent periods of hypoxia ischemia that may evolve to hypoxic ischemic encephalopathy associated with neurocognitive, motor, and neurosensorial impairment. Free radicals generated upon reoxygenation may notably increase brain damage. Hence, clinical trials have shown that the use of 100% oxygen given with positive pressure in the airways of the newborn infant during resuscitation causes more oxidative stress than using air, and increases mortality. Preterm infants are endowed with an immature lung and antioxidant system. Clinical stabilization of preterm infants after birth frequently requires positive pressure ventilation with a gas admixture that contains oxygen to achieve a normal heart rate and arterial oxygen saturation. In randomized controlled trials the use high oxygen concentrations (90% to 100%) has caused more oxidative stress and clinical complications that the use of lower oxygen concentrations (30-60%). A correlation between the amount of oxygen received during resuscitation and the level of biomarkers of oxidative stress and clinical outcomes was established. Thus, based on clinical outcomes and analytical results of oxidative stress biomarkers relevant changes were introduced in the resuscitation policies. However, it should be underscored that analysis of oxidative stress biomarkers in biofluids has only been used in experimental and clinical research but not in clinical routine. The complexity of the technical procedures, lack of automation, and cost of these determinations have hindered the routine use of biomarkers in the clinical setting. Overcoming these technical and economical difficulties constitutes a

  12. [Maternal mortality and perinatal mortality].

    PubMed

    Boutaleb, Y; Mesbahi, M; Lahlou, D; Aderdour, M

    1982-01-01

    94 maternal deaths and 1546 fetal and neonatal deaths were registered among 28,706 births at the CHU Averroes in Casablanca between 1978-80. 45% of women who deliver at the clinic are very poor and only 10% are relatively well off. Obstetrical antecedents were noted in 27% of the fetal deaths. 70% of the maternal deaths occurred in women aged 20-34. 32 maternal deaths occurred among 16,232 women with 1-2 children, 30 among 6514 women with 3-5 children, and 32 among 5960 women with 6-14 children. 11,027 of the 28,706 were primaparas. Perinatal mortality was 4.46% among primaparas, 8.24% among grand multiparas, and 4.1% among secondiparas. In 58 of the 94 cases of maternal mortality the woman was hospitalized after attempting delivery at home or in a village clinic. Among women with 1 or 2 children, hemorrhage was the cause of death in 8 cases, infection in 7 cases, eclampsia in 3 cases, thromboembolism in 2 cases, uterine inversion in 2 cases, pulmonary tuberculosis in 1 case, embolism in 5 cases, and other causes 1 case each. Among women with 3-5 children hemorrhage was the cause of death in 10 cases, septicemia in 3 cases, uterine rupture in 3 cases, eclampsia in 3 cases, uterine inversion in 2 cases, viral hepatitis in 2 cases, emboli in 2 cases, and other reasons 1 case each. Among grand multiparas hemorrhage was the cause of death in 11 cases, uterine rupture in 12 cases, peritonitis in 2 cases, eclampsia in 2 cases, emboli in 2 cases, and other causes 1 case each. 19 of the maternal deaths were judged to have been avoidable with better management. Prematurity and birth weight of 1000-2500 g associated or not with other pathology were found in 714 of 1546 perinatal deaths. Of 390 cases of death in utero with retention and maceration, 68 were caused by reno-vascular syndromes, 76 by maternal infections, 33 by maternal syphilis, 26 by fetal malformation, 18 by maternal diabetes, 10 by Rh incompatability, and 159 by indeterminate causes. In 795 cases of

  13. Del(12)(p11.21p12.2) associated with an asphyxiating thoracic dystrophy or chondroectodermal dysplasia-like syndrome

    SciTech Connect

    Nagai, T.; Kato, R.; Hasegawa, T.

    1995-01-02

    We describe a 5-year-old Japanese boy who has some radiographic findings characteristic of asphyxiating thoracic dystrophy (ATD)-chondroectodermal dysplasia with a de novo chromosome abnormality. He also has mild mental retardation, short stature, hypoplastic hair and skin, oligodontia, small thoracic cage, hypoplastic pelvis and cone-shaped epiphyses of hands. On cytogenetic studies he was found to have a de novo del(12)(p11.21p12.2). These results suggest that the locus of the gene associated with ATD-chondroectodermal dysplasia may be situated at 12p11.21p12.2. 11 refs., 2 figs.

  14. Prenatal ultrasound and MRI Diagnosis of Jeune syndrome type I (asphyxiating thoracic dystrophy) with histology and post-mortem three-dimensional CT confirmation.

    PubMed

    Tonni, Gabriele; Panteghini, Marco; Bonasoni, Mariapaola; Pattacini, Pierpaolo; Ventura, Alessandro

    2013-04-01

    Asphyxiating thoracic dystrophy (ATD) also known as Jeune syndrome is a rare autosomal recessive multisystem disorder with an incidence estimated in 1:100.000-130.000 live births. Associated findings may include hepatic fibrosis and renal cysts. A prenatal ultrasound and MRI diagnosis performed in the early second-trimester of pregnancy is reported together with DNA analysis. Post-mortem diagnostic investigations such as radiograph and three-dimensional CT scan and histology have been useful in the final diagnosis of this rare skeletal dysplasia.

  15. Intrapartum fever and the risk for perinatal complications - the effect of fever duration and positive cultures.

    PubMed

    Ashwal, Eran; Salman, Lina; Tzur, Yossi; Aviram, Amir; Ben-Mayor Bashi, Tali; Yogev, Yariv; Hiersch, Liran

    2017-04-24

    To estimate the association between intrapartum fever and adverse perinatal outcome. A retrospective cohort study of women attempting vaginal delivery at term in a tertiary hospital (2012-2015). Perinatal outcome of deliveries complicated by intrapartum fever (≥38.0 °C) were compared to women with no intrapartum fever matched by parity and gestational age at delivery in a 1:2 ratio. Maternal outcome included cesarean section (CS), operative vaginal delivery (OVD), retained placenta or post-partum hemorrhage. Neonatal outcome included 5-minute Apgar score <7, umbilical artery pH <7.1, meconium aspiration syndrome, need for mechanical ventilation or hypoxic ischemic encephalopathy. Overall, 309 women had intrapartum fever and 618 served as controls. Women with intrapartum fever had higher rates of OVD (34.3 versus 19.6%, p < .001) and CS (20.7 versus 8.7%, p < .001). In multivariate analysis, intrapartum fever was independently associated with adverse maternal (3.75, 95%CI 2.65-5.30, p < .001) and neonatal outcome (3.39, 95%CI 1.78-6.45, p < .001). In febrile women, fever duration was related to maternal complications, specifically to CS. In addition, maternal bacteremia and positive placental cultures were risk factors for neonatal complications compared to those with negative cultures (23.3 versus 9.8%, p = .01). Intrapartum fever was associated with adverse perinatal complications. The duration of intrapartum fever, maternal bacteremia, and positive cultures further increase this risk.

  16. Probiotics for people with hepatic encephalopathy.

    PubMed

    Dalal, Rohan; McGee, Richard G; Riordan, Stephen M; Webster, Angela C

    2017-02-23

    Hepatic encephalopathy is a disorder of brain function as a result of liver failure or portosystemic shunt or both. Both hepatic encephalopathy (clinically overt) and minimal hepatic encephalopathy (not clinically overt) significantly impair patient's quality of life and daily functioning, and represent a significant burden on healthcare resources. Probiotics are live micro-organisms, which when administered in adequate amounts, may confer a health benefit on the host. To determine the beneficial and harmful effects of probiotics in any dosage, compared with placebo or no intervention, or with any other treatment for people with any grade of acute or chronic hepatic encephalopathy. This review did not consider the primary prophylaxis of hepatic encephalopathy. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, MEDLINE, Embase, Science Citation Index Expanded, conference proceedings, reference lists of included trials, and the World Health Organization International Clinical Trials Registry Platform until June 2016. We included randomised clinical trials that compared probiotics in any dosage with placebo or no intervention, or with any other treatment in people with hepatic encephalopathy. We used standard methodological procedures expected by The Cochrane Collaboration. We conducted random-effects model meta-analysis due to obvious heterogeneity of participants and interventions. We defined a P value of 0.05 or less as significant. We expressed dichotomous outcomes as risk ratio (RR) and continuous outcomes as mean difference (MD) with 95% confidence intervals (CI). We included 21 trials with 1420 participants, of these, 14 were new trials. Fourteen trials compared a probiotic with placebo or no treatment, and seven trials compared a probiotic with lactulose. The trials used a variety of probiotics; the most commonly used group of probiotic was VSL#3, a proprietary name for a group of eight probiotics. Duration of administration

  17. The Spectrum of Disease in Chronic Traumatic Encephalopathy

    ERIC Educational Resources Information Center

    McKee, Ann C.; Stein, Thor D.; Nowinski, Christopher J.; Stern, Robert A.; Daneshvar, Daniel H.; Alvarez, Victor E.; Lee, Hyo-Soon; Hall, Garth; Wojtowicz, Sydney M.; Baugh, Christine M.; Riley, David O.; Kubilus, Caroline A.; Cormier, Kerry A.; Jacobs, Matthew A.; Martin, Brett R.; Abraham, Carmela R.; Ikezu, Tsuneya; Reichard, Robert Ross; Wolozin, Benjamin L.; Budson, Andrew E.; Goldstein, Lee E.; Kowall, Neil W.; Cantu, Robert C.

    2013-01-01

    Chronic traumatic encephalopathy is a progressive tauopathy that occurs as a consequence of repetitive mild traumatic brain injury. We analysed post-mortem brains obtained from a cohort of 85 subjects with histories of repetitive mild traumatic brain injury and found evidence of chronic traumatic encephalopathy in 68 subjects: all males, ranging…

  18. Early Recognition of Chronic Traumatic Encephalopathy through FDDNP PET Imaging

    DTIC Science & Technology

    2014-10-01

    head injuries sustained in battle have been associated with the development of chronic traumatic encephalopathy (CTE). Pathological series have...Keywords:Traumatic Brain Injury, Chronic Traumatic Encephalopathy ,PET imaging, Tau Overall Project Summary:Preparation for enrollment...AD_________________ Award Number: W81XWH-13-1-0486 TITLE: Early Recognition of Chronic Traumatic

  19. Comparison of Transmissible Mink Encephalopathy Isolates in Raccoons

    USDA-ARS?s Scientific Manuscript database

    Owing to its susceptibility to various transmissible spongiform encephalopathies (TSE) and relatively short incubation times, the raccoon (Procyon lotor) has been suggested as a model for TSE strain differentiation. Transmissible mink encephalopathy (TME) is a prion disease of undetermined origin in...

  20. Evaluation of the zoonotic potential of transmissible mink encephalopathy

    USDA-ARS?s Scientific Manuscript database

    Successful transmission of Transmissible Mink Encephalopathy (TME) to cattle supports the bovine hypothesis to the still controversial origin of TME outbreaks. Human and primate susceptibility to classical Bovine Spongiform Encephalopathy (c-BSE) and the transmissibility of L-type BSE to macaques as...

  1. The Spectrum of Disease in Chronic Traumatic Encephalopathy

    ERIC Educational Resources Information Center

    McKee, Ann C.; Stein, Thor D.; Nowinski, Christopher J.; Stern, Robert A.; Daneshvar, Daniel H.; Alvarez, Victor E.; Lee, Hyo-Soon; Hall, Garth; Wojtowicz, Sydney M.; Baugh, Christine M.; Riley, David O.; Kubilus, Caroline A.; Cormier, Kerry A.; Jacobs, Matthew A.; Martin, Brett R.; Abraham, Carmela R.; Ikezu, Tsuneya; Reichard, Robert Ross; Wolozin, Benjamin L.; Budson, Andrew E.; Goldstein, Lee E.; Kowall, Neil W.; Cantu, Robert C.

    2013-01-01

    Chronic traumatic encephalopathy is a progressive tauopathy that occurs as a consequence of repetitive mild traumatic brain injury. We analysed post-mortem brains obtained from a cohort of 85 subjects with histories of repetitive mild traumatic brain injury and found evidence of chronic traumatic encephalopathy in 68 subjects: all males, ranging…

  2. Typical and atypical cases of bovine spongiform encephalopathy

    USDA-ARS?s Scientific Manuscript database

    Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the United Kingdom and subsequently in other countries. It is the most likely cause of variant Creutzfeldt-Jakob disease (vCJD) in humans, but the origin of BSE has not been eluci...

  3. Maternal nutrition and perinatal outcomes.

    PubMed

    Barger, Mary K

    2010-01-01

    Diet and patterns of eating during pregnancy can affect perinatal outcomes through direct physiologic effects or by stressing the fetus in ways that permanently affect phenotype. Supplements are not a magic nutritional remedy, and evidence of profound benefit for most supplements remains inconclusive. However, research supports calcium supplements to decrease preeclampsia. Following a low glycemic, Mediterranean-type diet appears to improve ovulatory infertility, decrease preterm birth, and decrease the risk of gestational diabetes. Although women in the United States have adequate levels of most nutrients, subpopulations are low in vitamin D, folate, and iodine. Vitamin D has increasingly been shown to be important not only for bone health, but also for glucose regulation, immune function, and good uterine contractility in labor. To ensure adequate vitamin and micronutrient intake, especially of folate before conception, all reproductive age women should take a multivitamin daily. In pregnancy, health care providers need to assess women's diets, give them weight gain recommendations based on their body mass index measurement, and advise them to eat a Mediterranean diet rich in omega-3 fatty acids (ingested as low-mercury risk fatty fish or supplements), ingest adequate calcium, and achieve adequate vitamin D levels through sun exposure or supplements. Health care providers should continue to spend time on nutrition assessment and counseling.

  4. Fourth goal of perinatal medicine.

    PubMed Central

    Ounsted, C; Roberts, J C; Gordon, M; Milligan, B

    1982-01-01

    Reduction in maternal mortality, infant mortality, and infant morbidity have been successively the goals of perinatal medicine. The fourth is to reduce bonding failure. In July 1978 a preventive service was started in the John Radcliffe Maternity Hospital. A twice-weekly round is made. Midwives refer families who cause them concern. In the first year the referral rate ws 20.5 per 1000 liveborn babies. The referred sample differed from the hospital population in terms of maternal psychiatric history, marital state and babies' admission to special care. The main reasons for referral were: doubt about parenting ability (27%), psychiatric history (15%), disturbed behaviour in hospital (14%), and diffuse social and medical problems (17%). Long-term care was needed for only 14% of families. At their first birthdays, six babies were placed away from their natural parents; the sample had had a slightly higher than expected admission rate to hospital; the distribution of weights did not differ from the expected; doctors and health visitors were still concerned about one-quarter of the families. Seven cases of screening failure were found among those not referred to our service, but only one was seriously abused. No child referred in the first year has been seriously neglected or abused. PMID:6802338

  5. Hurricane Katrina and perinatal health.

    PubMed

    Harville, Emily W; Xiong, Xu; Buekens, Pierre

    2009-12-01

    We review the literature on the effects of Hurricane Katrina on perinatal health, and providing data from our own research on pregnant and postpartum women. After Katrina, obstetric, prenatal, and neonatal care was compromised in the short term, but increases in adverse birth outcomes such as preterm birth, low birthweight, and maternal complications were mostly limited to highly exposed women. Both pregnant and postpartum women had rates of post-traumatic stress disorder similar to, or lower than, others exposed to Katrina, and rates of depression similar to other pregnant and postpartum populations. Health behaviors, such as smoking and breastfeeding, may have been somewhat negatively affected by the disaster, whereas effects on nutrition were likely associated with limited time, money, and food choices, and indicated by both weight gain and loss. We conclude that, with a few specific exceptions, postdisaster concerns and health outcomes for pregnant and postpartum women were similar to those of other people exposed to Hurricane Katrina. In such situations, disaster planners and researchers should focus on providing care and support for the normal concerns of the peripartum period, such as breastfeeding, depression, and smoking cessation. Contraception needs to be available for those who do not want to become pregnant. Although additional physical and mental health care needs to be provided for the most severely exposed women and their babies, many women are capable of surviving and thriving in postdisaster environments.

  6. [Analysis of risk factors for adverse perinatal outcomes in women with pancytopenia].

    PubMed

    Wang, Xueyan; Zhang, Chao; Liang, Meiying; Zhang, Xiaohong; Wang, Jianliu; Zhang, Xia

    2015-12-01

    To explore the pregnancy outcomes of women with pancytopenia and the risk factors for the adverse perinatal outcomes. A total of 106 pregnant women with pancytopenia were admitted to Peking University People's Hospital from Jan. 2005 to Sep. 2014. The clinical data and the pregnancy outcomes were reviewed retrospectively to investigate the risk factors for the adverse perinatal outcomes. (1 ) Eighty-four patients were found pancytopenia before pregnancy while 22 were found for the first time during pregnancy. Sixty-four patients were diagnosed as aplastic anemia; 30 as myelodysplastic syndrome; 2 as paroxysmal nocturnal hemoglobinuria; 4 as hypersplenism, and 1 as anti-phospholipid syndrome. Diagnoses of the remaining 5 patients were uncertain. (2) Sixty-nine patients received at least one time blood transfusion before delivery. (3) As for the complications, nine women developed gestational diabetes; twenty-two suffered severe preeclampsia (SPE); two were diagnosed as anemic heart disease and three experienced respiratory tract infection. The postpartum blood loss ranged from 50 ml to 3 800 ml, with the median of 400 ml. And six women had the blood loss more than 1 000 ml. The gestational age at delivery ranged from 24 weeks to 40 weeks, with the median of 37.0 weeks. (4) Thirty-one patients suffered adverse perinatal outcomes, including 3 cases of intrauterine death, 4 therapeutic labor induction before 28 gestational weeks, 6 premature delivery before 34 weeks. There were 2 neonates complicated with intracranial hemorrhage, 2 with neonatal respiratory distress syndrome, 3 with hypoxic-ischemic encephalopathy, 2 with severe asphyxia and death, and 14 with small for gestational age. Among the patients with adverse perinatal outcomes, 26 women received blood transfusion during pregnancy and 17 developed SPE. The maximum and the minimum value of their white cell count (WBC), hemoglobin concentration (Hb) and blood platelet count (BPC) were (4.9±1.4)×10(9)/L, (2.9

  7. [Assessment of cochlea activity in a group of newborns with central nervous system impairment as an effect of perinatal asphyxia using click-evoked otoacoustic emissions (CEOAEs)].

    PubMed

    Widziszowska, Agnieszka; Namysłowski, Grzegorz; Genge, Anna; Buczyńska, Grazyna; Hajduk, Agata; Godula-Stuglik, Urszula

    2005-09-01

    During adaptation of newborn to extra-uterine environment dramatic changes in functioning in the inner organs and the entire human body is observed. Complicated or premature birth as well as complicated course of a perinatal period may cause reversible or irreversible damage of various tissues, organs or systems, and, consequently, their dysfunction. The most often changes in the central nervous system (CNS) in newborns occurring as an effect of perinatal asphyxia are found to be ischemic-hypoxic encephalopathy (IHE), periventricular leucomalacia (PVL) and intraventricular hemorrhages (IVH). Chronic hypoxia is considered to affect the hearing organ in newborns. Reversible or irreversible changes within the cochlea, brainstem or cortex may result in perceptive hearing losses. The aim of the study was to carry out the objective assessment of the cochlea activity using CEOAEs in neonates with CNS impairment occurring following perinatal asphyxia. To the investigation 16 newborns with IHE, PVL or IVH were included. The control group encompassed the health newborns matched as to the age. Perinatal anamnesis, general pediatric status, results of trans-fontanel ultrasonography and biochemical test results were taken into account in statistical analyses. In all newborns otoscopic examination and CEOAEs after birth and 3 months later were performed. CEOAEs in session 1 were significantly reduced in the investigated group comparing to control babies but 3 months later no differences between groups were observed. The outer hair cells activity in first days of life is reduced in newborns with asphyxia in anamnesis but with time no differences in cochlea development are observed.

  8. The Expanding Clinical Spectrum of Genetic Pediatric Epileptic Encephalopathies.

    PubMed

    Shbarou, Rolla; Mikati, Mohamad A

    2016-05-01

    Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various encephalopathies that are reviewed in this article include the following: early infantile epileptic encephalopathy or Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, severe myoclonic epilepsy in infancy (Dravet syndrome), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epileptic encephalopathy with continuous spike-and-wave during sleep. Their clinical features, prognosis as well as underlying genetic etiologies are presented and updated. Copyright © 2016. Published by Elsevier Inc.

  9. Hemophagocytic lymphohistiocytosis: A rare cause of recurrent encephalopathy

    PubMed Central

    Sulaiman, Raashda Ainuddin; Shaheen, Marwan Yassin; Al-Zaidan, Hamad; Al-Hassnan, Zuhair; Al-Sayed, Moeenaldeen; Rahbeeni, Zuhair; Bakshi, Nasir Ahmed; Kaya, Namik; Aldosary, Mazhor; Al-Owain, Mohammed

    2016-01-01

    Summary We report an unusual case of recurrent encephalopathy due to acquired hemophagocytic lymphohistiocytosis (HLH) in a patient with propionic acidemia (PA). PA is an inherited metabolic disorder in which patients often present with encephalopathy and pancytopenia during metabolic decompensation. However, these patients may rarely develop HLH with similar presentation. This case illustrates the need to distinguish HLH induced encephalopathy from the one secondary to metabolic decompensation in these patients, as early diagnosis and treatment of HLH improves prognosis. This case also highlights the importance of considering HLH in patients presenting with unexplained encephalopathy, as early diagnosis and treatment is lifesaving in this otherwise lethal condition. To our knowledge this is the first case report of acquired HLH presenting as recurrent encephalopathy followed by complete recovery, in a metabolically stable patient with PA. PMID:27672548

  10. Clinical Characteristics of Transplant-associated Encephalopathy in Children

    PubMed Central

    2017-01-01

    We aimed to analyze characteristics of encephalopathy after both hematopoietic stem cell and solid organ pediatric transplantation. We retrospectively reviewed medical records of 662 pediatric transplant recipients (201 with liver transplantation [LT], 55 with heart transplantation [HT], and 67 with kidney transplantation [KT], 339 with allogeneic hematopoietic stem cell transplantation [HSCT]) who received their graft organs at Asan Medical Center between January 2000 and July 2014. Of the 662 patients, 50 (7.6%) experienced encephalopathy after transplantation. The incidence of encephalopathy was significantly different according to the type of organ transplant: LT, 16/201 (8.0%), HT, 13/55 (23.6%), KT, 5/67 (7.5%), and HSCT, 16/339 (4.7%) (P < 0.001). Drug-induced encephalopathy (n = 14) was the most common encephalopathy for all transplant types, but particularly after HSCT. Hypertensive encephalopathy was the most common after KT and HT, whereas metabolic encephalopathy was the most common after LT. The median time to encephalopathy onset also differed according to the transplant type: 5 days after KT (range 0–491 days), 10 days after HT (1–296 days), 49.5 days after HSCT (9–1,405 days), and 39 days after LT (1–1,092 days) (P = 0.018). The mortality rate among patients with encephalopathy was 42.0% (n = 21/50). Only 5 patients died of neurologic complications. Transplant-associated encephalopathy presented different characteristics according to the type of transplant. Specialized diagnostic approach for neurologic complications specific to the type of transplant may improve survival and quality of life in children after transplantation. PMID:28145649

  11. Clinical Characteristics of Transplant-associated Encephalopathy in Children.

    PubMed

    Lee, Yun Jeong; Yum, Mi Sun; Kim, Eun Hee; Kim, Min Jee; Kim, Kyung Mo; Im, Ho Joon; Kim, Young Hwue; Park, Young Seo; Ko, Tae Sung

    2017-03-01

    We aimed to analyze characteristics of encephalopathy after both hematopoietic stem cell and solid organ pediatric transplantation. We retrospectively reviewed medical records of 662 pediatric transplant recipients (201 with liver transplantation [LT], 55 with heart transplantation [HT], and 67 with kidney transplantation [KT], 339 with allogeneic hematopoietic stem cell transplantation [HSCT]) who received their graft organs at Asan Medical Center between January 2000 and July 2014. Of the 662 patients, 50 (7.6%) experienced encephalopathy after transplantation. The incidence of encephalopathy was significantly different according to the type of organ transplant: LT, 16/201 (8.0%), HT, 13/55 (23.6%), KT, 5/67 (7.5%), and HSCT, 16/339 (4.7%) (P < 0.001). Drug-induced encephalopathy (n = 14) was the most common encephalopathy for all transplant types, but particularly after HSCT. Hypertensive encephalopathy was the most common after KT and HT, whereas metabolic encephalopathy was the most common after LT. The median time to encephalopathy onset also differed according to the transplant type: 5 days after KT (range 0-491 days), 10 days after HT (1-296 days), 49.5 days after HSCT (9-1,405 days), and 39 days after LT (1-1,092 days) (P = 0.018). The mortality rate among patients with encephalopathy was 42.0% (n = 21/50). Only 5 patients died of neurologic complications. Transplant-associated encephalopathy presented different characteristics according to the type of transplant. Specialized diagnostic approach for neurologic complications specific to the type of transplant may improve survival and quality of life in children after transplantation.

  12. Ifosfamide associated myoclonus-encephalopathy syndrome.

    PubMed

    Savica, Rodolfo; Rabinstein, Alejandro A; Josephs, Keith A

    2011-09-01

    The aim of this study was to investigate the presence of movement disorders associated with ifosfamide toxicity. One of the most common adverse events of ifosfamide treatment is central nervous system toxicity. However, little is known about the occurrence of movement disorders associated with ifosfamide toxicity. We performed a retrospective computer search of the electronic medical records database of the Mayo Clinic, Rochester, MN from 1 January 1997-30 June 2010, using a series of search terms to identify all patients that had been treated with ifosfamide for systemic cancer. Among 400 patients that have ever used ifosfamide, we selected those patients that had any neurological complication in their medical records after the use of ifosfamide. Fifty-two had a neurological complication after ifosfamide administration. The most common neurological complication was encephalopathy that was present in 11 cases (21%). The presence of a movement disorder time locked to the administration of ifosfamide was reported in seven cases (13%). Generalized myoclonus was most common, occurring in four patients while postural tremor was documented in the other three. All patients with myoclonus had asterixis. Four of the patients also had encephalopathy. In six patients the movement disorders resolved within 48 h, spontaneously, after the discontinuation of ifosfamide, while in one case resolved in 24 h after the treatment with methylene blue. Our study demonstrates that although encephalopathy is the most common adverse neurological event associated with ifosfamide toxicity, movement disorders, including generalized myoclonus, asterixis, and postural tremors may also occur. Treatment with methylene blue may be further considered as useful to ameliorate the movement disorders.

  13. Perinatal Practices & Traditions Among Asian Indian Women.

    PubMed

    Goyal, Deepika

    2016-01-01

    As the population in the United States grows more diverse, nurses caring for childbearing women must be aware of the many cultural traditions and customs unique to their patients. This knowledge and insight supports women and their families with the appropriate care, information, and resources. A supportive relationship builds trust, offers guidance, and allows for the new family to integrate information from nurses and other healthcare providers with the practice of certain perinatal cultural traditions. The Asian Indian culture is rich in tradition, specifically during the perinatal period. To support the cultural beliefs and practices of Asian Indian women during this time, nurses need to be aware of and consider multiple factors. Many women are navigating the new role of motherhood while making sense of and incorporating important cultural rituals. The purpose of this article is to provide an overview of perinatal cultural practices and traditions specific to the Asian Indian culture that perinatal nurses may observe in the clinical setting. Cultural traditions and practices specific to the pregnancy and postpartum period are described together with symbolism and implications for nursing practice. It is important to note that information regarding perinatal customs is provided in an effort to promote culturally sensitive nursing care and may not pertain to all Asian Indian women living in the United States.

  14. Inequalities in perinatal and maternal health.

    PubMed

    de Graaf, Johanna P; Steegers, Eric A P; Bonsel, Gouke J

    2013-04-01

    To describe inequalities in perinatal and maternal mortality, and morbidity from an international high-income country perspective. Measures of inequalities are socioeconomic status, ethnic background, and living area. Despite decreasing overall perinatal and maternal mortality in high-income countries, perinatal and maternal health inequalities persist. Inequalities in fetal, neonatal, and maternal adverse outcome relate to specific groups of risk factors. They commonly have a background in so-called structural risk factors, that is low level of education and income, being a migrant and living in disadvantaged areas. Structural risk factors therefore drive inequalities, and simultaneously represent the common perspective to judge perinatal and maternal health gaps. The effect of risk factors is further magnified in urban areas through risk accumulation.As mother and child share their background, neonatal, and maternal adverse health outcome patterns coincide, resulting in similar inequalities and similar epidemiological trends. The structural background explains the difficulty of improving this. Inequalities in perinatal and maternal outcome persist in women from lower socioeconomic groups, from specific ethnic groups, and from those living in deprived areas. In view of the lifelong consequences, these marked social disparities pose an important challenge for the political decision makers and the healthcare system.

  15. Caring for families coping with perinatal loss.

    PubMed

    Roehrs, Carol; Masterson, Anne; Alles, Ruth; Witt, Catherine; Rutt, Phillis

    2008-01-01

    To describe support needs and comfort level of labor nurses caring for families experiencing perinatal loss. Qualitative descriptive study. A western hospital birthing unit. Ten labor nurses. Participants completed online surveys and follow-up interviews; data saturation was reached. Content analysis produced themes and recommendations related to providing perinatal bereavement care. Participants reviewed and confirmed accuracy of the results. Nurses are generally comfortable but find it difficult to provide perinatal bereavement care. Strategies for coping include focusing on needed care, talking to nursing peers, and spending time with their own family members. Nurses take turns providing care depending on "who is best able to handle it that day" and prefer not to be assigned a laboring patient in addition to the grieving parents. Developing clinical expertise is necessary to gain the comfort level and the skills necessary to care for these vulnerable families. Orientation experiences and nursing staff debriefing would help. Needed education includes grief training, communication techniques, and guidelines for the extensive paperwork. Initial and ongoing education of nurses about perinatal bereavement care is needed. Effective strategies for coping during and after providing care would support nurses in meeting the emotional challenge of providing high quality perinatal bereavement care.

  16. Chronic Traumatic Encephalopathy and Movement Disorders: Update.

    PubMed

    Tarazi, Apameh; Tator, Charles H; Tartaglia, Maria Carmela

    2016-05-01

    Association of repetitive brain trauma with progressive neurological deterioration has been described since the 1920s. Punch drunk syndrome and dementia pugilistica (DP) were introduced first to explain symptoms in boxers, and more recently, chronic traumatic encephalopathy (CTE) has been used to describe a neurodegenerative disease in athletes and military personal with a history of multiple concussions. Although there are many similarities between DP and CTE, a number of key differences are apparent especially when comparing movement impairments. The aim of this review is to compare clinical and pathological aspects of DP and CTE with a focus on disorders of movement.

  17. Chronic Traumatic Encephalopathy: Known Causes, Unknown Effects.

    PubMed

    Iacono, Diego; Shively, Sharon B; Edlow, Brian L; Perl, Daniel P

    2017-05-01

    Chronic traumatic encephalopathy (CTE) is a neuropathologic diagnosis typically made in human brains with a history of repetitive traumatic brain injury (rTBI). It remains unknown whether CTE occurs exclusively after rTBI, or whether a single TBI (sTBI) can cause CTE. Similarly, it is unclear whether impact (eg, motor vehicle accidents) and non-impact (eg, blasts) types of energy transfer trigger divergent or common pathologies. While it is established that a history of rTBI increases the risk of multiple neurodegenerative diseases (eg, dementia, parkinsonism, and CTE), the possible pathophysiologic and molecular mechanisms underlying these risks have yet to be elucidated. Published by Elsevier Inc.

  18. Multicystic encephalopathy in abusive head trauma.

    PubMed

    Kubat, Bela; Bilo, Rob A C; van Rijn, Rick R

    2014-01-01

    The proof of abusive head trauma (AHT) in infants is difficult, especially in cases with a long posttraumatic survival period. In the acute phase, injury to the cranio-cervical junction causes disturbances in respiratory and cardiac control, leading to apnea and bradycardia. Infants who survive the acute phase may subsequently develop multicystic encephalopathy. Because some types of changes are age-dependent, examination of the patterns of brain damage in these cases could provide information about the time in which they were inflicted. In particular, this could apply to the extent of the cystic changes, namely that the severity thereof may decrease with older age upon infliction of the trauma. This could potentially date the injury and thereby help to identify the perpetrator. We present an analysis of the patterns of brain damage in cases of AHT-induced multicystic encephalopathy and comment on the possible etiology and the implications thereof. Nine archival cases of trauma-induced multicystic encephalopathy, originating between the years 2005 and 2011, were identified. In 8 of these cases, hematoxilin-eosin-stained whole-hemisphere histologic slides, as well as small histologic slides of cerebellar hemispheres, were available for the evaluation of the topographic distribution of the macroscopic and microscopic changes. The cerebral hemispheres were more affected than the cerebellum. The magnitude of the cystic changes did not correlate with the age at which the trauma had occurred, nor the surviva period. All cases showed asymmetrical affection of the cerebral hemispheres, which in 3 cases was very pronounced. The analysis revealed both ischemia- and hypoperfusion-induced injury patterns. Analysis of the magnitude and the distribution of the damage do not assist in the estimation of the period at which the trauma had occurred. The evaluation showed that ischemia, and to a lesser extent, hypoperfusion, were the major mechanisms of brain injury in these cases

  19. Hashimoto's encephalopathy mimicking Creutzfeldt-Jakob disease.

    PubMed

    Gauthier, Angela C; Baehring, Joachim M

    2017-01-01

    Hashimoto's encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto's thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966U/mL. After one month of 60mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved. Physicians should strongly consider this uncommon diagnosis in patients with rapid cognitive decline and no other clear etiology.

  20. Chronic traumatic encephalopathy and the availability cascade.

    PubMed

    Solomon, Gary S; Sills, Allen

    2014-09-01

    Chronic traumatic encephalopathy (CTE) in sports has been known for > 85 years, and has experienced a resurgence of interest over the past decade, both in the media and in the scientific community. However, there appears to be a disconnection between the public's perception of CTE and the currently available scientific data. The cognitive bias known as the "availability cascade" has been suggested as a reason to explain this rift in knowledge. This review summarizes and updates the history of CTE in sports, discusses recent epidemiological and autopsy studies, summarizes the evidence base related to CTE in sports, and offers recommendations for future directions.

  1. Hepatic Encephalopathy and Sleepiness: An Interesting Connection?

    PubMed Central

    Montagnese, Sara; Turco, Matteo; Amodio, Piero

    2015-01-01

    Sleep-wake abnormalities in patients with cirrhosis have been traditionally associated with hepatic encephalopathy (HE). In recent years, a certain amount of work has been devoted to the study of this relationship. This has lead to a modified picture, with weakening of the association between HE and poor night sleep, and the emergence of stronger links between HE and excessive daytime sleepiness. This brief review focuses on the evidence in favor of the interpretation of HE as a sleepiness syndrome, and on the diagnostic, therapeutic and social implications of such an interpretation. PMID:26041958

  2. Epileptic phenomena in bismuth toxic encephalopathy.

    PubMed Central

    Buge, A; Supino-Viterbo, V; Rancurel, G; Pontes, C

    1981-01-01

    Seventy patients admitted to hospital with bismuth encephalopathy had repeated clinical and EEG examinations. All the patients exhibited myoclonic jerks, but no paroxysmal features ever appeared on EEG. Computed tomography showed cortical hyperdensities. Seizures were observed in 22 patients, but epileptic EEG patterns appeared only when the bismuth blood level was below 1500 microgram/1. It is suggested that a high cortical intracellular bismuth concentration induces a "cortical inhibition" which causes suppression of physiological electrical brain activity, the absence of EEG paroxysmal phenomena during myoclonic jerks, and explains the rarity of epileptic seizures. Images PMID:7205307

  3. Methadone intoxication in a child: toxic encephalopathy?

    PubMed

    Anselmo, Marisol; Campos Rainho, António; do Carmo Vale, Maria; Estrada, João; Valente, Rosalina; Correia, Manuela; Vieira, José Pedro; Barata, Deolinda

    2006-07-01

    Methadone is used in the treatment of opioid addiction. Acute intoxication can lead to severe consequences and can even be lethal. In several case reports and small series, a presumably toxic leukoencephalopathy is described resulting from inhalation of heroin. We present the case of a 3-year-old boy who ingested methadone accidentally. In a coma with acute obstructive hydrocephalus owing to massive cerebellar edema and supratentorial lesions, he was successfully treated with methylprednisolone and cerebrospinal fluid external drainage. To our knowledge, this is the first report of an encephalopathy associated with synthetic opioid intoxication.

  4. [Star fruit (Averrhoa carambola) toxic encephalopathy].

    PubMed

    Signaté, A; Olindo, S; Chausson, N; Cassinoto, C; Edimo Nana, M; Saint Vil, M; Cabre, P; Smadja, D

    2009-03-01

    Ingestion of star fruit (Averrhoa carambola) can induce severe intoxication in subjects with chronic renal failure. Oxalate plays a key role in the neurotoxicity of star fruit. We report the cases of two patients with unknown chronic renal insufficiency who developed severe encephalopathy after ingestion of star fruit. The two patients developed intractable hiccups, vomiting, impaired consciousness and status epilepticus. Diffusion-weighted MR imaging showed cortical and thalamic hyperintense lesions related to epileptic status. They improved after being submitted to continuous hemofiltration which constitutes the most effective treatment during the acute phase.

  5. Repetitive Head Impacts and Chronic Traumatic Encephalopathy.

    PubMed

    McKee, Ann C; Alosco, Michael L; Huber, Bertrand R

    2016-10-01

    Chronic traumatic encephalopathy (CTE) is a distinctive neurodegenerative disease that occurs as a result of repetitive head impacts. CTE can only be diagnosed by postmortem neuropathologic examination of brain tissue. CTE is a unique disorder with a pathognomonic lesion that can be reliably distinguished from other neurodegenerative diseases. CTE is associated with violent behaviors, explosivity, loss of control, depression, suicide, memory loss and cognitive changes. There is increasing evidence that CTE affects amateur athletes as well as professional athletes and military veterans. CTE has become a major public health concern.

  6. Therapeutic Hypothermia for Neonatal Encephalopathy in Low- and Middle-Income Countries: A Systematic Review and Meta-Analysis

    PubMed Central

    Pauliah, Shreela S.; Shankaran, Seetha; Wade, Angie; Cady, Ernest B.; Thayyil, Sudhin

    2013-01-01

    Although selective or whole body cooling combined with optimal intensive care improves outcomes following neonatal encephalopathy in high-income countries, the safety and efficacy of cooling in low-and middle-income countries is not known. Objective We performed a systematic review and meta-analysis of all published randomised or quasi-randomised controlled trials of cooling therapy for neonatal encephalopathy in low-and middle-income countries. Results Seven trials, comprising a total of 567 infants were included in the meta-analysis. Most study infants had mild (15%) or moderate encephalopathy (48%) and did not receive invasive ventilation (88%). Cooling devices included water-circulating cooling caps, frozen gel packs, ice, water bottles, and phase-changing material. No statistically significant reduction in neonatal mortality was seen with cooling (risk ratio: 0.74, 95% confidence intervals: 0.44 to 1.25). Data on other neonatal morbidities and long-term neurological outcomes were insufficient. Conclusion Cooling therapy was not associated with a statistically significant reduction in neonatal mortality in low-and middle-income countries although the confidence intervals were wide and not incompatible with results seen in high-income countries. The apparent lack of treatment effect may be due to the heterogeneity and poor quality of the included studies, inefficiency of the low technology cooling devices, lack of optimal neonatal intensive care, sedation and ventilatory support, overuse of oxygen, or may be due to the intrinsic difference in the population, for example higher rates of perinatal infection, obstructed labor, intrauterine growth retardation and maternal malnutrition. Evaluation of the safety and efficacy of cooling in adequately powered randomised controlled trials is required before cooling is offered in routine clinical practice in low-and middle-income countries. PMID:23527034

  7. Correlation of EEG, CT, and MRI Brain with Neurological Outcome at 12 Months in Term Newborns with Hypoxic Ischemic Encephalopathy

    PubMed Central

    Jose, Annu; Matthai, John; Paul, Sarah

    2013-01-01

    Objective: To correlate electroencephalogram (EEG), computed tomography (CT), and magnetic resonance imaging (MRI) brain with neurological outcome at 12 months in term neonates with hypoxic ischemic encephalopathy. Design: Prospective observational study. Setting: Neonatal intensive care unit (NICU) in a tertiary care teaching hospital. Materials and Methods: The study was conducted between June 2010 and November 2011. Consecutive term neonates with perinatal asphyxia and hypoxic ischemic encephalopathy were the subjects. All babies were managed as per standard protocol. EEG was done as soon as the baby was stable and CT brain within 7 days. MRI was done at 3 months. Neurodevelpmental assessment was done at 12 months. Results: Of the 31 babies, four died and one was lost to follow-up. Neurodevelopmental at 12 months of age was normal in 15 babies. EEG was normal in six babies and all of them had a normal neurodevelopment. Thirteen of the 14 babies with burst suppression pattern were abnormal (P<0.001). CT brain was normal in 14 and all of them had normal neurodevelopment (P<0.001), while 11 of the 12 with cerebral edema had abnormal outcome (P<0.001). Of the 16 babies with normal MRI, 14 were normal, while all six babies with abnormal signals in the cortex and thalamus had abnormal outcome (P=0.002). Conclusions: A normal EEG and CT brain in a term newborn with hypoxic ischemic encephalopathy (HIE) is associated with good neurological outcome. Burst suppression pattern in EEG, bleeds, or hypodensities in the CT and involvement of basal ganglia/thalamus in the MRI are predictors of abnormal outcome. PMID:24251256

  8. [Moderate cerebral hypothermia in hypoxic-ischaemic encephalopathy: Experience after one year].

    PubMed

    Tenorio, V; Alarcón, A; García-Alix, A; Arca, G; Camprubí, M; Agut, T; Figueras, J

    2012-08-01

    Moderate cerebral hypothermia has been shown to be an effective intervention in decreasing mortality and major disabilities in infants with moderate-severe hypoxic-ischaemic encephalopathy (HIE). To describe our experience within the first year of implementation, and to evaluate the feasibility and safety of this intervention. Retrospective study of 20 patients with moderate-severe HIE treated with whole body hypothermia in the Agrupación Sanitaria Hospital Sant Joan de Déu-Hospital Clínic, between January 2009 and June 2010. During this period, 50 patients with perinatal HIE, 26 of them moderate- severe, were admitted to our units. Twenty patients received hypothermia (13 with severe and 7 with moderate HIE). All of them had at least one risk factor for perinatal hypoxia-ischaemia, and clinical signs of HIE. Fifteen had clinical and/or EEG seizures. Core temperature was maintained at 33.5 ± 0.5°C in 76.5% of determinations for infants cooled with a manual control device, and in 93.6% for those cooled with a servo-controlled device (P<.0001). Re-warming took a median time of 10.5 hours. No potentially severe complications related to hypothermia were observed. Seven patients (35%) died, all of them with severe HIE. There were no difficulties in any of the steps of this intervention, and no potentially severe complications related to it were recorded. Both manual and servo-control methods are equally effective on maintaining the target temperature, although temperature shows less variability using the servo-controlled equipment. Copyright © 2011 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  9. Predicting motor outcome and death in term hypoxic-ischemic encephalopathy

    PubMed Central

    Martinez-Biarge, M.; Diez-Sebastian, J.; Kapellou, O.; Gindner, D.; Allsop, J.M.; Rutherford, M.A.

    2011-01-01

    Objectives: Central gray matter damage, the hallmark of term acute perinatal hypoxia-ischemia, frequently leads to severe cerebral palsy and sometimes death. The precision with which these outcomes can be determined from neonatal imaging has not been fully explored. We evaluated the accuracy of early brain MRI for predicting death, the presence and severity of motor impairment, and ability to walk at 2 years in term infants with hypoxic-ischemic encephalopathy (HIE) and basal ganglia–thalamic (BGT) lesions. Methods: From 1993 to 2007, 175 term infants with evidence of perinatal asphyxia, HIE, and BGT injury seen on early MRI scans were studied. BGT, white matter, posterior limb of the internal capsule (PLIC), and cortex and brainstem abnormality were classified by severity. Motor impairment was staged using the Gross Motor Function Classification System. Results: The severity of BGT lesions was strongly associated with the severity of motor impairment (Spearman rank correlation 0.77; p < 0.001). The association between white matter, cortical, and brainstem injury and motor impairment was less strong and only BGT injury correlated significantly in a logistic regression model. The predictive accuracy of severe BGT lesions for severe motor impairment was 0.89 (95% confidence interval 0.83–0.96). Abnormal PLIC signal intensity predicted the inability to walk independently by 2 years (sensitivity 0.92, specificity 0.77, positive predictive value 0.88, negative predictive value 0.85). Brainstem injury was the only factor with an independent association with death. Conclusion: We have shown that in term newborns with HIE and BGT injury, early MRI can be used to predict death and specific motor outcomes. PMID:21670434

  10. Electrocardiographic and enzymatic correlations with outcome in neonates with hypoxic-ischemic encephalopathy

    PubMed Central

    2012-01-01

    Background Perinatal asphyxia leading to hypoxic-ischemic encephalopathy (HIE) is a common problem causing multi organ dysfunction including myocardial involvement which can affect the outcome. Objective To evaluate the myocardial dysfunction in neonates having HIE by electrocardiographic(ECG) and cardiac enzymes (CK Total, CK-MB and Troponin I) and find out the relationship with HIE and outcome. Design/Methods This was a hospital based prospective study. Sixty term neonates who had suffered perinatal asphyxia and developed HIE were enrolled. Myocardial involvement was assessed by clinical, ECG, and CK Total, CK-MB and Troponin I measurements. Results Of 60 cases, 13(21.7%) were in mild, 27(45%) in moderate and 20(33.3%) belonged to severe,HIE. ECG was abnormal in 46 (76.7%); of these 19 (41.3%) had grade I, 13 (28.2%) grades II and III each and 1 (2.1%) with grade IV changes. Serum levels of CK Total, CK- MB and Troponin I were raised in 54 (90%), 52 (86.6%) and 48 (80%) neonates, respectively. ECG changes and enzymatic levels showed increasing abnormalities with severity of HIE, and the differences among different grades were significant (p = 0.002, 0.02, <0.001 and 0.004, respectively). Nineteen (32%) cases died during hospital stay. The non- survivors had high proportion of abnormal ECG (p = 0.024), raised levels of CK-MB (p = 0.018) and Troponin I (p = 0.008) in comparison to survivors. Conclusions Abnormal ECG and cardiac enzymes levels are found in HIE and can lead to poor outcome due to myocardial damage Early detection can help in better management and survival of these neonates. PMID:22823976

  11. Association of NOS3 tag polymorphisms with hypoxic-ischemic encephalopathy.

    PubMed

    Kuzmanić Samija, Radenka; Primorac, Dragan; Resić, Biserka; Lozić, Bernarda; Krzelj, Vjekoslav; Tomasović, Maja; Stoini, Eugenio; Samanović, Ljubo; Benzon, Benjamin; Pehlić, Marina; Boraska, Vesna; Zemunik, Tatijana

    2011-06-01

    To test the association of NOS3 gene with hypoxic-ischemic encephalopathy (HIE). The study included 110 unrelated term or preterm born children (69 boys and 41 girls) with HIE and 128 term and preterm born children (60 boys and 68 girls) without any neurological problems after the second year of life. Children with perinatal HIE fulfilled the diagnostic criteria for perinatal asphyxia. All children were admitted to the Clinical Hospital Split between 1992 and 2008. We analyzed 6 tagging single nucleotide polymorphisms (SNP) within NOS3 gene (rs3918186, rs3918188, rs1800783, rs1808593, rs3918227, rs1799983), in addition to previously confirmed NOS3-associated SNP rs1800779. Genotyping was conducted using real-time polymerase chain reaction (PCR). Association analyses were performed according to allelic and genotypic distribution. Allelic test did not show any SNP association with HIE. SNP rs1808593 showed genotype association (P=0.008) and rs1800783-rs1800779 TG haplotype showed an association with HIE (P<0.001). The study had 80% statistical power to detect (α=0.05) an effect with odds ratio (OR)=2.07 for rs3918186, OR=1.69 for rs3918188, OR=1.70 for rs1800783, OR=1.80 for rs1808593, OR=2.10 for rs3918227, OR=1.68 for rs1800779, and OR=1.76 for rs1799983, assuming an additive model. Despite the limited number of HIE patients, we observed genotypic and haplotype associations of NOS3 polymorphisms with HIE.

  12. Current Reports on Perinatal Intimate Partner Violence.

    PubMed

    Stewart, Donna E; Vigod, Simone N; MacMillan, Harriet L; Chandra, Prabha S; Han, Alice; Rondon, Marta B; MacGregor, Jennifer C D; Riazantseva, Ekaterina

    2017-05-01

    The purpose of this study was to review the literature on perinatal intimate partner violence, focusing on recent knowledge to guide mental health professionals on the best approaches to identify and treat women exposed to perinatal intimate partner violence. Risk factors have been broadened from individual victim and perpetrator factors to include relationship, community, and societal factors which interact together. Better information is now available on how to identify, document, and treat women exposed to violence around the time of conception, pregnancy, and the postpartum period. Recent information helps psychiatrists and other mental health professionals assist women exposed to violence related to the perinatal period; however, further research is needed to provide improved evidence for optimal interventions for better patient outcomes.

  13. Fetal and perinatal consequences of maternal obesity.

    PubMed

    Vasudevan, Chakrapani; Renfrew, Mary; McGuire, William

    2011-09-01

    In many industrialised countries, one in five women booking for antenatal care is obese. As well as affecting maternal health, maternal obesity may have important adverse consequences for fetal, neonatal and long-term health and well-being. Maternal obesity is associated with a higher risk of stillbirth, elective preterm birth and perinatal mortality. The incidence of severe birth defects, particularly neural tube and structural cardiac defects, appears to be higher in infants of obese mothers. Fetal macrosomia associated with maternal obesity and gestational diabetes predisposes infants to birth injuries, perinatal asphyxia and transitional problems such as neonatal respiratory distress and metabolic instability. Maternal obesity may also result in long-term health problems for offspring secondary to perinatal problems and to intrauterine and postnatal programming effects. Currently, the available interventions to prevent and treat maternal obesity are of limited proven utility and further research is needed to define the effects of maternal weight management interventions on fetal and neonatal outcomes.

  14. Substance use in the perinatal period

    PubMed Central

    Forray, Ariadna; Foster, Dawn

    2015-01-01

    Perinatal substance use remains a major public health problem and is associated with a number of deleterious maternal and fetal effects. Polysubstance use in pregnancy is common, and can potentiate adverse maternal and fetal outcomes. Tobacco is the most commonly used substance in pregnancy, followed by alcohol and illicit substances. The treatments for perinatal substance use are limited and consist mostly of behavioral and psychosocial interventions. Of these contingency management has shown the most efficacy. More recently, novel interventions such as progesterone for postpartum cocaine use have shown promise. The purpose of this review is to examine the recent literature on the use of tobacco, alcohol, cannabis, stimulants, and opioids in the perinatal period, their effects on maternal and fetal health and current treatments. PMID:26386836

  15. Perinatal Care for Women Who Are Addicted: Implications for Empowerment.

    ERIC Educational Resources Information Center

    Carter, Carolyn S.

    2002-01-01

    This article explores societal responses to perinatal drug abuse, including stigmatic attitudes and behaviors of health care workers. Empowering strategies are suggested by which social workers and clients can potentially redefine perinatal drug abuse as a health problem rather than a legal issue and improve the environment in which perinatal care…

  16. [Tobacco control policies and perinatal health].

    PubMed

    Peelen, M J; Sheikh, A; Kok, M; Hajenius, P; Zimmermann, L J; Kramer, B W; Hukkelhoven, C W; Reiss, I K; Mol, B W; Been, J V

    2017-01-01

    Study the association between the introduction of tobacco control policies in the Netherlands and changes in perinatal outcomes. National quasi-experimental study. We used Netherlands Perinatal Registry data (now called Perined) for the period 2000-2011. We studied whether the introduction of smoke-free legislation in workplaces plus a tobacco tax increase and mass media campaign in January 2004, and extension of the smoke-free law to the hospitality industry accompanied by another tax increase and media campaign in July 2008, was associated with changes in perinatal outcomes. We studied all singleton births (gestational age: 24+0 to 42+6 weeks). Our primary outcome measures were: perinatal mortality, preterm birth and being small-for-gestational-age (SGA). Interrupted time series logistic regression analyses were performed to investigate changes in these outcomes occurred after the introduction of the aforementioned tobacco control policies (ClinicalTrials.gov: NCT02189265). Among 2,069,695 singleton births, 13,027 (0.6%) perinatal deaths, 116,043 (5.6%) preterm live-births and 187,966 (9.1%) SGA live-births were observed. The policies introduced in January 2004 were not associated with significant changes in any of the primary outcome measures. A -4.4% (95% CI: -6.4 to -2.4; p < 0.001) decrease in odds of a SGA birth was observed after the policy extension in July 2008 to include a smoke-free hospitality industry, a further tax increase and another media campaign. This translates to an estimated over 500 cases of SGA being averted per year. A reduction in SGA births, but not preterm birth or perinatal mortality, was observed in the Netherlands after extension of the smoke-free workplace law to include bars and restaurants, in conjunction with a tax increase and media campaign in 2008.

  17. Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

    PubMed Central

    Wu, Xiujuan; Wu, Wei; Pan, Wei; Wu, Limin; Liu, Kangding; Zhang, Hong-Liang

    2015-01-01

    Acute necrotizing encephalopathy (ANE) is a rare but distinctive type of acute encephalopathy with global distribution. Occurrence of ANE is usually preceded by a virus-associated febrile illness and ensued by rapid deterioration. However, the causal relationship between viral infections and ANE and the exact pathogenesis of ANE remain unclear; both environmental and host factors might be involved. Most cases of ANE are sporadic and nonrecurrent, namely, isolated or sporadic ANE; however, few cases are recurrent and with familial episodes. The recurrent and familial forms of ANE were found to be incompletely autosomal-dominant. Further the missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 (RANBP2) were identified. Although the clinical course and the prognosis of ANE are diverse, the hallmark of neuroradiologic manifestation of ANE is multifocal symmetric brain lesions which are demonstrated by computed tomography (CT) or magnetic resonance imaging (MRI). The treatment of ANE is still under investigation. We summarize the up-to-date knowledge on ANE, with emphasis on prompt diagnosis and better treatment of this rare but fatal disease. PMID:25873770

  18. [Clinical Features and Treatment of Hashimoto Encephalopathy].

    PubMed

    Maki, Yoshimitsu; Takashima, Hiroshi

    2016-09-01

    Hashimoto encephalopathy (HE) is characterized by heterogeneous neurological symptoms. HE is diagnosed based on three criteria-the presence of antithyroid antibodies, neurological symptoms from the cerebrum and/or cerebellum, and a positive response to immunotherapy. We clinically analyzed 18 patients (3 men, 15 women; age range, 38-81years) diagnosed with HE in our hospital from May 2013 to January 2016. Eleven patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or neuralgia. Surprisingly, the majority of the pain was distributed in a manner that was not explainable anatomically. Seventeen patients showed motor disturbances, such as weakness, paresis of extremities, or dexterity movement disorder, and eight patients showed give-way weakness, which is disruption of continuous muscle contraction. Other symptoms indicative of brain-related anomalies such as tremor, dystonia, involuntary movements, cerebellar ataxia, parkinsonism, memory loss, and chronic fatigue were also seen. In most patients, such motor, sensory, or higher brain functions were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immunoadsorption therapy. Although give-way weakness and anatomically unexplainable pain are typically considered as being psychogenic in origin, the presence of these symptoms is indicative of HE. HE exhibits diffuse involvement of the entire brain and thus, these symptoms are explainable. We propose that physicians should not diagnose somatoform disorders without first excluding autoimmune encephalopathy.

  19. Endotoxemia, encephalopathy, and mortality in cirrhotic patients.

    PubMed

    Bigatello, L M; Broitman, S A; Fattori, L; Di Paoli, M; Pontello, M; Bevilacqua, G; Nespoli, A

    1987-01-01

    Endotoxemia without sepsis was detected with a chromogenic Limulus assay in 36 of 39 (92.3%) cirrhotic patients and was absent in seven healthy volunteers. In 11 patients who underwent elective portasystemic shunt, portal vein endotoxemia was higher than inferior vena caval: p less than 0.05, systemic endotoxin levels did not change, compared to preoperative levels, on the 1st, 2nd, and 3rd postoperative days, attendant to an uneventful recovery. In 21 patients in hepatic encephalopathy after esophagogastric hemorrhage, systemic endotoxemia was higher than in well-compensated cirrhotics: p less than 0.001; it was higher in deep than in light coma: p less than 0.05; it was higher in those who died than in those who survived: p less than 0.001. Endotoxin levels showed a positive correlation with serum bilirubin: r = 0.59, p less than 0.001, and a negative correlation with prothrombin activity: r = -0.59, p less than 0.001. These data show endotoxemia without sepsis is a constant finding in cirrhosis and increasing levels of endotoxemia are associated with hepatic failure, encephalopathy, and death.

  20. Neuronal cell death in hepatic encephalopathy.

    PubMed

    Butterworth, Roger F

    2007-12-01

    It is generally assumed that neuronal cell death is minimal in liver failure and is insufficient to account for the neuropsychiatric symptoms characteristic of hepatic encephalopathy. However, contrary to this assumption, neuronal cell damage and death are well documented in liver failure patients, taking the form of several distinct clinical entities namely acquired (non-Wilsonian) hepatocerebral degeneration, cirrhosis-related Parkinsonism, post-shunt myelopathy and cerebellar degeneration. In addition, there is evidence to suggest that liver failure contributes to the severity of neuronal loss in Wernicke's encephalopathy. The long-standing nature of the thalamic and cerebellar lesions, over 80% of which are missed by routine clinical evaluation, together with the probability that they are nutritional in origin, underscores the need for careful nutritional management (adequate dietary protein, Vitamin B(1)) in liver failure patients. Mechanisms identified with the potential to cause neuronal cell death in liver failure include NMDA receptor-mediated excitotoxicity, lactic acidosis, oxidative/nitrosative stress and the presence of pro-inflammatory cytokines. The extent of neuronal damage in liver failure may be attenuated by compensatory mechanisms that include down-regulation of NMDA receptors, hypothermia and the presence of neuroprotective steroids such as allopregnanolone. These findings suggest that some of the purported "sequelae" of liver transplantation (gait ataxia, memory loss, confusion) could reflect preexisting neuropathology.

  1. Clinical presentation of chronic traumatic encephalopathy

    PubMed Central

    Daneshvar, Daniel H.; Baugh, Christine M.; Seichepine, Daniel R.; Montenigro, Philip H.; Riley, David O.; Fritts, Nathan G.; Stamm, Julie M.; Robbins, Clifford A.; McHale, Lisa; Simkin, Irene; Stein, Thor D.; Alvarez, Victor E.; Goldstein, Lee E.; Budson, Andrew E.; Kowall, Neil W.; Nowinski, Christopher J.; Cantu, Robert C.; McKee, Ann C.

    2013-01-01

    Objective: The goal of this study was to examine the clinical presentation of chronic traumatic encephalopathy (CTE) in neuropathologically confirmed cases. Methods: Thirty-six adult male subjects were selected from all cases of neuropathologically confirmed CTE at the Boston University Center for the Study of Traumatic Encephalopathy brain bank. Subjects were all athletes, had no comorbid neurodegenerative or motor neuron disease, and had next-of-kin informants to provide retrospective reports of the subjects' histories and clinical presentations. These interviews were conducted blind to the subjects' neuropathologic findings. Results: A triad of cognitive, behavioral, and mood impairments was common overall, with cognitive deficits reported for almost all subjects. Three subjects were asymptomatic at the time of death. Consistent with earlier case reports of boxers, 2 relatively distinct clinical presentations emerged, with one group whose initial features developed at a younger age and involved behavioral and/or mood disturbance (n = 22), and another group whose initial presentation developed at an older age and involved cognitive impairment (n = 11). Conclusions: This suggests there are 2 major clinical presentations of CTE, one a behavior/mood variant and the other a cognitive variant. PMID:23966253

  2. Wernicke encephalopathy with atypical magnetic resonance imaging.

    PubMed

    Liou, Kuang-Chung; Kuo, Shu-Fan; Chen, Lu-An

    2012-11-01

    Wernicke encephalopathy (WE) is a medical emergency caused by thiamine (vitamin B1) deficiency. Typical clinical manifestations are mental change, ataxia, and ocular abnormalities. Wernicke encephalopathy is an important differential diagnosis in all patients with acute mental change. However, the disorder is greatly underdiagnosed. Clinical suspicion, detailed history taking, and neurologic evaluations are important for early diagnosis. Magnetic resonance imaging (MRI) is currently considered the diagnostic method of choice. Typical MRI findings of WE are symmetrical involvement of medial thalamus, mammillary body, and periaqueductal gray matter. Prompt thiamine supplement is important in avoiding unfavorable outcomes. Here, we report a case of alcoholic WE with typical clinical presentation but with atypical MRI. Axial fluid-attenuated inversion recovery images showing symmetrical hyperintensity lesions in dentate nuclei of cerebellum, olivary bodies, and dorsal pons. Although atypical MRI findings are more common in nonalcoholic WE, it can also occur in alcoholic WE. This article is aimed to highlight the potential pitfalls in diagnosing acute mental change, the importance of clinical suspicion, and early treatment in WE.

  3. Aluminum induced encephalopathy in the rat

    SciTech Connect

    Lipman, J.J.; Colowick, S.P.; Lawrence, P.P.; Abumrad, N.N.

    1988-01-01

    Aluminum tartrate (AlT) but not sodium tartrate (NaT) produces a progressive encephalopathy when injected intracerebroventricularly in the rat. This syndrome, lethal within 30-35 days, is characterized by progressively deranged behavior. An early startle reaction, later joined by locomotor discoordination is followed by locomotor and electrocorticographic (ECoG) seizures in chronically instrumented AlT rats. There is early dissociation between ECoG and locomotor aspects. When tested in the shuttlebox for estimation of learning and memory function 7-8 days after AlT injection, marked impairment of both active and passive avoidance was observed. Glucose uptake capacity of synaptosomes from brain areas of AlT and NaT animals was indexed by the 2-deoxy-D-glucose method. Striatal and cortical synaptosomes showed reduced uptake activity 7 days following Alt injection. By day 14, hypothalamic areas also became affected, striatal uptake was further inhibited, and cortical uptake was reduced to 57% of control. The ECoG background rhythm remained unchanged until days 20-23, when the mean peak frequency was reduced. The model may be useful in the study of central aluminum toxicity and may have predictive validity in the testing of procedures to counter aluminum-associated encephalopathies in man. 44 references, 4 figures, 1 table.

  4. The Frequency and Severity of Magnetic Resonance Imaging Abnormalities in Infants with Mild Neonatal Encephalopathy.

    PubMed

    Walsh, Brian H; Neil, Jeffrey; Morey, JoAnn; Yang, Edward; Silvera, Michelle V; Inder, Terrie E; Ortinau, Cynthia

    2017-08-01

    To assess and contrast the incidence and severity of abnormalities on cerebral magnetic resonance imaging (MRI) between infants with mild, moderate, and severe neonatal encephalopathy who received therapeutic hypothermia. This retrospective cohort studied infants with mild, moderate, and severe neonatal encephalopathy who received therapeutic hypothermia at a single tertiary neonatal intensive care unit between 2013 and 2015. Two neuroradiologists masked to the clinical condition evaluated brain MRIs for cerebral injury after therapeutic hypothermia using the Barkovich classification system. Additional abnormalities not included in this classification system were also noted. The rate, pattern, and severity of abnormalities/injury were compared across the grades of neonatal encephalopathy. Eighty-nine infants received therapeutic hypothermia and met study criteria, 48 with mild neonatal encephalopathy, 35 with moderate neonatal encephalopathy, and 6 with severe neonatal encephalopathy. Forty-eight infants (54%) had an abnormality on MRI. There was no difference in the rate of overall MRI abnormalities by grade of neonatal encephalopathy (mild neonatal encephalopathy 54%, moderate neonatal encephalopathy 54%, and severe neonatal encephalopathy 50%; P= .89). Basal ganglia/thalamic injury was more common in those with severe neonatal encephalopathy (mild neonatal encephalopathy 4%, moderate neonatal encephalopathy 9%, severe neonatal encephalopathy 34%; P = .03). In contrast, watershed injury did not differ between neonatal encephalopathy grades (mild neonatal encephalopathy 36%, moderate neonatal encephalopathy 32%, severe neonatal encephalopathy 50%; P = .3). Mild neonatal encephalopathy is commonly associated with MRI abnormalities after therapeutic hypothermia. The grade of neonatal encephalopathy during the first hours of life may not discriminate adequately between infants with and without cerebral injury noted on MRI after therapeutic hypothermia

  5. Genetics and genomics: impact on perinatal nursing.

    PubMed

    Lewis, Judith A

    2011-01-01

    In 1953, Watson and Crick first described the structure of the DNA molecule, an event that led to a new understanding of the nature of heredity. Just 50 years later, a conference was held in Bethesda, Maryland to announce the completion of the sequencing of the human genome. The era of genomic healthcare has begun, and it has profound implications for nursing education, nursing practice, and nursing research. This article will highlight some important areas in perinatal and neonatal nursing that have been affected by genetics and genomics, as well as some emerging areas of research that will be relevant to perinatal and neonatal nursing.

  6. Harnessing gene expression networks to prioritize candidate epileptic encephalopathy genes.

    PubMed

    Oliver, Karen L; Lukic, Vesna; Thorne, Natalie P; Berkovic, Samuel F; Scheffer, Ingrid E; Bahlo, Melanie

    2014-01-01

    We apply a novel gene expression network analysis to a cohort of 182 recently reported candidate Epileptic Encephalopathy genes to identify those most likely to be true Epileptic Encephalopathy genes. These candidate genes were identified as having single variants of likely pathogenic significance discovered in a large-scale massively parallel sequencing study. Candidate Epileptic Encephalopathy genes were prioritized according to their co-expression with 29 known Epileptic Encephalopathy genes. We utilized developing brain and adult brain gene expression data from the Allen Human Brain Atlas (AHBA) and compared this to data from Celsius: a large, heterogeneous gene expression data warehouse. We show replicable prioritization results using these three independent gene expression resources, two of which are brain-specific, with small sample size, and the third derived from a heterogeneous collection of tissues with large sample size. Of the nineteen genes that we predicted with the highest likelihood to be true Epileptic Encephalopathy genes, two (GNAO1 and GRIN2B) have recently been independently reported and confirmed. We compare our results to those produced by an established in silico prioritization approach called Endeavour, and finally present gene expression networks for the known and candidate Epileptic Encephalopathy genes. This highlights sub-networks of gene expression, particularly in the network derived from the adult AHBA gene expression dataset. These networks give clues to the likely biological interactions between Epileptic Encephalopathy genes, potentially highlighting underlying mechanisms and avenues for therapeutic targets.

  7. [Clinical importance and diagnostic methods of minimal hepatic encephalopathy].

    PubMed

    Stawicka, Agnieszka; Zbrzeźniak, Justyna; Świderska, Aleksandra; Kilisińska, Natalia; Świderska, Magdalena; Jaroszewicz, Jerzy; Flisiak, Robert

    2016-02-01

    Minimal hepatic encephalopathy (MHE) encompasses a number of neuropsychological and neurophysiological disorders in patients suffering from liver cirrhosis, who do not display abnormalities during a medical interview or physical examination. A negative influence of MHE on the quality of life of patients suffering from liver cirrhosis was confirmed, which include retardation of ability of operating motor vehicles and disruption of multiple health-related areas, as well as functioning in the society. The data on frequency of traffic offences and accidents amongst patients diagnosed with MHE in comparison to patients diagnosed with liver cirrhosis without MHE, as well as healthy persons is alarming. Those patients are unaware of their disorder and retardation of their ability to operate vehicles, therefore it is of utmost importance to define this group. The term minimal hepatic encephalopathy (formerly "subclinical" encephalopathy) erroneously suggested the unnecessity of diagnostic and therapeutic procedures in patients with liver cirrhosis. Diagnosing MHE is an important predictive factor for occurrence of overt encephalopathy - more than 50% of patients with this diagnosis develop overt encephalopathy during a period of 30 months after. Early diagnosing MHE gives a chance to implement proper treatment which can be a prevention of overt encephalopathy. Due to continuing lack of clinical research there exist no commonly agreed-upon standards for definition, diagnostics, classification and treatment of hepatic encephalopathy. This article introduces the newest findings regarding the importance of MHE, scientific recommendations and provides detailed descriptions of the most valuable diagnostic methods.

  8. Venlafaxine as single therapy associated with hypertensive encephalopathy.

    PubMed

    Edvardsson, Bengt

    2015-01-01

    Hypertensive encephalopathy with the clinicoradiological entity posterior reversible encephalopathy syndrome in the setting of venlafaxine as single therapy has not been reported earlier. A 46-year-old man developed hypertensive encephalopathy associated with venlafaxine as single therapy. Magnetic resonance imaging of the brain, pre and post gadolinium, carried out on day 2, displayed an increased T2 signal in the cortex on both the T2 and FLAIR images throughout the frontal and temporal lobes and in the cerebellum. Venlafaxine therapy was stopped. The patient gradually improved and he became seizure free and the blood pressure successively became normal. A magnetic resonance imaging after six weeks displayed marked regression of the abnormalities. On follow-up after 3 months, his blood pressure had been normal and he had not had any symptoms. The prescribed antiepileptic drug was discontinued as well as antihypertensive treatment. He had not experienced any new symptoms at follow-up after one year. The patient in this report had hypertensive encephalopathy associated with venlafaxine therapy. The imaging findings are compatible with hypertensive encephalopathy/posterior reversible encephalopathy syndrome. Venlafaxine is a drug used very frequently. Venlafaxine may infrequently induce hypertensive crisis. Hypertensive encephalopathy may rarely occur in the setting of venlafaxine as single therapy even in low to moderate doses. Patients on venlafaxine should have regular monitoring of blood pressure. Knowledge of the side effects is vital. Venlafaxine must be discontinued if significant hypertension persists.

  9. Hyponatremia in hepatic encephalopathy: an accomplice or innocent bystander?

    PubMed

    Yun, Byung Cheol; Kim, W Ray

    2009-06-01

    Hyponatremia, a common complication inpatients with advanced liver disease and impaired free water clearance, has been shown to be an important predictor of short-term mortality. Hepatic encephalopathy, also a late complication of end-stage liver disease, has been associated with low-grade cerebral edema as a result of swelling of astrocytes. Guevara et al. hypothesized that hyponatremia and the resultant depletion of organic osmolytes (e.g.,myo-inositol) from brain cells contribute to brain edema, playing an important role in the pathogenesis of hepatic encephalopathy. Using a multivariable analysis, they demonstrated that hyponatremia increased the risk of hepatic encephalopathy more than eightfold, after adjustment for serum bilirubin and creatinine concentrations and previous history of encephalopathy. Their magnetic resonance spectroscopy data correlated low brain concentrations of myoinositol with hepatic encephalopathy. As both hyponatremia and encephalopathy occur in patients with advanced liver disease, it has been difficult to implicate hyponatremia independently in the pathogenesis of hepatic encephalopathy. Guevara's data do suggest that hyponatremia is more likely an accomplice than an innocent bystander.

  10. Efficacy of dextromethorphan and cyclosporine a for acute encephalopathy.

    PubMed

    Matsuo, Muneaki; Maeda, Toshiyuki; Ono, Nobuyasu; Sugihara, Susumu; Kobayashi, Ikuko; Koga, Daisuke; Hamasaki, Yuhei

    2013-03-01

    Acute encephalopathy with biphasic seizures and late reduced diffusion was recently established clinicoradiologically as an encephalopathy syndrome. The outcome of this encephalopathy is characterized by a low mortality rate and high incidence of neurologic sequelae. Although the exact pathogenesis of this encephalopathy is uncertain, excitotoxic injury with delayed neuronal death is proposed. On the basis of this hypothesis, we tried a combination therapy of N-methyl-D-aspartate receptor antagonist, dextromethorphan, and apoptosis inhibitor, cyclosporine A, in four patients with acute encephalopathy with biphasic seizures and late reduced diffusion. All patients recovered except for hyperactivity in one patient. Furthermore, an additional four patients with near-miss encephalopathy, who showed mild disturbance of consciousness at 24 hours after prolonged febrile seizures associated with exanthem subitum, recovered without secondary seizures by the early administration of dextromethorphan. The combination regimen of dextromethorphan and cyclosporine A could be effective for the treatment and prevention of acute encephalopathy with biphasic seizures and late reduced diffusion. Copyright © 2013 Elsevier Inc. All rights reserved.

  11. Harnessing Gene Expression Networks to Prioritize Candidate Epileptic Encephalopathy Genes

    PubMed Central

    Oliver, Karen L.; Lukic, Vesna; Thorne, Natalie P.; Berkovic, Samuel F.; Scheffer, Ingrid E.; Bahlo, Melanie

    2014-01-01

    We apply a novel gene expression network analysis to a cohort of 182 recently reported candidate Epileptic Encephalopathy genes to identify those most likely to be true Epileptic Encephalopathy genes. These candidate genes were identified as having single variants of likely pathogenic significance discovered in a large-scale massively parallel sequencing study. Candidate Epileptic Encephalopathy genes were prioritized according to their co-expression with 29 known Epileptic Encephalopathy genes. We utilized developing brain and adult brain gene expression data from the Allen Human Brain Atlas (AHBA) and compared this to data from Celsius: a large, heterogeneous gene expression data warehouse. We show replicable prioritization results using these three independent gene expression resources, two of which are brain-specific, with small sample size, and the third derived from a heterogeneous collection of tissues with large sample size. Of the nineteen genes that we predicted with the highest likelihood to be true Epileptic Encephalopathy genes, two (GNAO1 and GRIN2B) have recently been independently reported and confirmed. We compare our results to those produced by an established in silico prioritization approach called Endeavour, and finally present gene expression networks for the known and candidate Epileptic Encephalopathy genes. This highlights sub-networks of gene expression, particularly in the network derived from the adult AHBA gene expression dataset. These networks give clues to the likely biological interactions between Epileptic Encephalopathy genes, potentially highlighting underlying mechanisms and avenues for therapeutic targets. PMID:25014031

  12. Wernicke encephalopathy in a patient with liver failure

    PubMed Central

    Zhao, Pan; Zhao, Yanling; Wei, Zhenman; Chen, Jing; Yan, Lilong

    2016-01-01

    Abstract Early recognition and diagnosis of Wernicke encephalopathy is pivotal for the prognosis of this medical emergency, especially in patients with liver failure which predisposes individuals to develop hepatic encephalopathy. For these patients, distinguishing between hepatic encephalopathy and Wernicke encephalopathy is a challenge in real-world clinical practice. A male patient with 21-year medical history of liver cirrhosis presented diarrhea and ascites. One month before this visit, he was noted to have poor appetite and progressive fatigue. After admission, although several major symptoms, including diarrhea, ascites, hyponatremia, and hypoproteinemia, were greatly improved through appropriate treatments, his laboratory indicators were not changed much. His appetite was not reversed at discharge. On the 5th day after discharge, the patient suddenly became reluctant to speak and did not remember the recent happenings. Simultaneously, unsteady gait and strabismus occurred. On the basis of clinical manifestations and brain magnetic resonance imaging scan results, the patient was diagnosed as Wernicke encephalopathy and these relative symptoms were resolved after intravenous vitamin B1. To our knowledge, this is the second case report of Wernicke encephalopathy developing in a critically ill cirrhotic patient without hepatocellular carcinoma or operative intervention. Wernicke encephalopathy may be underdiagnosed in these patients and this case raises physicians’ awareness of its possible onset. PMID:27399058

  13. The role of methanethiol in the pathogenesis of hepatic encephalopathy.

    PubMed

    Blom, H J; Ferenci, P; Grimm, G; Yap, S H; Tangerman, A

    1991-03-01

    Mixed disulfides of methanethiol represent a relative estimate for an exposure to methanethiol. The concentrations of methanethiol-mixed disulfides, methionine, 4-methylthio-2-oxobutyrate and ammonia were measured in patients with different stages of hepatic encephalopathy, in patients with chronic kidney failure and in healthy subjects. In patients with hepatic encephalopathy, the mean serum concentrations of all these compounds were elevated. However, the elevations of methanethiol-mixed disulfides were small and partly caused by decreased renal function. In addition, the levels of methanethiol-mixed disulfides did not differ significantly between the different grades of hepatic encephalopathy. The concentrations of methanethiol-mixed disulfides were substantially lower than those previously observed in healthy subjects after an oral methionine load or in a patient with a deficiency in methionine adenosyltransferase, the latter without causing encephalopathy. We concluded that the role of methanethiol in the pathogenesis of hepatic encephalopathy is probably minor, if not insignificant. In the patients with hepatic encephalopathy, a significant correlation was found between the concentrations of methionine and 4-methylthio-2-oxobutyrate and between 4-methylthio-2-oxobutyrate and methanethiol-mixed disulfides, supporting the theory that methanethiol is formed by way of the methionine transamination pathway. Evidence is provided that, besides the methionine transsulfuration pathway, the transamination pathway is also impaired in patients with hepatic encephalopathy.

  14. Hepatic Encephalopathy: An Update on the Pathophysiology and Therapeutic Options

    PubMed Central

    Elwir, Saleh; Rahimi, Robert S.

    2017-01-01

    Abstract Hepatic encephalopathy is a spectrum of reversible neuropsychiatric abnormalities, seen in patients with liver dysfunction and/or portosystemic shunting. One of the most debilitating complications of cirrhosis, encephalopathy affects 30–45% of cirrhotics. In addition to significantly affecting the lives of patients and their caregivers, it is also associated with increased morbidity and mortality as well as significant utilization of health care resources. In this paper, we provide an overview on the pathophysiology, diagnosis, management and newer therapies of hepatic encephalopathy. PMID:28660152

  15. Legal Responsibilities of Physicians When They Diagnose Hepatic Encephalopathy.

    PubMed

    Vierling, John M

    2015-08-01

    Both covert hepatic encephalopathy (CHE) and overt hepatic encephalopathy (OHE) impair the ability to operate machinery. The legal responsibilities of US physicians who diagnose and treat patients with hepatic encephalopathy vary among states. It is imperative that physicians know the laws regarding reporting in their state. OHE represents a neuropsychiatric impairment that meets general reporting criteria. The medical advisory boards of the states have not identified OHE as a reportable condition. In the absence of validated diagnostic guidelines, physicians are not obligated to perform tests for CHE. There is a need for explicit guidance from professional associations regarding this issue.

  16. Isolated brainstem involvement in a patient with hypertensive encephalopathy.

    PubMed

    Osman, Y; Imam, Y Z; Salem, K; Al-Hail, H; Uthman, B; Deleu, D

    2013-01-01

    Hypertensive encephalopathy typically presents with headache, confusion, and bilateral parietooccipital vasogenic edema. Brainstem edema in hypertensive encephalopathy usually occurs in association with typical supratentorial parieto-occipital changes and is usually asymptomatic. We report here a patient with hypertensive encephalopathy, with isolated brain stem involvement on magnetic resonance imaging (MRI). Rapid treatment of hypertension resulted in clinical and radiological improvement. Prompt recognition of the condition and aggressive treatment of hypertension in such patients is crucial to relieve edema and prevent life-threatening progression.

  17. Two Cases of Hypertensive Encephalopathy Involving the Brainstem

    PubMed Central

    Choi, Jay Chol; Kang, Ji-Hoon

    2007-01-01

    Hypertensive encephalopathy is a medical emergency whose clinical manifestations are usually associated with bilateral parieto-occipital lesions. Predominant brainstem edema without accompanying occipital lesions is rare in hypertensive encephalopathy and usually occurs in patients with secondary hypertension. We describe the clinical and radiological features of two patients with reversible hypertensive brainstem encephalopathy. Both patients had chronic renal failure, but the extensive neuroimaging abnormalities revealed few clinical features of brainstem involvement. The clinical findings and neuroimaging abnormalities resolved once the hypertension was treated. PMID:19513343

  18. Prominent Bilateral Hand Tremor in Hashimoto's Encephalopathy: A Video Demonstration.

    PubMed

    Ramcharan, Kanterpersad; Hosein, Nadeem; Teelucksingh, Joel David; Rampersad, Fidel; Teelucksingh, Surujpal

    2016-01-01

    Hashimoto's encephalopathy often presents with neuropsychiatric manifestations including seizures and movement disorders. We describe a patient who presented with bilateral hand tremor and mild cognitive defects that fulfilled the criteria for a diagnosis of Hashimoto's encephalopathy. There was a rapid response to glucocorticoid therapy with relapse following treatment withdrawal. Recently published clinical criteria for the diagnosis of Hashimoto's encephalopathy include seizures, myoclonus, hallucinations, or stroke-like episodes but do not include tremor. Our case had mild cognitive dysfunction and a coarse tremor as the predominant clinical features, which probably represent mild disease.

  19. Severe early onset ethylmalonic encephalopathy with West syndrome.

    PubMed

    Papetti, Laura; Garone, Giacomo; Schettini, Livia; Giordano, Carla; Nicita, Francesco; Papoff, Paola; Zeviani, Massimo; Leuzzi, Vincenzo; Spalice, Alberto

    2015-12-01

    Ethylmalonic encephalopathy (EE) is a rare autosomal recessive disorder characterized by early onset encephalopathy, chronic diarrhoea, petechiae, orthostatic acrocyanosis and defective cytochrome c oxidase (COX) in muscle and brain. High levels of lactic, ethylmalonic and methylsuccinic acids are detected in body fluids. EE is caused by mutations in ETHE1 gene, a mitochondrial sulfur dioxygenase. Neurologic signs and symptoms include progressively delayed development, hypotonia, seizures, and abnormal movements. We report on the clinical, electroencephalographic and MRI findings of a baby with a severe early onset encephalopathy associated with novel ETHE1 gene mutation. This is the first case described in literature with an early pure epileptic onset, presenting with West syndrome.

  20. Global and cultural perinatal nursing research: improving clinical practice.

    PubMed

    Callister, Lynn Clark

    2011-01-01

    High-quality perinatal nursing care should be based on the best evidence including research findings, clinical expertise, and the preferences of women and their families. Principles of perinatal research initiatives are defined, with suggested research priorities designed to close current gaps in the micro and macro environments of perinatal nursing throughout the world. Nearly a decade ago, the following question was asked, "Where is the 'E' (evidence) in maternal child health?" Improving the quality and safety of perinatal nursing care for culturally diverse women globally is the primary goal of nurse researchers leading the future of perinatal healthcare.

  1. Clinical and Neurologic Manifestation of Minimal Hepatic Encephalopathy and Overt Hepatic Encephalopathy.

    PubMed

    Basu, P Patrick; Shah, Niraj James

    2015-08-01

    Hepatic encephalopathy (HE) shows a wide spectrum of neuropsychiatric manifestations. A combined effort with neuropsychological and psychometric evaluation has to be performed to recognize the syndrome, whereas minimal HE (MHE) is largely under-recognized. Subtle symptoms of MHE can only be diagnosed through specialized neuropsychiatric testing. Early diagnosis and treatment may drastically improve the quality of life for many cirrhotic patients. Further research to gain better insight into the pathophysiology and diagnostic accuracy of HE will help determine future management strategies.

  2. Chronic traumatic encephalopathy: contributions from the Boston University Center for the Study of Traumatic Encephalopathy.

    PubMed

    Riley, David O; Robbins, Clifford A; Cantu, Robert C; Stern, Robert A

    2015-01-01

    Chronic Traumatic Encephalopathy (CTE) is a neurodegenerative disease associated with repetitive brain trauma (RBT). Initially described in boxers, CTE has now been found in other contact sport athletes with a history of RBT. In recent years, there has been tremendous media attention regarding CTE, primarily because of the deaths of high profile American football players who were found to have CTE upon neuropathological examination. However, the study of CTE remains in its infancy. This review focuses on research from the Centre for the Study of Traumatic Encephalopathy (CSTE) at Boston University. This study reviews the formation of the CSTE, major CSTE publications and current ongoing research projects at the CSTE. The neuropathology of CTE has been well-described. Current research focuses on: methods of diagnosing the disease during life (including the development of biomarkers), examination of CTE risk factors (including genetic susceptibility and head impact exposure variables); description of the clinical presentation of CTE; development of research diagnostic criteria for Traumatic Encephalopathy Syndrome; and assessment of mechanism and pathogenesis. Current research at the BU CSTE is aimed at increasing understanding of the long-term consequences of repetitive head impacts and attempting to begin to answer several of the unanswered questions regarding CTE.

  3. Management of hepatic encephalopathy in the hospital.

    PubMed

    Leise, Michael D; Poterucha, John J; Kamath, Patrick S; Kim, W Ray

    2014-02-01

    Hepatic encephalopathy (HE) develops in up to 50% of patients with cirrhosis and is a feature of decompensated cirrhosis. With the goal of reviewing the evidence for treatment and prevention of overt hepatic encephalopathy, pubmed was searched using search terms hepatic encephalopathy AND treatment, limited to human studies from January 1, 2003, through December 1, 2013, and supplemented by key references. The inpatient incidence of HE is approximately 23,000 annually, and management of these patients is common for internists and subspecialists. Treatment of the hospitalized patient with HE has changed in recent years. Treatment entails 2 phases: induction and maintenance of remission. Most cases of significant HE are precipitated by infection, gastrointestinal bleeding, medications, or other culprits. All patients should be evaluated for secondary triggers of HE, and treatment should be initiated with a nonabsorbable disaccharide (ie, lactulose) in most patients. Rifaximin (off label) can be added in patients not responding to lactulose. Neomycin is a less preferred alternative to rifaximin owing to its adverse effect profile. Other therapies, including zinc, L-ornithine-L-aspartate, and branched-chain amino acids, can be considered for patients not responding to disaccharides and nonabsorbable antibiotics. Large portosystemic shunts may be embolized in patients with medically refractory recurrent or severe HE with otherwise well-compensated cirrhosis. Molecular Adsorbent Recirculating System is now available for patients with severe HE who do not respond to medical therapy. It is critically important that patients hospitalized with significant HE continue maintenance therapy at the time of dismissal to prevent further episodes. Patients with a first-time episode of HE can be administered lactulose, and careful instructions should be provided to patients and caregivers about dose titration to achieve 3 bowel movements daily. Patients with recurrent HE episodes

  4. DRINKING WATER ARSENIC AND PERINATAL OUTCOMES

    EPA Science Inventory

    Drinking Water Arsenic and Perinatal Outcomes
    DT Lobdell, Z Ning, RK Kwok, JL Mumford, ZY Liu, P Mendola

    Many studies have documented an association between drinking water arsenic (DWA) and cancer, vascular diseases, and dermatological outcomes, but few have investigate...

  5. Parental Grief Response to Perinatal Death.

    ERIC Educational Resources Information Center

    Smith, Anne Clarke; Borgers, Sherry B.

    1989-01-01

    Examined grief responses of parents suffering perinatal loss and explored effects of gender, type of loss, time since loss, number of losses, and subsequent pregnancy on grief response. Responses to Grief Experience Inventory from 176 such parents revealed subjects suffering grief. Grief response was affected by subjects' perception that loss was…

  6. Perinatal Depression Treatment Preferences Among Latina Mothers

    PubMed Central

    Lara-Cinisomo, Sandraluz; Wisner, Katherine L.; Burns, Rachel M.; Chaves-Gnecco, Diego

    2014-01-01

    The study described here was designed to determine treatment preferences among Latinas to identify treatment options that meet their needs and increase their engagement. Focus group interviews were conducted with 22 prenatal and postpartum Latinas at risk for depression. The group interviews were conducted in Spanish and English using a standardized interview protocol. Focus group transcripts were analyzed to identify themes regarding perinatal depression coping strategies, preferred approaches to treating perinatal depression, and recommendations for engaging perinatal Latinas in treatment. The results suggest that Latinas’ treatment preferences consist of a pathway (i.e., hierarchical) approach that begins with the use of one’s own resources, followed by the use of formal support systems (e.g., home-visiting nurse), and supplemented with the use of behavioral therapy. Antidepressant use was judged to be acceptable only in severe cases or after delivery. The data indicate that to increase health-seeking behaviors among perinatal Latinas, practitioners should first build trust. PMID:24469693

  7. Ethical issues in perinatal mental health research.

    PubMed

    Brandon, Anna R; Shivakumar, Geetha; Lee, Simon Craddock; Inrig, Stephen J; Sadler, John Z

    2009-11-01

    To review the background of current ethical standards for the conduct of perinatal mental health research and describe the ethical challenges in this research domain. Current literature reflects a growing sentiment in the scientific community that having no information regarding the impact of psychiatric treatment on the mother and developing fetus/infant poses dangers that may exceed the risks involved in research. However, without sufficient consensus across the scientific community, both regulatory bodies and perinatal researchers find themselves without a framework for decision making that satisfactorily limits the risks and facilitates the benefits of participation of pregnant and lactating women in clinical research. Psychiatric research in perinatal mental health is critically important as it enables clinicians and patients to participate in informed decision-making concerning treatment for psychiatric disorders. Specific areas of concern include fetal safety, maternal risk, the therapeutic misconception, commercial interests, forensic/legal issues, the informed consent process, and study design. Developing guidelines that address ethical challenges and include the views and concerns of multiple stakeholders could improve the access of perinatal women to the benefits of participation in mental health research in addition to providing evidence-based mental healthcare for this subpopulation.

  8. DRINKING WATER ARSENIC AND PERINATAL OUTCOMES

    EPA Science Inventory

    Drinking Water Arsenic and Perinatal Outcomes
    DT Lobdell, Z Ning, RK Kwok, JL Mumford, ZY Liu, P Mendola

    Many studies have documented an association between drinking water arsenic (DWA) and cancer, vascular diseases, and dermatological outcomes, but few have investigate...

  9. Is there an association between female circumcision and perinatal death?

    PubMed Central

    Essen, Birgitta; Bodker, Birgit; Sjoberg, N-O; Gudmundsson, Saemundur; Ostergren, P-O; Langhoff-Roos, Jens

    2002-01-01

    OBJECTIVE: In Sweden, a country with high standards of obstetric care, the high rate of perinatal mortality among children of immigrant women from the Horn of Africa raises the question of whether there is an association between female circumcision and perinatal death. METHOD: To investigate this, we examined a cohort of 63 perinatal deaths of infants born in Sweden over the period 1990-96 to circumcised women. FINDINGS: We found no evidence that female circumcision was related to perinatal death. Obstructed or prolonged labour, caused by scar tissue from circumcision, was not found to have any impact on the number of perinatal deaths. CONCLUSION: The results do not support previous conclusions that genital circumcision is related to perinatal death, regardless of other circumstances, and suggest that other, suboptimal factors contribute to perinatal death among circumcised migrant women. PMID:12219153

  10. Experiences with perinatal loss from the health professionals' perspective.

    PubMed

    Pastor Montero, Sonia María; Romero Sánchez, José Manuel; Hueso Montoro, César; Lillo Crespo, Manuel; Vacas Jaén, Ana Gema; Rodríguez Tirado, María Belén

    2011-01-01

    The purpose of this paper is to know the experience of health professionals in situations of perinatal death and grief and to describe their action strategies in the management of perinatal loss. A qualitative study with a phenomenological approach was carried out through interviews conducted with 19 professionals. Three thematic categories were identified: Healthcare practice, feelings aroused by perinatal loss and meaning and beliefs about perinatal loss and grief. The results revealed that the lack of knowledge and skills to deal with perinatal loss are identified as the main reason behind unsuitable attitudes that are usually adopted in these situations. This generates anxiety, helplessness and frustration that compromise professional competency. The conclusion reached is that the promotion of training programs to acquire knowledge, skills and abilities in management of perinatal bereavement and the development of a clinical practice guideline for perinatal loss are necessary.

  11. Hypoxic ischemic encephalopathy in a case of intranuclear rod myopathy without any prenatal sentinel event.

    PubMed

    Kawase, Koya; Nishino, Ichizo; Sugimoto, Mari; Kouwaki, Masanori; Koyama, Norihisa; Yokochi, Kenji

    2015-02-01

    Intranuclear rod myopathy (IRM), a variant of nemaline myopathy, is characterized by the presence of nemaline bodies in myonuclei. We report a case of IRM presenting with hypoxic ischemic encephalopathy (HIE). There were no prenatal complications caused by fetal brain injury. Although no nemaline bodies were observed in the cytoplasm, intranuclear rods were observed in some fibers under light and electron microscopy. Molecular analysis identified a heterozygous variant, c.449C>T (p.Thr150Ile), in ACTA1. On magnetic resonance imaging at 9days of age, injuries to the basal ganglia, thalamus, and brainstem consistent with perinatal HIE were seen. Respiratory insufficiency at birth was strongly suspected to be the cause of HIE. Our case highlights that a patient with a congenital neuromuscular disorder who presents with severe respiratory dysfunction requiring substantial resuscitative efforts at birth can be complicated by HIE without any prenatal sentinel event. Prenatal detection of neuromuscular disorders, careful management of delivery, and neonatal resuscitation and adequate respiratory management are important in preventing irreversible brain injury in these patients. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  12. [Can implementation of intensified perinatal survey be effective in improving the quality of perinatal care?].

    PubMed

    Troszyński, Michał

    2010-01-01

    Intensive scientific research and rapid technical progress have influenced the rapid fall in term newborn mortality. At the same time new problems have arisen such as saving the lives of infants with low and very low birth weight. Solving these problems needs reorganization of perinatal care, better equipment, especially in reference units and in outpatient clinics, as well as more intensive staff training. to obtain information whether implementation of intensified perinatal survey of fetus and newborn mortality can improve the quality of perinatal care in Poland. Implementation of the survey based on Central Statistics Office (GUS) data, Ministry of Health MZ-29 section X Document and the author's own studies. In the year 2008 newborn with birth weight less than 2500 g, constituted 6,06% liveborn infants, newborn weighing from 1000 to 2499 g - 5%, those with weight from 500 to 999 g - 0.51% of all live born infants. These figures differ according to voivodeship. The intensive survey concerning birth weight and perinatal mortality indeces in voivodeshipPoland, as well as in individual voivodeships, showed differences between data from the Central Statistics Office and data from the Ministry of Health MZ-29 document. This may be due to different methods of registrating newborn deaths eg. newborns transfered in the first weekoflife from the maternity ward to intensive care neonatal ward or to other specialistic departaments. Another reason for the difference may be discharge of the newborn data according to the place of birth or the mother's place of permanent domicile registration. This causes disturbances in flow of infomation resulting in ineffective analysis of perinatal mortality and of perinatal care evaluation. In the ongoing analysis it was found that in Poland stillbirths occur twice as often as perinatal deaths (4.3 per thousands) stillbirths and 2.15 per thousands perinatal deaths), with significant differences between voivodeships. This makes it

  13. Perinatal hypophosphatasia caused by uniparental isodisomy.

    PubMed

    Watanabe, Atsushi; Satoh, Shuhei; Fujita, Atsushi; Naing, Banyar Than; Orimo, Hideo; Shimada, Takashi

    2014-03-01

    Hypophosphatasia (HPP) is an inherited disorder characterized by defective bone mineralization caused by mutations in the alkaline phosphatase gene (ALPL). Clinically, the disease spans a great continuum of disease severity and six forms can be distinguished according to the age of onset. The most severe is the autosomal recessive perinatal form, a major prenatal skeletal dysplasia in Japan. The ALPL mutation c.1559delT causes perinatal HPP and occurs frequently in the Japanese. Most patients with perinatal HPP in Japan are homozygous for c.1559delT, and their parents are usually heterozygous with no evidence of consanguinity. Here we identified a fetus with perinatal HPP resulting from an unusual mechanism known as paternal uniparental isodisomy (UPD) of chromosome 1. Sequence analysis of ALPL in the patient revealed the presence of the homozygous mutation c.1559delT. We suspected UPD because the father and mother were heterozygous and wild type, respectively. Analysis of polymorphic microsatellite markers spanning chromosome 1 and whole-genome arrays revealed a uniparental inheritance from the father and excluded deletions or de novo mutations. This is the first description of perinatal HPP caused by UPD. This report also emphasizes the low recurrence risk of a non-Mendelian inheritance pattern in UPD and the value of determining parental genotypes with homozygous mutations in a patient to confirm whether the condition is caused by UPD or not, even when the mutation is detected as a hot spot, as described in the literature. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. [Follow-up of newborns with hypoxic-ischaemic encephalopathy].

    PubMed

    Martínez-Biarge, M; Blanco, D; García-Alix, A; Salas, S

    2014-07-01

    Hypothermia treatment for newborn infants with hypoxic-ischemic encephalopathy reduces the number of neonates who die or have permanent neurological deficits. Although this therapy is now standard of care, neonatal hypoxic-ischaemic encephalopathy still has a significant impact on the child's neurodevelopment and quality of life. Infants with hypoxic-ischaemic encephalopathy should be enrolled in multidisciplinary follow-up programs in order to detect impairments, to initiate early intervention, and to provide counselling and support for families. This article describes the main neurodevelopmental outcomes after term neonatal hypoxic-ischaemic encephalopathy. We offer recommendations for follow-up based on the infant's clinical condition and other prognostic indicators, mainly neonatal neuroimaging. Other aspects, such as palliative care and medico-legal issues, are also briefly discussed. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  15. Hepatic encephalopathy in acute-on-chronic liver failure.

    PubMed

    Lee, Guan-Huei

    2015-10-01

    The presence of hepatic encephalopathy (HE) within 4 weeks is part of the criteria for defining acute-on-chronic liver failure (ACLF). The pathophysiology of HE is complex, and hyperammonemia and cerebral hemodynamic dysfunction appear to be central in the pathogenesis of encephalopathy. Recent data also suggest that inflammatory mediators may have a significant role in modulating the cerebral effect of ammonia. Multiple prospective and retrospective studies have shown that hepatic encephalopathy in ACLF patients is associated with higher mortality, especially in those with grade III-IV encephalopathy, similar to that of acute liver failure (ALF). Although significant cerebral edema detected by CT in ACLF patients appeared to be less common, specialized MRI imaging was able to detect cerebral edema even in low grade HE. Ammonia-focused therapy constitutes the basis of current therapy, as in the treatment of ALF. Emerging treatment strategies focusing on modulating the gut-liver-circulation-brain axis are discussed.

  16. Brain proton magnetic resonance spectroscopy for hepatic encephalopathy

    NASA Astrophysics Data System (ADS)

    Ong, Chin-Sing; McConnell, James R.; Chu, Wei-Kom

    1993-08-01

    Liver failure can induce gradations of encephalopathy from mild to stupor to deep coma. The objective of this study is to investigate and quantify the variation of biochemical compounds in the brain in patients with liver failure and encephalopathy, through the use of water- suppressed, localized in-vivo Proton Magnetic Resonance Spectroscopy (HMRS). The spectral parameters of the compounds quantitated are: N-Acetyl Aspartate (NAA) to Creatine (Cr) ratio, Choline (Cho) to Creatine ratio, Inositol (Ins) to Creatine ratio and Glutamine-Glutamate Amino Acid (AA) to Creatine ratio. The study group consisted of twelve patients with proven advanced chronic liver failure and symptoms of encephalopathy. Comparison has been done with results obtained from five normal subjects without any evidence of encephalopathy or liver diseases.

  17. [Cognitive impairment in elderly patients with acute hypertensive encephalopathy].

    PubMed

    Baev, V M; Kozlov, D B

    2012-01-01

    Acute hypertensive encephalopathy in elderly patients appears reversible mild cognitive impairment. The erythrocyte sedimentation rate and blood creatinine measured during a hypertensive crisis are predictors of decline of visual-spatial orientation after two weeks of treatment.

  18. Genetics Home Reference: MECP2-related severe neonatal encephalopathy

    MedlinePlus

    ... related severe neonatal encephalopathy have severe to profound intellectual disability. Affected males have breathing problems, with some having ... syndrome , which has signs and symptoms that include intellectual disability, seizures, and movement problems. In some cases, males ...

  19. Another cause of vaccine encephalopathy: a case of Angelman syndrome.

    PubMed

    Novy, Jan; Catarino, Claudia B; Chinthapalli, Krishna; Smith, Shelagh M; Clayton-Smith, Jill; Hennekam, Raoul C M; Hammond, Peter; Sisodiya, Sanjay M

    2012-05-01

    Dravet syndrome has been found recently as an important underlying condition in cases of alleged vaccine encephalopathy after pertussis vaccination, where vaccination seemed to have precipitated the occurrence of the disease without modifying the long-term course. We report on a patient diagnosed with Angelman syndrome in her fifth decade, in whom the intellectual disability and epilepsy had been assumed to be caused by a vaccine encephalopathy following smallpox vaccination. Clinical features of Angelman syndrome had faded away. The history of the present patient suggests that genetic conditions other than Dravet syndrome can be associated with an alleged vaccine encephalopathy. A history of vaccine encephalopathy is rare among patients with learning disability and refractory epilepsy (1.4% in our cohort), but it should lead to consideration of a comprehensive genetic work-up if Dravet syndrome is excluded. The early history of the patient, when available, should guide the investigations. Medico-legal aspects are also discussed.

  20. Uremic encephalopathy and other brain disorders associated with renal failure.

    PubMed

    Seifter, Julian Lawrence; Samuels, Martin A

    2011-04-01

    Kidney failure is one of the leading causes of disability and death and one of the most disabling features of kidney failure and dialysis is encephalopathy. This is probably caused by the accumulation of uremic toxins. Other important causes are related to the underlying disorders that cause kidney failure, particularly hypertension. The clinical manifestations of uremic encephalopathy include mild confusional states to deep coma, often with associated movement disorders, such as asterixis. Most nephrologists consider cognitive impairment to be a major indication for the initiation of renal replacement therapy with dialysis with or without subsequent transplantation. Sleep disorders, including Ekbom's syndrome (restless legs syndrome) are also common in patients with kidney failure. Renal replacement therapies are also associated with particular neurologic complications including acute dialysis encephalopathy and chronic dialysis encephalopathy, formerly known as dialysis dementia. The treatments and prevention of each are discussed. © Thieme Medical Publishers.

  1. Hemolytic Uremic Syndrome-associated Encephalopathy Successfully Treated with Corticosteroids.

    PubMed

    Hosaka, Takashi; Nakamagoe, Kiyotaka; Tamaoka, Akira

    2017-09-25

    The encephalopathy that occurs in association with hemolytic uremic syndrome (HUS), which is caused by enterohemorrhagic Escherichia coli (E. coli), has a high mortality rate and patients sometimes present sequelae. We herein describe the case of a 20-year-old woman who developed encephalopathy during the convalescent stage of HUS caused by E.coli O26. Hyperintense lesions were detected in the pons, basal ganglia, and cortex on diffusion-weighted brain MRI. From the onset of HUS encephalopathy, we treated the patient with methylprednisolone (mPSL) pulse therapy alone. Her condition improved, and she did not present sequelae. Our study shows that corticosteroids appear to be effective for the treatment of some patients with HUS encephalopathy.

  2. Nonconvulsive status epilepticus disguising as hepatic encephalopathy.

    PubMed

    Jo, Yong Min; Lee, Sung Wook; Han, Sang Young; Baek, Yang Hyun; Ahn, Ji Hye; Choi, Won Jong; Lee, Ji Young; Kim, Sang Ho; Yoon, Byeol A

    2015-04-28

    Nonconvulsive status epilepticus has become an important issue in modern neurology and epileptology. This is based on difficulty in definitively elucidating the condition and its various clinical phenomena and on our inadequate insight into the intrinsic pathophysiological processes. Despite nonconvulsive status epilepticus being a situation that requires immediate treatment, this disorder may not be appreciated as the cause of mental status impairment. Although the pathophysiology of nonconvulsive status epilepticus remains unknown, this disorder is thought to lead to neuronal damage, so its identification and treatment are important. Nonconvulsive status epilepticus should be considered in the differential diagnosis of patients with liver cirrhosis presenting an altered mental status. We report a case of a 52-year-old male with liver cirrhosis presenting an altered mental status. He was initially diagnosed with hepatic encephalopathy but ultimately diagnosed with nonconvulsive status epilepticus by electroencephalogram.

  3. Chronic Traumatic Encephalopathy: The Impact on Athletes.

    PubMed

    Galgano, Michael A; Cantu, Robert; Chin, Lawrence S

    2016-03-14

    Chronic traumatic encephalopathy (CTE) is a devastating neuropsychological condition afflicting a small percentage of athletes partaking in high-impact sports. The onset of symptoms lags years behind the inciting events. Repetitive minor head injuries are felt to be the main etiology behind CTE. Routine radiographic imaging generally is unremarkable in cases of CTE. Functional magnetic resonance imaging (fMRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) are advanced MRI-based sequences that have shown promise in detecting early radiographic findings that may be reflective of CTE. Progressive neuronal loss is the histopathological hallmark of this neurodegenerative disease. Strategizing earlier detection techniques is paramount in delivering optimal care to athletes afflicted with CTE.

  4. Is chronic traumatic encephalopathy a real disease?

    PubMed

    Randolph, Christopher

    2014-01-01

    Chronic traumatic encephalopathy (CTE) has received widespread media attention and is treated in the lay press as an established disease, characterized by suicidality and progressive dementia. The extant literature on CTE is reviewed here. There currently are no controlled epidemiological data to suggest that retired athletes are at increased risk for dementia or that they exhibit any type of unique neuropathology. There remain no established clinical or pathological criteria for diagnosing CTE. Despite claims that CTE occurs frequently in retired National Football League (NFL) players, recent studies of NFL retirees report that they have an all-cause mortality rate that is approximately half of the expected rate, and even lower suicide rates. In addition, recent clinical studies of samples of cognitively impaired NFL retirees have failed to identify any unique clinical syndrome. Until further controlled studies are completed, it appears to be premature to consider CTE a verifiable disease.

  5. Chronic traumatic encephalopathy and other neurodegenerative proteinopathies.

    PubMed

    Tartaglia, Maria Carmela; Hazrati, Lili-Naz; Davis, Karen D; Green, Robin E A; Wennberg, Richard; Mikulis, David; Ezerins, Leo J; Keightley, Michelle; Tator, Charles

    2014-01-01

    "Chronic traumatic encephalopathy" (CTE) is described as a slowly progressive neurodegenerative disease believed to result from multiple concussions. Traditionally, concussions were considered benign events and although most people recover fully, about 10% develop a post-concussive syndrome with persisting neurological, cognitive and neuropsychiatric symptoms. CTE was once thought to be unique to boxers, but it has now been observed in many different athletes having suffered multiple concussions as well as in military personal after repeated blast injuries. Much remains unknown about the development of CTE but its pathological substrate is usually tau, similar to that seen in Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD). The aim of this "perspective" is to compare and contrast clinical and pathological CTE with the other neurodegenerative proteinopathies and highlight that there is an urgent need for understanding the relationship between concussion and the development of CTE as it may provide a window into the development of a proteinopathy and thus new avenues for treatment.

  6. Leucine metabolism in patients with Hepatic Encephalopathy

    SciTech Connect

    McGhee, A.S.; Kassouny, M.E.; Matthews, D.E.; Millikan, W.

    1986-03-01

    A primed continuous infusion of (/sup 15/N, 1-/sup 13/C)leucine was used to determine whether increased oxidation and/or protein synthesis of leucine occurs in patients with cirrhosis. Five controls and patients were equilibrated on a metabolic balance diet (0.6 g protein per kg ideal body weight (IBW)). An additional four patients were equilibrated in the same manner with the same type of diet with a protein level of 0.75 g per kg IBW. Plasma leucine and breath CO/sub 2/ enrichments were measured by mass spectrometry. Protein synthesis and leucine metabolism were identical in controls and patients when both were fed a diet with 0.6 g protein/kg IBW. Results indicate that systemic derangements of leucine metabolism are not the cause of Hepatic Encephalopathy.

  7. Does this patient have hypertensive encephalopathy?

    PubMed

    Christopoulou, Foteini; Rizos, Evangelos C; Kosta, Paraskevi; Argyropoulou, Maria I; Elisaf, Moses

    2016-05-01

    A 63-year-old man was admitted to our hospital for further investigation and management of brain metastases. The patient was initially presented with a 4-day history of confusion. On the day of admission, the patient was confused, agitated, disorientated in place and time, and had visual disturbances. His blood pressure was repeatedly recorded high, with levels of systolic blood pressure between 170-210 mm Hg. A brain magnetic resonance imaging showed areas of high signal on T2 and fluid-attenuated inversion recovery images, located bilaterally in the white matter of the occipital regions and unilateral in the left frontal lobe, suggestive of posterior reversible encephalopathy syndrome. Aggressive treatment of hypertension resulted in complete resolution of the clinical and radiologic features of the syndrome. Copyright © 2016 American Society of Hypertension. Published by Elsevier Inc. All rights reserved.

  8. Pathophysiology, diagnosis, and management of hepatic encephalopathy.

    PubMed

    Sheasgreen, Christopher; Lu, Lucy; Patel, Ameen

    2014-12-01

    Hepatic encephalopathy (HE) is a common complication of cirrhosis of the liver. It is also extremely debilitating, with an untreated 3-year survival of only 23 %. While the exact pathophysiology of HE has yet to be elucidated, a number of contributing factors have been described. Abnormal levels and altered metabolism of ammonia play a central role. Recently, inflammation has also been identified as a contributor to HE. Improved understanding of the pathophysiology of HE is crucial, as current therapy centers on reduction of the body's ammonia load. Lactulose is the first-line therapy for HE, with some antibiotics recently showing promise for improved outcomes in patients with HE. The role of anti-inflammatory therapies has yet to be evaluated.

  9. Elevated cerebrospinal fluid tau in Wernicke encephalopathy.

    PubMed

    Frijlink, Daphne W; Tilanus, Joachim J; Roks, Gerwin

    2012-08-08

    Wernicke encephalopathy (WE) commonly presents with oculomotor abnormalities, gait ataxia and confusion. WE can mimic rapidly progressive dementia syndromes, such as Creutzfeldt-Jakob disease (CJD). Cerebrospinal fluid (CSF) tau is frequently used for diagnosis of several dementia subtypes, predominantly CJD and Alzheimer's disease. The combination of very high CSF tau (tau) and normal phosphorylated tau (p-tau) levels is almost exclusively seen in aggressive diseases, such as CJD. The authors present a case of a woman with WE, caused by chronic insufficient dietary intake, with highly elevated CSF tau and normal p-tau. The clinical symptoms and CSF findings raised the suspicion of CJD. However, shortly after immediate treatment with thiamine the patient clinically improved. At follow-up, 2.5 months later, she had made a good recovery. This case of rapidly progressive dementia illustrates that, even in the case of a highly elevated CSF tau, clinicians should be alert for treatable causes such as WE.

  10. Rifaximin, Microbiota Biology, and Hepatic Encephalopathy

    PubMed Central

    Peleman, Cedric; Camilleri, Michael

    2016-01-01

    Rifaximin is beneficial in the treatment of minimal hepatic encephalopathy (MHE). Kang et al. (Clin Transl Gastroenterol 7: e187; doi:10.1038/ctg.2016.44) investigated the effects of rifaximin in a mouse model of MHE-associated microbiota without concomitant liver disease. In addition to some impact on the composition of microbiota, rifaximin altered bacterial functions, ameliorated local and systemic inflammation, and reduced enterocyte glutaminase activity. We discuss these effects as well as the interpretation of the permeability studies, given the potential interaction of dysbiosis with dysfunctional intestinal barrier, leading to systemic inflammation and increased uptake of bacterial metabolites that contribute to MHE in the presence of hepatic dysfunction. PMID:27711069

  11. Pathogenesis, Diagnosis, and Treatment of Hepatic Encephalopathy

    PubMed Central

    Atluri, Dileep K; Prakash, Ravi; Mullen, Kevin D

    2011-01-01

    Hepatic encephalopathy (HE) is a neuropsychiatric disorder seen in patients with advanced liver disease or porto-systemic shunts. Based on etiology and severity of HE, the World Congress of Gastroenterology has divided HE into categories and sub-categories. Many user-friendly computer-based neuropsychiatric tests are being validated for diagnosing covert HE. Currently, emphasis is being given to view HE deficits as a continuous spectrum rather than distinct stages. Ammonia is believed to play crucial role in pathogenesis of HE via astrocyte swelling and cerebral edema. However, evidence has been building up which supports the synergistic role of oxidative stress, inflammation and neurosteroids in pathogenesis of HE. At present, treatment of HE aims at decreasing the production and intestinal absorption of ammonia. But as the role of new pathogenetic mechanisms becomes clear, many potential new treatment strategies may become available for clinician. PMID:25755319

  12. [Wernicke Encephalopathy: the importance of the diagnosis].

    PubMed

    Ramos, Cristina Giesta; Pereira, Cláudia

    2006-01-01

    Wernicke Encephalopathy (WE) is a severe neurological disease caused by vitamin B1 (thiamine) deficiency, which is potentially treatable if early diagnosed. This is the clinical case of a young female patient, with renal insufficiency on haemodialysis, who has been submitted to an abdominal surgery. After the intervention, there were difficulties on beginning with enteric nutrition. Some days later she developed gait imbalance. A Brain Magnetic Resonance (MR) was performed and disclosed abnormalities suggestive of WE. After treatment with thiamine, the patient became asymptomatic. Brain MR is crucial for the confirmation of the diagnosis and early detection of WE, as the imagiologic pattern is typical and the clinical diagnosis is frequently difficult to obtain.

  13. Neuroprotective Strategies after Neonatal Hypoxic Ischemic Encephalopathy

    PubMed Central

    Dixon, Brandon J.; Reis, Cesar; Ho, Wing Mann; Tang, Jiping; Zhang, John H.

    2015-01-01

    Neonatal hypoxic ischemic encephalopathy (HIE) is a devastating disease that primarily causes neuronal and white matter injury and is among the leading cause of death among infants. Currently there are no well-established treatments; thus, it is important to understand the pathophysiology of the disease and elucidate complications that are creating a gap between basic science and clinical translation. In the development of neuroprotective strategies and translation of experimental results in HIE, there are many limitations and challenges to master based on an appropriate study design, drug delivery properties, dosage, and use in neonates. We will identify understudied targets after HIE, as well as neuroprotective molecules that bring hope to future treatments such as melatonin, topiramate, xenon, interferon-beta, stem cell transplantation. This review will also discuss some of the most recent trials being conducted in the clinical setting and evaluate what directions are needed in the future. PMID:26389893

  14. The Treatment of Hepatic Encephalopathy by Colectomy

    PubMed Central

    Singer, H.; Harrison, A. W.; Aggett, P. W.

    1965-01-01

    A 64-year-old alcoholic patient with cirrhosis and bleeding esophageal varices developed hepatic encephalopathy following portacaval shunt. The etiology of this syndrome is believed to be related to the absorption of toxic nitrogenous substances derived from the activity of bacteria in the large bowel. Treatment consisted of a low protein diet, frequent purgation, and oral neomycin. Even on a 20-g. protein diet the patient deteriorated to a state approaching coma. Colectomy with ileorectal anastomosis was performed with good result. The patient remained alert and active with no recurrences of cerebral disturbance while enjoying a 60-g. protein diet. No additional treatment was necessary. The literature on colectomy in the treatment of this condition, while brief, reports similar good results. Further trial of colectomy is recommended for cases refractory to more conservative methods of management. PMID:5831216

  15. Hyperammonemic encephalopathy caused by carnitine deficiency.

    PubMed

    Limketkai, Berkeley N; Zucker, Stephen D

    2008-02-01

    Carnitine is an essential co-factor in fatty acid metabolism. Carnitine deficiency can impair fatty acid oxidation, rarely leading to hyperammonemia and encephalopathy. We present the case of a 35-year-old woman who developed acute mental status changes, asterixis, and diffuse muscle weakness. Her ammonia level was elevated at 276 microg/dL. Traditional ammonia-reducing therapies were initiated, but proved ineffective. Pharmacologic, microbial, and autoimmune causes for the hyperammonemia were excluded. The patient was severely malnourished and her carnitine level was found to be extremely low. After carnitine supplementation, ammonia levels normalized and the patient's mental status returned to baseline. In the setting of refractory hyperammonemia, this case illustrates how careful investigation may reveal a treatable condition.

  16. Chronic Traumatic Encephalopathy: The Impact on Athletes

    PubMed Central

    Cantu, Robert; Chin, Lawrence S.

    2016-01-01

    Chronic traumatic encephalopathy (CTE) is a devastating neuropsychological condition afflicting a small percentage of athletes partaking in high-impact sports. The onset of symptoms lags years behind the inciting events. Repetitive minor head injuries are felt to be the main etiology behind CTE. Routine radiographic imaging generally is unremarkable in cases of CTE. Functional magnetic resonance imaging (fMRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) are advanced MRI-based sequences that have shown promise in detecting early radiographic findings that may be reflective of CTE. Progressive neuronal loss is the histopathological hallmark of this neurodegenerative disease. Strategizing earlier detection techniques is paramount in delivering optimal care to athletes afflicted with CTE. PMID:27088064

  17. Wernicke's Encephalopathy in a Patient with Schizophrenia

    PubMed Central

    Harrison, Rebecca A; Vu, Trung; Hunter, Alan J

    2006-01-01

    Clinically, we most often associate Wernicke's encephalopathy (WE) with an alcohol abusing population. However, it is important to consider other causes of malnutrition and vitamin deficiency as risk factors for the development of this disorder. We present a case of a 51-year-old man with schizophrenia and malnutrition who presented with delirium, ophthalmoplegia, and seizures. He responded rapidly to the administration of IV thiamine. Because of the high rate of mortality and morbidity, WE should be high on the differential of any patient at risk for malnutrition or with ophthalmoplegia, regardless of alcohol history. This is particularly important in psychiatric patients where the syndrome may be masked and thus treatment delayed. PMID:16925799

  18. Brain MRI findings in Wernicke encephalopathy.

    PubMed

    Wicklund, Meredith R; Knopman, David S

    2013-08-01

    A 71-year-old woman with myelofibrosis on chemotherapy experienced an acute illness with nausea, vomiting, and diarrhea. Two weeks later, she developed an acute confusional state characterized by disorientation and fluctuating alertness with normal speech and language. Her neurologic examination demonstrated an upper motor neuron pattern of right hemiparesis. She reported double vision though ophthalmoparesis was not appreciated. Her gait was normal. While hospitalized, she developed generalized tonic-clonic seizures. Brain MRI revealed a small area of restricted diffusion of the left precentral gyrus (figure). She was diagnosed with a stroke with secondary seizures; however, as the confusional state resolved, she developed profound retrograde and anterograde amnesia. Review of the brain MRI showed high T2 signal in the medial thalamus and contrast enhancement of the mamillary bodies; a diagnosis of Wernicke-Korsakoff syndrome was entertained and she was started on thiamine replacement. The encephalopathy and hemiparesis resolved though she remains severely amnestic.

  19. Wernicke's encephalopathy: expanding the diagnostic toolbox.

    PubMed

    Lough, Mary E

    2012-06-01

    Wernicke's encephalopathy (WE) is a life threatening neurological disorder that results from thiamine (Vitamin B1) deficiency. Clinical signs include mental status changes, ataxia, occulomotor changes and nutritional deficiency. The conundrum is that the clinical presentation is highly variable. WE clinical signs, brain imaging, and thiamine blood levels, are reviewed in 53 published case reports from 2001 to 2011; 81 % (43/53) were non-alcohol related. Korsakoff Syndrome or long-term cognitive neurological changes occurred in 28 % (15/53). Seven WE cases (13 %) had a normal magnetic resonance image (MRI). Four WE cases (8 %) had normal or high thiamine blood levels. Neither diagnostic tool can be relied upon exclusively to confirm a diagnosis of WE.

  20. National Childhood Encephalopathy Study: an interim report.

    PubMed Central

    Miller, D L; Ross, E M

    1978-01-01

    Data from the first year of the National Childhood Encephalopathy Study were reviewed to see whether any relation was apparent between pertussis vaccination and brain disease. Three hundred and eighty-seven cases of encephalitis and other specified neurological conditions in which the children were admitted to hospital were reported, of which 267 satisfied the study criteria. Control children were matched for age with the index cases, and medical and immunisation histories were reviewed. Few of the index cases had been vaccinated within 28 days before admission to hospital, so that no close association between vaccination and brain disease existed in most cases. The number of children who had recently been immunised was too small for any statistically useful conclusion to be reached about the risk associated with pertussis vaccine. The study is continuing. PMID:709204

  1. Wernicke encephalopathy and Creutzfeldt-Jakob disease.

    PubMed

    Bertrand, A; Brandel, J P; Grignon, Y; Sazdovitch, V; Seilhean, D; Faucheux, B; Privat, N; Brault, J L; Vital, A; Uro-Coste, E; Pluot, M; Chapon, F; Maurage, C A; Letournel, F; Vespignani, H; Place, G; Degos, C F; Peoc'h, K; Haïk, S; Hauw, J J

    2009-06-01

    We assessed the prevalence of Wernicke encephalopathy (WE) in all 657 cases suspected of Creutzfeldt-Jakob (CJD) referred from 2001 to 2006 to the French Neuropathology Network of CJD. Clinical, biological and imaging data were reviewed when the diagnosis of WE was made at autopsy. No CJD was found in five cases suspected of sporadic CJD. In these five cases, myoclonus had been observed in four, CSF 14-3-3 protein in two. In 14 other cases, WE was combined with CJD, 13 of which were sporadic. These belonged mainly to the molecular variants of sporadic CJD associated with a long duration of disease. This stresses the necessity of remaining alert to the diagnosis of WE when CJD is suspected.

  2. Estrogens are neuroprotective factors for hypertensive encephalopathy.

    PubMed

    Pietranera, Luciana; Brocca, Maria Elvira; Roig, Paulina; Lima, Analia; Garcia-Segura, Luis Miguel; De Nicola, Alejandro F

    2015-02-01

    Estrogens are neuroprotective factors for brain diseases, including hypertensive encephalopathy. In particular, the hippocampus is highly damaged by high blood pressure, with several hippocampus functions being altered in humans and animal models of hypertension. Working with a genetic model of primary hypertension, the spontaneously hypertensive rat (SHR), we have shown that SHR present decreased dentate gyrus neurogenesis, astrogliosis, low expression of brain derived neurotrophic factor (BDNF), decreased number of neurons in the hilus of the dentate gyrus, increased basal levels of the estrogen-synthesizing enzyme aromatase, and atrophic dendritic arbor with low spine density in the CA1 region compared to normotensive Wistar Kyoto (WKY) ratsl. Changes also occur in the hypothalamus of SHR, with increased expression of the hypertensinogenic peptide arginine vasopressin (AVP) and its V1b receptor. Following chronic estradiol treatment, SHR show decreased blood pressure, enhanced hippocampus neurogenesis, decreased the reactive astrogliosis, increased BDNF mRNA and protein expression in the dentate gyrus, increased neuronal number in the hilus of the dentate gyrus, further increased the hyperexpression of aromatase and replaced spine number with remodeling of the dendritic arbor of the CA1 region. We have detected by qPCR the estradiol receptors ERα and ERβ in hippocampus from both SHR and WKY rats, suggesting direct effects of estradiol on brain cells. We hypothesize that a combination of exogenously given estrogens plus those locally synthesized by estradiol-stimulated aromatase may better alleviate the hippocampal and hypothalamic encephalopathy of SHR. This article is part of a Special Issue entitled "Sex steroids and brain disorders". Copyright © 2014 Elsevier Ltd. All rights reserved.

  3. Hepatic encephalopathy and fitness to drive.

    PubMed

    Kircheis, Gerald; Knoche, Anja; Hilger, Norbert; Manhart, Frank; Schnitzler, Alfons; Schulze, Horst; Häussinger, Dieter

    2009-11-01

    Low-grade hepatic encephalopathy (HE) may impair fitness to drive. Driving deficits have not yet been characterized, and their relation to psychometric test results is unclear. Fifty-one cirrhotic patients and 48 age-matched controls underwent real driving in a multiple sensor and camera-equipped car, laboratory and "in-car" computer psychometry, and driving instructor's assessment. Ten cirrhotic patients had no hepatic encephalopathy (HE0); 27 and 14 patients suffered from minimal HE (mHE) and overt HE grade I (oHE), respectively. During real driving, mHE and oHE patients showed significantly more violations of in-lane keeping, reduced break use, prolonged reaction times, and diminished stress tolerance compared with control or cirrhotic HE0 patients. In a self-evaluation questionnaire, mHE and oHE, but not the HE0, patients strongly overestimated their driving abilities. According to the driving instructor's assessment, 75%, 48%, and 39% of the patients with HE0, mHE, and oHE, respectively, were fit to drive, compared with 87% in the control group. Driving deficits in oHE patients were largely due to cognitive defects and prolonged reaction times, whereas, in mHE patients, mistakes and attention deficits predominated. Computer psychometric test results worsened with HE severity and age, whereas real driving was age independent. In 25 out of 94 patients, discordant results for driving fitness were obtained (driving instructor's assessment vs computer psychometry); in mHE and oHE patients, the concordance rates were only 62% and 64%, respectively. Despite significant driving deficits, HE patients overestimate their driving abilities. The presence of mHE does not necessarily predict driving unfitness, and computer-based testings cannot reliably predict driving fitness.

  4. Quantitative Risk Assessment of Bovine Spongiform Encephalopathy

    NASA Astrophysics Data System (ADS)

    Tsutsui, Toshiyuki; Kasuga, Fumiko

    Bovine spongiform encephalopathy (BSE) is a progressive neurological disease of cattle affecting the central nervous system and was first diagnosed in the United Kingdom (UK) in 1986 (Wells et al., 1987). This disease is one of the transmissible spongiform encephalopathy (TSE) which includes Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep. The causative agent of TSE is considered to be an abnormal form of prion protein. However, the details of its pathogenic mechanism have not been fully identified. Scrapie, which causes neurological symptoms in sheep and goats, has existed in the UK for 200 years (Hoinville, 1996) and spread across the rest of the world in the 1900s (Detwiler & Baylis, 2003). There has been no report so far that scrapie can be transmitted to humans. Initially, BSE was also considered as a disease affecting only animals. However, a variant type of Creutzfeldt-Jakob disease (vCJD) was first reported in the UK, and exposure to a BSE agent was suspected (Collinge, Sidle, Meads, Ironside, & Hill, 1996). vCJD is clinically and pathologically different from the sporadic type of CJD, and age at clinical onset of vCJD is younger than sporadic type (Will et al., 1996). Since the UK government announced the possible association between BSE and vCJD in 1996, BSE has become a huge public health concern all over the world. Of particular concern about vCJD, the fatal disease in younger age, distorted consumer confidence in beef safety, and as a result reduced beef consumption has been seen in many BSE-affected countries.

  5. Pathology of the Superior Colliculus in Chronic Traumatic Encephalopathy.

    PubMed

    Armstrong, Richard A; McKee, Ann C; Cairns, Nigel J

    2017-01-01

    To investigate neuropathological changes in the superior colliculus in chronic traumatic encephalopathy. The densities of the tau-immunoreactive neurofibrillary tangles, neuropil threads, dot-like grains, astrocytic tangles, and neuritic plaques, together with abnormally enlarged neurons, typical neurons, vacuolation, and frequency of contacts with blood vessels, were studied across the superior colliculus from pia mater to the periaqueductal gray in eight chronic traumatic encephalopathy and six control cases. Tau-immunoreactive pathology was absent in the superior colliculus of controls but present in varying degrees in all chronic traumatic encephalopathy cases, significant densities of tau-immunoreactive neurofibrillary tangles, NT, or dot-like grains being present in three cases. No significant differences in overall density of the tau-immunoreactive neurofibrillary tangles, neuropil threads, dot-like grains, enlarged neurons, vacuoles, or contacts with blood vessels were observed in control and chronic traumatic encephalopathy cases, but chronic traumatic encephalopathy cases had significantly lower mean densities of neurons. The distribution of surviving neurons across the superior colliculus suggested greater neuronal loss in intermediate and lower laminae in chronic traumatic encephalopathy. Changes in density of the tau-immunoreactive pathology across the laminae were variable, but in six chronic traumatic encephalopathy cases, densities of tau-immunoreactive neurofibrillary tangles, neuropil threads, or dot-like grains were significantly greater in intermediate and lower laminae. Pathological changes were not correlated with the distribution of blood vessels. The data suggest significant pathology affecting the superior colliculus in a proportion of chronic traumatic encephalopathy cases with a laminar distribution which could compromise motor function rather than sensory analysis.

  6. Acute Necrotizing Encephalopathy of Childhood (ANEC): A Case Report

    PubMed Central

    HASSANZADEH RAD, Afagh; AMINZADEH, Vahid

    2017-01-01

    Acute Necrotizing Encephalopathy of childhood (ANEC) is a specific type of encephalopathy. After viral infection, it can be diagnosed by bilateral symmetrical lesions predominantly observed in thalami & brainstem of infants & children. Although, it is commonly occurred in Japanese and Taiwanese population. The goal of this article is to report a rare case of ANEC in a 15 months old girl infant from Thaleghani Hospital, Ramian, Gorgan, northern Iran. PMID:28277560

  7. Late onset arginase deficiency presenting with encephalopathy and midbrain hyperintensity

    PubMed Central

    Maramattom, Boby Varkey; Raja, Rajat; Balagopal, Anuroop

    2016-01-01

    Urea cycle disorders (UCD) are very rare metabolic disorders that present with encephalopathy and hyperammonemia. Of the UCDs, Arginase deficiency (ARD) is the rarest and presents in childhood with a progressive spastic diplegia or seizures. Acute presentation in adulthood is extremely unusual.[1] We present the first case of adult onset ARD presenting with encephalopathy and diffusion weighted MRI findings that resembled a moustache in the midbrain. PMID:27570396

  8. Early progressive encephalopathy in boys and MECP2 mutations.

    PubMed

    Kankirawatana, P; Leonard, H; Ellaway, C; Scurlock, J; Mansour, A; Makris, C M; Dure, L S; Friez, M; Lane, J; Kiraly-Borri, C; Fabian, V; Davis, M; Jackson, J; Christodoulou, J; Kaufmann, W E; Ravine, D; Percy, A K

    2006-07-11

    MECP2 mutations mainly occur in females with Rett syndrome. Mutations have been described in 11 boys with progressive encephalopathy: seven of nine with affected sisters and two de novo. The authors report four de novo occurrences: three pathogenic and one potentially pathogenic. Common features include failure to thrive, respiratory insufficiency, microcephaly, and abnormal motor control. MECP2 mutations should be assessed in boys with progressive encephalopathy and one or more of respiratory insufficiency, abnormal movements or tone, and intractable seizures.

  9. Acute encephalopathy with biphasic seizures and late reduced diffusion

    PubMed Central

    Yadav, Saroj S.; Lawande, Malini A.; Kulkarni, Shilpa D.; Patkar, Deepak A.

    2013-01-01

    Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is a syndrome of encephalopathy characterized by biphasic seizures and altered consciousness in the acute stage followed in the subacute stage by restricted diffusion in the subcortical white matter on magnetic resonance imaging. The etiology of AESD has been attributed to viral infection like influenza A and human herpes virus 6. The exact pathogenesis of AESD is uncertain. Here we report a case of AESD, diagnosed based on clinicoradiological correlation. PMID:23772250

  10. Diagnosis and Management of Epileptic Encephalopathies in Children

    PubMed Central

    Jain, Puneet; Tripathi, Manjari

    2013-01-01

    Epileptic encephalopathies refer to a group of disorders in which the unremitting epileptic activity contributes to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone, and these can worsen over time leading to progressive cerebral dysfunction. Several syndromes have been described based on their electroclinical features (age of onset, seizure type, and EEG pattern). This review briefly describes the clinical evaluation and management of commonly encountered epileptic encephalopathies in children. PMID:23970964

  11. Risk of suicidal ideation in adolescents with both self-asphyxial risk-taking behavior and non-suicidal self-injury.

    PubMed

    Brausch, Amy M; Decker, Kristina M; Hadley, Andrea G

    2011-08-01

    This study examined adolescent participation in self-asphyxial risk-taking behaviors (SAB), sometimes known as the "choking game," and its relationship with other adolescent risk behaviors, including non-suicidal self-injury (NSSI). Researchers proposed that participation in SAB and NSSI would be associated with suicidal behavior, disordered eating, and substance use. Using a large community-based sample, results revealed preliminary associations between SAB and other risk-taking behaviors. Adolescents who had engaged in both SAB and NSSI reported more concurrent risk behaviors than adolescents who participated in only one of the behaviors or neither behavior. Results indicate that greater awareness of SAB is important, and continued research can evaluate the possible link between the behavior and risk for suicide.

  12. De novo KCNB1 mutations in epileptic encephalopathy.

    PubMed

    Torkamani, Ali; Bersell, Kevin; Jorge, Benjamin S; Bjork, Robert L; Friedman, Jennifer R; Bloss, Cinnamon S; Cohen, Julie; Gupta, Siddharth; Naidu, Sakkubai; Vanoye, Carlos G; George, Alfred L; Kearney, Jennifer A

    2014-10-01

    Numerous studies have demonstrated increased load of de novo copy number variants or single nucleotide variants in individuals with neurodevelopmental disorders, including epileptic encephalopathies, intellectual disability, and autism. We searched for de novo mutations in a family quartet with a sporadic case of epileptic encephalopathy with no known etiology to determine the underlying cause using high-coverage whole exome sequencing (WES) and lower-coverage whole genome sequencing. Mutations in additional patients were identified by WES. The effect of mutations on protein function was assessed in a heterologous expression system. We identified a de novo missense mutation in KCNB1 that encodes the KV 2.1 voltage-gated potassium channel. Functional studies demonstrated a deleterious effect of the mutation on KV 2.1 function leading to a loss of ion selectivity and gain of a depolarizing inward cation conductance. Subsequently, we identified 2 additional patients with epileptic encephalopathy and de novo KCNB1 missense mutations that cause a similar pattern of KV 2.1 dysfunction. Our genetic and functional evidence demonstrate that KCNB1 mutation can result in early onset epileptic encephalopathy. This expands the locus heterogeneity associated with epileptic encephalopathies and suggests that clinical WES may be useful for diagnosis of epileptic encephalopathies of unknown etiology. © 2014 American Neurological Association.

  13. De Novo KCNB1 Mutations in Epileptic Encephalopathy

    PubMed Central

    Bjork, Robert L.; Friedman, Jennifer R.; Bloss, Cinnamon S.; Cohen, Julie; Gupta, Siddharth; Naidu, Sakkubai; Vanoye, Carlos G.; George, Alfred L.; Kearney, Jennifer A.

    2014-01-01

    Background Numerous studies have demonstrated increased load of de novo copy number variants (CNVs) or single nucleotide variants (SNVs) in individuals with neurodevelopmental disorders, including epileptic encephalopathies, intellectual disability and autism. Methods We searched for de novo mutations in a family quartet with a sporadic case of epileptic encephalopathy with no known etiology to determine the underlying cause using high coverage whole exome sequencing (WES) and lower coverage whole genome sequencing (WGS). Mutations in additional patients were identified by WES. The effect of mutations on protein function was assessed in a heterologous expression system. Results We identified a de novo missense mutation in KCNB1 that encodes the KV2.1 voltage-gated potassium channel. Functional studies demonstrated a deleterious effect of the mutation on KV2.1 function leading to a loss of ion selectivity and gain of a depolarizing inward cation conductance. Subsequently, we identified two additional patients with epileptic encephalopathy and de novo KCNB1 missense mutations that cause a similar pattern of KV2.1 dysfunction. Interpretation Our genetic and functional evidence demonstrate that KCNB1 mutation can result in early onset epileptic encephalopathy. This expands the locus heterogeneity associated with epileptic encephalopathies and suggests that clinical WES may be useful for diagnosis of epileptic encephalopathies of unknown etiology. PMID:25164438

  14. Ifosfamide related encephalopathy: the need for a timely EEG evaluation.

    PubMed

    Feyissa, Anteneh M; Tummala, Sudhakar

    2014-01-15

    Ifosfamide is an alkylating agent useful in the treatment of a wide range of cancers including sarcomas, lymphoma, gynecologic and testicular cancers. Encephalopathy has been reported in 10-40% of patients receiving high-dose IV ifosfamide. To highlight the role of electroencephalogram (EEG) in the early detection and management of ifosfamide related encephalopathy. Retrospective chart review including clinical data and EEG recordings was done on five patients, admitted to MD Anderson Cancer Center between years 2009 and 2012, who developed ifosfamide related acute encephalopathy. All five patients experienced symptoms of encephalopathy soon after (within 12 h-2 days) receiving ifosfamide. Two patients developed generalized convulsions while one patient developed continuous non-convulsive status epilepticus (NCSE) that required ICU admission and intubation. Initial EEG showed epileptiform discharges in three patients; run of triphasic waves in one patient and moderate degree diffuse generalized slowing. Mixed pattern with the presence of both sharps and triphasic waves were also noted. Repeat EEGs within 24_h of symptom onset showed marked improvement that was correlated with clinical improvement. Severity of ifosfamide related encephalopathy correlates with EEG changes. We suggest a timely EEG evaluation for patients receiving ifosfamide who develop features of encephalopathy. © 2013.

  15. Assessment of low-grade hepatic encephalopathy: a critical analysis.

    PubMed

    Kircheis, Gerald; Fleig, Wolfgang E; Görtelmeyer, Roman; Grafe, Susanne; Häussinger, Dieter

    2007-11-01

    The value of paper-pencil tests and West-Haven-criteria for assessment of low-grade hepatic encephalopathy under conditions of a randomized, double-blind, placebo-controlled, clinical trial was evaluated in a cohort of 217 cirrhotics. Patients were graded at least twice clinically for severity of hepatic encephalopathy and tested concomitantly with a recommended psychometric test battery. Re-evaluation of the study documentation showed that at study entry 33% and during the study even 50% of the patients were wrongly allocated to minimal or overt hepatic encephalopathy. Despite the participating physicians' training, 31% of the number-connection-tests-A, 20% of the number-connection-tests-B and 28% of the line-tracing-test were in retrospect considered invalid by an independent psychologist. Neither the Portosystemic-Encephalopathy-Syndrome (PSE) test nor the Psychometric-Hepatic-Encephalopathy-Sum (PHES)-score reliably picked up clinical improvement in the individual patient. Although these test scores could statistically differentiate between patients with minimal and overt hepatic encephalopathy, the clinical classification of individual patients into one of the groups will have a high rate of error. The PHES-Score was less balanced than the score derived from the PSE-Syndrome-Test. Inaccuracies in conducting paper-pencil tests together with the subjectivity and incorrectness of clinical HE-grading question the usefulness of West-Haven-criteria and paper-pencil tests including related scores for quantification of low-grade HE at least in multicenter approaches.

  16. Single Sustained Inflation followed by Ventilation Leads to Rapid Cardiorespiratory Recovery but Causes Cerebral Vascular Leakage in Asphyxiated Near-Term Lambs

    PubMed Central

    Sobotka, Kristina S.; Hooper, Stuart B.; Crossley, Kelly J.; Ong, Tracey; Schmölzer, Georg M.; Barton, Samantha K.; McDougall, Annie R. A.; Miller, Suzie L.; Tolcos, Mary; Klingenberg, Claus; Polglase, Graeme R.

    2016-01-01

    Background A sustained inflation (SI) rapidly restores cardiac function in asphyxic, bradycardic newborns but its effects on cerebral haemodynamics and brain injury are unknown. We determined the effect of different SI strategies on carotid blood flow (CaBF) and cerebral vascular integrity in asphyxiated near-term lambs. Methods Lambs were instrumented and delivered at 139 ± 2 d gestation and asphyxia was induced by delaying ventilation onset. Lambs were randomised to receive 5 consecutive 3 s SI (multiple SI; n = 6), a single 30 s SI (single SI; n = 6) or conventional ventilation (no SI; n = 6). Ventilation continued for 30 min in all lambs while CaBF and respiratory function parameters were recorded. Brains were assessed for gross histopathology and vascular leakage. Results CaBF increased more rapidly and to a greater extent during a single SI (p = 0.01), which then decreased below both other groups by 10 min, due to a higher cerebral oxygen delivery (p = 0.01). Blood brain barrier disruption was increased in single SI lambs as indicated by increased numbers of blood vessel profiles with plasma protein extravasation (p = 0.001) in the cerebral cortex. There were no differences in CaBF or cerebral oxygen delivery between the multiple SI and no SI lambs. Conclusions Ventilation with an initial single 30 s SI improves circulatory recovery, but is associated with greater disruption of blood brain barrier function, which may exacerbate brain injury suffered by asphyxiated newborns. This injury may occur as a direct result of the initial SI or to the higher tidal volumes delivered during subsequent ventilation. PMID:26765258

  17. Onset of asphyxial state in nonrespiring interval between cord clamping and ventilation increases hemodynamic lability of birth transition in preterm lambs.

    PubMed

    Smolich, Joseph J; Kenna, Kelly R; Cheung, Michael M

    2015-03-15

    Experimentally, a typical ∼2-min cord clamp-to-ventilation interval in preterm lambs is accompanied by increased hemodynamic lability of the birth transition. However, whether this lability is related to development of asphyxia after cord clamping, or can be avoided with a shorter clamp-to-ventilation interval, is unknown. To address these questions, anesthetized preterm fetal lambs (gestation 127 ± 2 days) were instrumented with ductus arteriosus and left pulmonary artery flow probes to obtain right ventricular (RV) output, brachiocephalic trunk and aortic isthmus flow probes to measure left ventricular (LV) output, and aortic trunk catheters for pressure measurement and blood gas analysis. With hemodynamics recorded continuously, fetuses were delivered onto the ewe's abdomen and the cord clamped for 1.5 min before ventilation (n = 8), with aortic sampling at 15, 30, 45, and 60 s, or for 0.5 min, with sampling at 15 s (n = 4). With 1.5-min cord clamping, an asphyxial state (Po2 < 10 mmHg) was evident at ≥45 s, with bradycardia and marked falls in LV and RV outputs (by 60% and 50%, P < 0.001), followed after ventilation onset by tachycardia and LV and RV output surges (4- and 3-fold, P < 0.001). By contrast, heart rate and outputs remained stable after 0.5-min cord clamping, with no postventilation change in heart rate or RV output, and a lesser rise in LV output (22%, P < 0.005). In preterm lambs, rapid development of an asphyxial state within 45 s in the cord clamp-to-ventilation interval increased hemodynamic lability of the birth transition, which was reduced with a shorter (∼0.5 min) cord clamp-to-ventilation interval. Copyright © 2015 the American Physiological Society.

  18. Perinatal bereavement: a principle-based concept analysis.

    PubMed

    Fenstermacher, Kimberly; Hupcey, Judith E

    2013-11-01

    To report an analysis of the concept of perinatal bereavement. The concept of perinatal bereavement emerged in the scientific literature during the 1970s. Perinatal bereavement is a practice-based concept, although it is not well-defined in the scientific literature and is often intermingled with the concepts of mourning and grief. Concept Analysis. Using the term 'perinatal bereavement' and limits of only English and human, Pub Med and CINAHL were searched to yield 278 available references dating from 1974-2011. Articles specific to the experience of perinatal bereavement were reviewed. The final data set was 143 articles. The methods of principle-based concept analysis were used. Results reveal conceptual components (antecedents, attributes and outcomes) which are delineated to create a theoretical definition of perinatal bereavement. The concept is epistemologically immature, with few explicit definitions to describe the phenomenon. Inconsistency in conceptual meaning threatens the construct validity of measurement tools for perinatal bereavement and contributes to incongruent theoretical definitions. This has implications for both nursing science (how the concept is studied and theoretically integrated) and clinical practice (timing and delivery of support interventions). Perinatal bereavement is a multifaceted global phenomenon that follows perinatal loss. Lack of conceptual clarity and lack of a clearly articulated conceptual definition impede the synthesis and translation of research findings into practice. A theoretical definition of perinatal bereavement is offered as a platform for researchers to advance the concept through research and theory development. © 2013 Blackwell Publishing Ltd.

  19. [Blood ammonia and transaminases in full term infants suffering from perinatal asphyxia].

    PubMed

    Esqué-Ruiz, M T; Figueras-Aloy, J; Salvia-Roigés, M D; Carbonell-Estrany, X

    To find hepatic markers of perinatal asphyxia. Variations in blood ammonia during the first week of life and in transaminase in serum during the first 48 hours were analysed in four groups of newly born infants (NBI): Group I or control, in which 65 NBI were included, with suspected unconfirmed infection and no other pathologies; Group II, made up of 15 NBI with loss of foetal well being (LFW) with no posterior neurological clinical features; Group III, consisting of 27 NBI with LFW criteria and mild hypoxic ischemic encephalopathy (HIE); and Group IV, with 25 NBI with LFW criteria and mild HIE according to Amiel s criteria. The average blood ammonia values in full term infants remain steady during the first week of life (87.66 21.69 mg/dL), as occurs in infants with LFW but without HIE (89.08 24.69 mg/dL) and in those with mild HIE (89.08 20.75 mg/dL). In moderate HIE, the blood ammonia level rises until the third day (108.55 7.04 mg/dL) and then drops back to the initial values (p= 0.0045). When grouped by days, these values show significant differences (p= 0.04), with higher values in Group IV. The NBI with HIE presented higher levels of transaminases, especially of AST (GOT) (p= 0.000001), and this increase is proportional to its gravity. No relation was found between values of blood ammonia and transaminases. Both blood ammonia and transaminases can be considered to be perinatal asphyxia markers.

  20. Higher Grades and Repeated Recurrence of Hepatic Encephalopathy May Be Related to High Serum Manganese Levels.

    PubMed

    Kobtan, Abdelrahman A; El-Kalla, Ferial S; Soliman, Hanan H; Zakaria, Soha S; Goda, Mohamed A

    2016-02-01

    Hepatic encephalopathy is a serious complication of liver failure. Until now, the precise pathophysiologic mechanisms are not fully determined. It has been demonstrated that manganese plays an important role in the pathogenesis of hepatic encephalopathy. Therefore, we studied manganese levels in serum of cirrhotic patients with hepatic encephalopathy in relation to grading and recurrence of hepatic encephalopathy. One hundred persons were enrolled in the study, 80 cirrhotic patients with or without encephalopathy and 20 healthy controls. Hepatic encephalopathy was diagnosed clinically and by laboratory findings. Serum manganese levels were measured in all participants. The grading of hepatic encephalopathy was significantly correlated to the severity of liver dysfunction. The mean serum manganese level was significantly higher in cirrhotic patients than in controls and in cirrhotic patients with encephalopathy than in those without encephalopathy. It was also significantly higher in patients with advanced grading of hepatic encephalopathy. Serum manganese level was positively correlated to number of recurrences of encephalopathy during a 6-month follow-up period. Serum manganese levels were able to predict recurrence of hepatic encephalopathy within 6 months following the episode. Serum manganese levels are positively correlated to the modified Child-Pugh score of cirrhosis as well as grading and number of recurrences of hepatic encephalopathy. Higher manganese levels seem to be related to worsening of the condition, and its measurement may be used as a predictor of repeated recurrences.

  1. Relationship of EEG sources of neonatal seizures to acute perinatal brain lesions seen on MRI: a pilot study.

    PubMed

    Despotovic, Ivana; Cherian, Perumpillichira J; De Vos, Maarten; Hallez, Hans; Deburchgraeve, Wouter; Govaert, Paul; Lequin, Maarten; Visser, Gerhard H; Swarte, Renate M; Vansteenkiste, Ewout; Van Huffel, Sabine; Philips, Wilfried

    2013-10-01

    Even though it is known that neonatal seizures are associated with acute brain lesions, the relationship of electroencephalographic (EEG) seizures to acute perinatal brain lesions visible on magnetic resonance imaging (MRI) has not been objectively studied. EEG source localization is successfully used for this purpose in adults, but it has not been sufficiently explored in neonates. Therefore, we developed an integrated method for ictal EEG dipole source localization based on a realistic head model to investigate the utility of EEG source imaging in neonates with postasphyxial seizures. We describe here our method and compare the dipole seizure localization results with acute perinatal lesions seen on brain MRI in 10 full-term infants with neonatal encephalopathy. Through experimental studies, we also explore the sensitivity of our method to the electrode positioning errors and the variations in neonatal skull geometry and conductivity. The localization results of 45 focal seizures from 10 neonates are compared with the visual analysis of EEG and MRI data, scored by expert physicians. In 9 of 10 neonates, dipole locations showed good relationship with MRI lesions and clinical data. Our experimental results also suggest that the variations in the used values for skull conductivity or thickness have little effect on the dipole localization, whereas inaccurate electrode positioning can reduce the accuracy of source estimates. The performance of our fused method indicates that ictal EEG source imaging is feasible in neonates and with further validation studies, this technique can become a useful diagnostic tool.

  2. Effect of high-dose phenobarbital on oxidative stress in perinatal asphyxia: an open label randomized controlled trial.

    PubMed

    Gathwala, Geeta; Marwah, Ashish; Gahlaut, Veena; Marwah, Poonam

    2011-08-01

    To evaluate the effect of high dose phenobarbital on lipid peroxidation and antioxidant enzymes in perinatal asphyxia. Open label, Randomized controlled trial. Neonatal intensive care unit of a tertiary care teaching hospital. 72 full term inborn neonates with severe birth asphyxia. Neonates were randomized to Study (phenobarbital) group and Control group. The infants in the study group received phenobarbital infusion (40 mg/kg) within first two hours of life while babies in the control group did not receive any phenobarbital. Rest of the management in both the groups was as per the unit protocol for the management of hypoxic ischemic encephalopathy. A cerebrospinal fluid examination was done at 12 ± 2 hours of life to determine the levels of superoxide dismutase, glutathione peroxidise and malonyldialdehyde. 60 neonates were followed up at 1 month of age when a detailed neurological examination was done. Four neonates in the study group and six neonates in the control group died during the study. Two neonates in the study group were lost to follow up. The cerebrospinal fluid lipid peroxides and antioxidant enzymes were significantly lower in the phenobarbital group as compared to the control group. The neurological outcome at one month follow up was found to be comparable between the two groups. Phenobarbital (40 mg/kg) given in the first two hours of life in term neonates with perinatal asphyxia led to a decrease in CSF levels of lipid peroxides and antioxidant enzymes at 12 ± 2 hours of life.

  3. Nicotine Dependence Measures for Perinatal Women.

    PubMed

    Yang, Irene; Hall, Lynne A

    2016-03-02

    This integrative review provides an overview of nicotine dependence measures used with perinatal women and an evaluation of their psychometric properties. Fifty-five articles that met inclusion and exclusion criteria were identified from five different databases. Most of the studies used the Fagerström Test for Nicotine Dependence (FTND). Other approaches included diagnostic tests, the Wisconsin Inventory of Smoking Dependence Motives (WISDM), the Tobacco Dependence Screener, and single-item measures. This review indicated that the FTND may not be the best option for measuring nicotine dependence in this population. The WISDM is a newer instrument that has excellent psychometric properties and captures nonnicotinic dimensions of nicotine dependence relevant to women. Future research is needed to assess its reliability in the perinatal population. Other recommendations from this review include the use of biomarker validation, thorough psychometric reporting on nicotine dependence instruments, and the use of multiple instruments to maximize comparability between nicotine dependence instruments.

  4. Autonomy and advocacy in perinatal nursing practice.

    PubMed

    Simmonds, Anne H

    2008-05-01

    Advocacy has been positioned as an ideal within the practice of nursing, with national guidelines and professional standards obliging nurses to respect patients' autonomous choices and to act as their advocates. However, the meaning of advocacy and autonomy is not well defined or understood, leading to uncertainty regarding what is required, expected and feasible for nurses in clinical practice. In this article, a feminist ethics perspective is used to examine how moral responsibilities are enacted in the perinatal nurse-patient relationship and to explore the interaction between the various threads that influence, and are in turn affected by, this relationship. This perspective allows for consideration of contextual and relational factors that impact on the way perinatal nursing care is given and received, and provides a framework for exploring the ways in which patient autonomy, advocacy and choice are experienced by childbearing women and their nurses during labour and birth.

  5. Highly improved perinatal states in Japan.

    PubMed

    Maeda, Kazuo

    2014-08-01

    To report on improved perinatal states in Japan, governmental and United Nations Children's Fund reports were analyzed. Initial maternal mortality, which was 409.8 in 1899, decreased to 4.1 in 2010, with a reduction rate of 409.8/4.1 (102.4) in 111 years: 2.5 in the initial 50 years in home delivery and 39.3 in the later 60 years in hospital births. The difference between 2.5 versus 39.3 was attributed to the medicine and medical care provided in hospital births. The total reduction of neonatal mortality was 77.9/1.1 (70.8), and the rate in the initial 50 versus later 60 years was 2.8/25. Also, there was a big difference after introduction of extensive neonatal care. Virtual perinatal mortality after 22 weeks was estimated to be 428 in 1000 births in 1900 (i.e. those infants born at 22-28 weeks were unlikely to survive at that time), while the perinatal mortality was reported to be 22 weeks or more in 1979 (i.e. premature babies born at ≥22 weeks survived in 1979 because of the improved neonatal care). Actually, 60% of premature infants of 400-500 g survived in the neonatal intensive care unit. In a recent report, 36% of infants born at 22 weeks survived to 3 years. Although there were neurodevelopmental impairments, outcomes were improved. In conclusion, perinatal states have remarkably improved in Japan.

  6. Perinatal Risk Factors for Mild Motor Disability

    ERIC Educational Resources Information Center

    Hands, Beth; Kendall, Garth; Larkin, Dawne; Parker, Helen

    2009-01-01

    The aetiology of mild motor disability (MMD) is a complex issue and as yet is poorly understood. The aim of this study was to identify the prevalence of perinatal risk factors in a cohort of 10-year-old boys and girls with (n = 362) and without (n = 1193) MMD. Among the males with MMD there was a higher prevalence of postpartum haemorrhage,…

  7. Specific ultrasonographic features of perinatal lethal hypophosphatasia.

    PubMed

    Zankl, Andreas; Mornet, Etienne; Wong, Shell

    2008-05-01

    Prenatal diagnosis of perinatal lethal hypophosphatasia (PL-HPH) by ultrasonography is difficult as PL-HPH must be differentiated from other skeletal dysplasias with short long bones and poor mineralization of the skeleton, such as osteogenesis imperfecta type II and achondrogenesis/hypochondrogenesis. Here we present a case of molecularly confirmed PL-HPH and illustrate specific ultrasonographic findings that help to distinguish PL-HPH from similar conditions. (c) 2008 Wiley-Liss, Inc.

  8. Predicting grief intensity after recent perinatal loss.

    PubMed

    Hutti, Marianne H; Myers, John; Hall, Lynne A; Polivka, Barbara J; White, Susan; Hill, Janice; Kloenne, Elizabeth; Hayden, Jaclyn; Grisanti, Meredith McGrew

    2017-08-02

    The Perinatal Grief Intensity Scale (PGIS) was developed for clinical use to identify and predict intense grief and need for follow-up after perinatal loss. This study evaluates the validity of the PGIS via its ability to predict future intense grief based on a PGIS score obtained early after a loss. A prospective observational study was conducted with 103 international, English-speaking women recruited at hospital discharge or via the internet who experienced a miscarriage, stillbirth, or neonatal death within the previous 8weeks. Survey data were collected at baseline using the PGIS and the Perinatal Grief Scale (PGS). Follow-up data on the PGS were obtained 3months later. Data analysis included descriptive statistics, Cronbach's alpha, receiver operating characteristic curve analysis, and confirmatory factor analysis. Cronbach's alphas were ≥0.70 for both instruments. PGIS factor analysis yielded three factors as predicted, explaining 57.7% of the variance. The optimal cutoff identified for the PGIS was 3.535. No difference was found when the ability of the PGIS to identify intense grief was compared to the PGS (p=0.754). The PGIS was not inferior to the PGS (AUC=0.78, 95% CI 0.68-0.88, p<0.001) in predicting intense grief at the follow-up. A PGIS score≥3.53 at baseline was associated with increased grief intensity at Time 2 (PGS: OR=1.97, 95% CI 1.59-2.34, p<0.001). The PGIS is comparable to the PGS, has a lower response burden, and can reliably and validly predict women who may experience future intense grief associated with perinatal loss. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Perinatal Risk Factors for Mild Motor Disability

    ERIC Educational Resources Information Center

    Hands, Beth; Kendall, Garth; Larkin, Dawne; Parker, Helen

    2009-01-01

    The aetiology of mild motor disability (MMD) is a complex issue and as yet is poorly understood. The aim of this study was to identify the prevalence of perinatal risk factors in a cohort of 10-year-old boys and girls with (n = 362) and without (n = 1193) MMD. Among the males with MMD there was a higher prevalence of postpartum haemorrhage,…

  10. Racial discrimination and perinatal sleep quality.

    PubMed

    Francis, Brittney; Klebanoff, Mark; Oza-Frank, Reena

    2017-08-01

    This research examined the association between perceived everyday racial discrimination, as a psychosocial stressor, and perinatal sleep quality. Cross-sectional (N=640) and longitudinal associations (N=133) between everyday experiences of discrimination and sleep quality were examined using a pregnancy and postpartum data registry. We studied a sample of 640 unique women from the Perinatal Research Repository (PRR), a longitudinal study of mothers, fathers, and babies recruited from Nationwide Children's Hospital and The Ohio State University in Columbus, Ohio. Discrimination and sleep quality were assessed using the Experiences of Discrimination Scale and the Pittsburgh Sleep Quality Index, respectively. Overall, everyday discrimination was associated with poorer global sleep quality and all but three sleep sub-measures of the PSQI cross-sectionally, but not longitudinally. When stratified, the adverse effects of everyday discrimination varied by race and perinatal time period. Increases in everyday discrimination were independently associated with poorer sleep initiation, poorer sleep maintenance and poorer daytime dysfunction. Findings suggest that the immediate stressors of everyday racial discrimination were independently associated with poorer sleep quality among pregnant women cross-sectionally. Poorer sleep quality has been associated with numerous adverse perinatal outcomes and this association may be important in understanding racial discrimination as a risk factor. Our failure to identify a longitudinal association makes the direction of causation uncertain, however. Further longitudinal studies are necessary to clarify the association, given the potential importance of poor sleep quality in the pathogenesis of pregnancy complications. Copyright © 2017 National Sleep Foundation. Published by Elsevier Inc. All rights reserved.

  11. Perinatal mortality attributable to complications of childbirth in Matlab, Bangladesh.

    PubMed Central

    Kusiako, T.; Ronsmans, C.; Van der Paal, L.

    2000-01-01

    Very few population-based studies of perinatal mortality in developing countries have examined the role of intrapartum risk factors. In the present study, the proportion of perinatal deaths that are attributable to complications during childbirth in Matlab, Bangladesh, was assessed using community-based data from a home-based programme led by professional midwives between 1987 and 1993. Complications during labour and delivery--such as prolonged or obstructed labour, abnormal fetal position, and hypertensive diseases of pregnancy--increased the risk of perinatal mortality fivefold and accounted for 30% of perinatal deaths. Premature labour, which occurred in 20% of pregnancies, accounted for 27% of perinatal mortality. Better care by qualified staff during delivery and improved care of newborns should substantially reduce perinatal mortality in this study population. PMID:10859856

  12. Sleep and perinatal mood disorders: a critical review

    PubMed Central

    Ross, Lori E.; Murray, Brian J.; Steiner, Meir

    2005-01-01

    Pregnancy and the postpartum period are recognized as times of vulnerability to mood disorders, including postpartum depression and psychosis. Recently, changes in sleep physiology and sleep deprivation have been proposed as having roles in perinatal psychiatric disorders. In this article we review what is known about changes in sleep physiology and behaviour during the perinatal period, with a focus on the relations between sleep and postpartum “blues,” depression and psychosis and on sleep-based interventions for the treatment and prevention of perinatal mood disorders. The interaction between sleep and perinatal mood disorders is significant, but evidence-based research in this field is limited. Studies that measure both sleep and mood during the perinatal period, particularly those that employ objective measurement tools such as polysomnography and actigraphy, will provide important information about the causes, prevention and treatment of perinatal mood disorders. PMID:16049568

  13. Neoplasms in young dogs after perinatal irradiation

    SciTech Connect

    Benjamin, S.A.; Lee, A.C.; Angleton, G.M.; Saunders, W.J.; Miller, G.K.; Williams, J.S.; Brewster, R.D.; Long, R.I.

    1986-08-01

    For a study of the life-time effects of irradiation during development, 1,680 beagles were given single, whole-body exposures to /sup 60/Co gamma-radiation at one of three prenatal (preimplantation, embryonic, and fetal) or at one of three postnatal (neonatal, juvenile, and young adult) ages. Mean doses were 0, 0.16, or 0.83 Gy. For comparison with data on childhood cancer after prenatal irradiation, examination was made of tumors occurring in young dogs in this life-span experiment. Up to 4 years of age, 18 dogs had neoplasms diagnosed, 2 of these being in controls. Four dogs that were irradiated in the perinatal (late fetal or neonatal) period died of cancers prior to 2 years of age. This risk was of significant increase compared to the risks for other experimental groups and for the canine population in general. Overall, 71% (5 of 7) of all cancers and 56% (10 of 18) of all benign and malignant neoplasms seen in the first 4 years of life occurred in 29% (480 of 1680) of the dogs irradiated in the perinatal period. These data suggest an increased risk for neoplasia after perinatal irradiation in dogs.

  14. Incarceration, maternal hardship, and perinatal health behaviors.

    PubMed

    Dumont, Dora M; Wildeman, Christopher; Lee, Hedwig; Gjelsvik, Annie; Valera, Pamela; Clarke, Jennifer G

    2014-11-01

    Parental incarceration is associated with mental and physical health problems in children, yet little research directly tests mechanisms through which parental incarceration could imperil child health. We hypothesized that the incarceration of a woman or her romantic partner in the year before birth constituted an additional hardship for already-disadvantaged women, and that these additionally vulnerable women were less likely to engage in positive perinatal health behaviors important to infant and early childhood development. We analyzed 2006-2010 data from the Pregnancy Risk Assessment and Monitoring System to assess the association between incarceration in the year prior to the birth of a child and perinatal maternal hardships and behaviors. Women reporting incarceration of themselves or their partners in the year before birth of a child had .86 the odds (95 % CI .78-.95) of beginning prenatal care in the first trimester compared to women not reporting incarceration. They were nearly twice as likely to report partner abuse and were significantly more likely to rely on WIC and/or Medicaid for assistance during pregnancy. These associations persist after controlling for socioeconomic measures and other stressors, including homelessness and job loss. Incarceration of a woman or her partner in the year before birth is associated with higher odds of maternal hardship and poorer perinatal health behaviors. The unprecedented scale of incarceration in the US simultaneously presents an underutilized public health opportunity and constitutes a social determinant of health that may contribute to disparities in early childhood development.

  15. Action plan to reduce perinatal mortality.

    PubMed

    Bhakoo, O N; Kumar, R

    1990-01-01

    The government of India has set a goal of reducing perinatal mortality from its current rate of 48/1000 to 30-35/1000 by the year 2000. Perinatal deaths result from maternal malnutrition, inadequate prenatal care, complications of delivery, and infections in the postpartum period. Since reductions in perinatal mortality require attention to social, economic, and behavioral factors, as well as improvements in the health care delivery system, a comprehensive strategy is required. Social measures, such as raising the age at marriage to 18 years for females, improving the nutritional status of adolescent girls, reducing the strenuousness of work during pregnancy, improving female literacy, raising women's status in the society and thus in the family, and poverty alleviation programs, would all help eliminate the extent of complications of pregnancy. Measures required to enhance infant survival include improved prenatal care, prenatal tetanus toxoid immunization, use of sterile disposable cord care kits, the provision of mucus extractors and resuscitation materials to birth attendants, the creation of neonatal care units in health facilities, and more efficient referral of high-risk newborns and mothers. Since 90% of births in rural India take place at home priority must be given to training traditional birth attendants in the identification of high risk factors during pregnancy, delivery, and the newborn period.

  16. Perinatal risks of untreated depression during pregnancy.

    PubMed

    Bonari, Lori; Pinto, Natasha; Ahn, Eric; Einarson, Adrienne; Steiner, Meir; Koren, Gideon

    2004-11-01

    To review the literature on the perinatal risks involved in untreated depression during pregnancy. We searched Medline and medical texts for all studies pertaining to this area up to the end of April 2003. Key phrases entered were depression and pregnancy, depression and pregnancy outcome, and depression and untreated pregnancy. We did not include bipolar depression. While there is wide variability in reported effects, untreated depression during pregnancy appears to carry substantial perinatal risks. These may be direct risks to the fetus and infant or risks secondary to unhealthy maternal behaviours arising from the depression. Recent human data suggest that untreated postpartum depression, not treatment with antidepressants in pregnancy, results in adverse perinatal outcome. The biological dysregulation caused by gestational depression has not received appropriate attention: most studies focus on the potential but unproven risks of psychotropic medication. No in-depth discussion of the role of psychotherapy is available. Because they are not aware of the potentially catastrophic outcome of untreated maternal depression, this imbalance may lead women suffering from depression to fear teratogenic effects and refuse treatment.

  17. Cerebral palsy after perinatal arterial ischemic stroke.

    PubMed

    Golomb, Meredith R; Garg, Bhuwan P; Saha, Chandan; Azzouz, Faouzi; Williams, Linda S

    2008-03-01

    The frequency of cerebral palsy, degree of disability, and predictors of disability were assessed in children in a perinatal arterial stroke database. Risk factors were assessed at the univariate level using the Pearson chi(2) and Fisher exact test and at the multivariate level using logistic regression analysis. Seventy-six of 111 children with perinatal stroke (68%) had cerebral palsy, most commonly hemiplegic (66/76; 87%). Multivariate analysis of the entire cohort showed both delayed presentation (OR,9.96; 95% CI, 3.10-32.02) and male sex (OR, 2.55; 95% CI, 1.03-6.32) were associated with cerebral palsy. In subgroup multivariate analyses: in children with neonatal presentation, bilateral infarcts were associated with triplegia or quadriplegia (OR, 5.33; 95% CI, 1.28-22.27); in children with unilateral middle cerebral artery infarcts, delayed presentation (OR, 10.60; 95% CI, 2.28-72.92) and large-branch infarction (OR, 8.78; 95% CI, 2.18-43.67) were associated with cerebral palsy. These data will aid physicians in planning long-term rehabilitative care for children with perinatal stroke.

  18. Mechanisms of perinatal arterial ischemic stroke

    PubMed Central

    Fernández-López, David; Natarajan, Niranjana; Ashwal, Stephen; Vexler, Zinaida S

    2014-01-01

    The incidence of perinatal stroke is high, similar to that in the elderly, and produces a significant morbidity and severe long-term neurologic and cognitive deficits, including cerebral palsy, epilepsy, neuropsychological impairments, and behavioral disorders. Emerging clinical data and data from experimental models of cerebral ischemia in neonatal rodents have shown that the pathophysiology of perinatal brain damage is multifactorial. These studies have revealed that, far from just being a smaller version of the adult brain, the neonatal brain is unique with a very particular and age-dependent responsiveness to hypoxia–ischemia and focal arterial stroke. In this review, we discuss fundamental clinical aspects of perinatal stroke as well as some of the most recent and relevant findings regarding the susceptibility of specific brain cell populations to injury, the dynamics and the mechanisms of neuronal cell death in injured neonates, the responses of neonatal blood–brain barrier to stroke in relation to systemic and local inflammation, and the long-term effects of stroke on angiogenesis and neurogenesis. Finally, we address translational strategies currently being considered for neonatal stroke as well as treatments that might effectively enhance repair later after injury. PMID:24667913

  19. Obstetric and perinatal outcome of teenage pregnancy.

    PubMed

    Suwal, A

    2012-01-01

    Adolescents are at higher risk during childbirth than women between 20 to 25 years. Adolescent childbearing initiates a syndrome of failure: failure to complete one's education; failure in limiting family size; failure to establish a vocation and become independent. This study was done to find out the obstetric and perinatal outcome of teenage pregnancy along with factors contributing to teenage pregnancy. A prospective, cross sectional study was carried out in College of Medical Sciences Teaching Hospital (CMSTH), Bharatpur during the period for two years from September 2008 to August 2010. Pregnant girls ≤19 years admitted to labour ward were taken for the study. Cases planned for abortion and MTP were also taken. One hundred cases of pregnant teenagers were admitted in CMSTH during a period of two years. Incidence was 6.85%. In our study, most of the teenagers were unbooked, from low socioeconomic status and with no or inadequate education. They had little knowledge about contraception and less number of teenagers used temporary means of contraception. Because of our social custom of early marriage, most of the teenage mothers were married. All these factors were correlated with teenage pregnancy in present study. This study failed to show any statistically significant difference in the incidence of anaemia, LBW babies, preterm delivery, hypertensive disorder of pregnancy, mode of delivery in different ages of teenage mothers. However, there was significant difference in the incidence of perinatal death in different ages of teenage mothers indicating that perinatal deaths were more in younger teenagers.

  20. Untreated perinatal paternal depression: Effects on offspring.

    PubMed

    Gentile, Salvatore; Fusco, Maria Luigia

    2017-03-02

    Transition to parenthood represents an important life event which increases vulnerability to psychological disorders. Aim of this article is to analyze all studies which investigated the effects of untreated perinatal paternal depression in offspring. We searched pertinent, peer-reviewed articles published in English (January 1980 to April 2016) on MEDLINE, PsycINFO, and Science.gov. Twenty-three studies met the inclusion criteria. Most of the reviewed studies suffer from methodological limitations, including the small sample, the lack of a structured psychiatric diagnosis, and inclusion bias. Despite such limitations, paternal depression seems to be associated with an increased risk of developmental and behavioural problems and even psychiatric disorders in offspring. In particular, in infants and toddlers such problems vary from increased crying to hyperactivity and conduct problems to psychological and developmental impairment, and poor social outcomes. School-age children of depressed fathers have a doubled risk for suffering from specific psychiatric disorders. Hence, facilitating access to vigorous and evidence based treatments is a public health opportunity for improving the quality of life of depressed parents and their children. Evidences emerging from this review actually suggest that the traditional gender-focused approach to perinatal mood disorders should be completed by a family-centred approach, in order to improve the effectiveness of perinatal mental health programs.