Wassink, Guido; Gunn, Eleanor R.; Drury, Paul P.; Bennet, Laura; Gunn, Alistair J.
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
Borron, Stephen W; Bebarta, Vikhyat S
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.
Graham, Ernest M.; Burd, Irina; Everett, Allen D.; Northington, Frances J.
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
Mitchell, Charles D.
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…
Patel, Kunjal; Ming, Xue; Williams, Paige L.; Robertson, Kevin R.; Oleske, James M.; Seage, George R.
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
Distefano, Giuseppe; Praticò, Andrea D
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.
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
Azzopardi, Denis; Robertson, Nicola J; Kapetanakis, Andrew; Griffiths, James; Rennie, Janet M; Mathieson, Sean R; Edwards, A David
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.
van Schie, Petra Em; Becher, Jules G.; Dallmeijer, Annet J.; Barkhof, Frederik; van Weissenbruch, Mirjam M.; Vermeulen, R. Jeroen
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…
Hendaus, Mohamed A; Jomha, Fatima A; Alhammadi, Ahmed H
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
Saha, D; Ali, M A; Haque, M A; Ahmed, M S; Sutradhar, P K; Latif, T; Sarkar, D; Husain, F
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
Huang, Yuejun; Lai, Huihong; Xu, Hongwu; Wu, Weizhao; Lai, Xiulan; Ho, Guyu; Chen, Yunbin; Ma, Lian
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.
Shaikh, Henna; Boudes, Elodie; Khoja, Zehra; Shevell, Michael; Wintermark, Pia
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
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
Mandujano-Valdés, Mario Antonio; Sánchez-Pérez, María del Carmen
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.
Chen, H C; Marsharani, U
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.
Gruskin, A B; Baluarte, H J; Cote, M L; Elfenbein, I B
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.
Howard, Matthew O; Hall, Martin T; Edwards, Jeffrey D; Vaughn, Michael G; Perron, Brian E; Winecker, Ruth E
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.
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…
Kim, Jae Woo
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
Myers, Wade C; Bukhanovskiy, Alexandr; Justen, Elle; Morton, Robert J; Tilley, John; Adams, Kenneth; Vandagriff, Virgil L; Hazelwood, Robert R
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.
Lemmon, Monica E; Donohue, Pamela K; Parkinson, Charlamaine; Northington, Frances J; Boss, Renee D
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.
Djelantik, M; Bloemkolk, D; Tijdink, J
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.
Chalak, Lina F.; Tarumi, Takashi; Zhang, Rong
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
Turkel, S B; Diehl, E J; Richmond, J A
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
Bingham, Peter; Edwards, Erika M.; Horbar, Jeffrey D.; Kenny, Michael J.; Inder, Terrie; Pfister, Robert H.; Raju, Tonse; Soll, Roger F.
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
Jelin, Angie C.; Thiet, Mari-Paule
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
Kletkiewicz, Hanna; Nowakowska, Anna; Siejka, Agnieszka; Mila-Kierzenkowska, Celestyna; Woźniak, Alina; Caputa, Michał; Rogalska, Justyna
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.
Seibel, Brad A
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.
Fatemi, Ali; Wilson, Mary Ann; Johnston, Michael V.
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
Harris, C S; Baker, S P; Smith, G A; Harris, R M
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.
Pearce, S; Taylor, B
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.
Leypoldt, Frank; Armangue, Thaís; Dalmau, Josep
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
Suruda, A; Agnew, J
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
Sharma, Manisha; Dolker, Stanzin; Kothapalli, Sharada
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
Petruk, J; Shields, E; Cummings, G E; Francescutti, L H
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
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...
Ahearne, Caroline E; Boylan, Geraldine B; Murray, Deirdre M
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
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
Laish, Ido; Ben Ari, Ziv
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.
Sachse, Daniel; Solevåg, Anne Lee; Berg, Jens Petter; Nakstad, Britt
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
Cichoż-Lach, Halina; Michalak, Agata
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.
Kaczmarczyk, Aleksandra; Patalong-Ogiewa, M; Krzystanek, E
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.
Zamora Nava, Luis Eduardo; Torre Delgadillo, Aldo
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.
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
Esqué Ruiz, M; Figueras Aloy, J; García Alix, A; Alomar Ribes, A; Blanco Bravo, D; Ferández Lorenzo, J R
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.
Samuels, R.; Chadwick, D. D.
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…
Stolen, Megan; St. Leger, Judy; Durden, Wendy Noke; Mazza, Teresa; Nilson, Erika
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
Stolen, Megan; St Leger, Judy; Durden, Wendy Noke; Mazza, Teresa; Nilson, Erika
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.
Mohnot, D; Snead, O C; Benton, J W
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.
Van Tilburg, Christopher
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.
Sharma, Deepak; Choudhary, Mukesh; Lamba, Mamta; Shastri, Sweta
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
Waghray, Abhijeet; Waghray, Nisheet; Mullen, Kevin
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.
Verma, Rajesh; Praharaj, Heramba Narayan
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.
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...
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
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
Silver, B E; Bean, C S
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.
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.
Karthiga, Vikneswari; Kommu, Peter Prasanth Kumar; Krishnan, Lalitha
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
Chandrasekharan, Praveen Kumar; Rawat, Munmun; Nair, Jayasree; Gugino, Sylvia F.; Koenigschnekt, Carmon; Swartz, Daniel D.; Vali, Payam; Mathew, Bobby; Lakshminrusimha, Satyan
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
Geng, Longwu; Jiang, Haifeng; Tong, Guangxiang; Xu, Wei
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.
Geng, Longwu; Jiang, Haifeng; Tong, Guangxiang; Xu, Wei
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.
Samnakay, Naeem; Tudehope, David; Walker, Rosslyn
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.
Lado, Fred A; Rubboli, Guido; Capovilla, Giuseppe; Capovilla, Pippo; Avanzini, Giuliano; Moshé, Solomon L
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.
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
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
Price, Raymond S; Kasner, Scott E
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.
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.
Portman, R J; Carter, B S; Gaylord, M S; Murphy, M G; Thieme, R E; Merenstein, G B
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.
Hagberg, Henrik; Mallard, Carina; Ferriero, Donna M.; Vannucci, Susan J.; Levison, Steven W.; Vexler, Zinaida S.; Gressens, Pierre
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
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
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
Liu, Qing; Zhu, Hai-Yan; Liu, Fang; Ding, Zi-Yuan
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.
Verma, Rajesh; Praharaj, Heramba Narayan
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
Carbonell, X; Figueras, J; Salvia, M D; Esque, M T; Delgado, M P; Jimenez, R
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.
Byard, R W; Hucker, S J; Hazelwood, R R
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.
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...
Gofton, Teneille E; Young, G Bryan
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.
Chaudhry, Neera; Duggal, Ashish Kumar
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.
Cotena, Simona; Piazza, Ornella
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.
Yi, Juneyoung; Padalino, David J; Chin, Lawrence S; Montenegro, Philip; Cantu, Robert C
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.
Algahtani, Abdulhadi; Aldarmahi, Ahmad; Hmoud, Mohammed; Marzuk, Yousef; Shirah, Bader
Objectives: Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome characterized by headache, altered mental status, seizures, or loss of vision. In this study, we report the largest series of PRES coming from Saudi Arabia and explore the etiology, clinical presentation, and outcome. We also report new imaging findings associated with this condition. Methods: We performed a retrospective study of all cases of PRES admitted to King Abdulaziz Medical City, Jeddah, Saudi Arabia, between the years 2005 and 2015. A neurologist reviewed all charts and analyzed the clinical presentations, etiological factors, and outcomes, and a neuroradiologist reviewed the imaging studies. Only patients with clinical and imaging features consistent with PRES were included in the study. Results: We collected 31 patients who had clinical and radiological features consistent with PRES. Females were more affected than males (18 females and 13 males), and patients’ age ranged from 6 to 95 years, with a mean of 38.3 years. Patients were treated by removing the precipitating causes and treating the underlying conditions. Resolution of neurologic signs occurred within 2 to 3 weeks in all patients. Conclusion: In our opinion, PRES itself is usually a benign condition with complete recovery if the condition is recognized early and managed appropriately. Although clinical signs are nonspecific, the constellation of symptoms including headache, visual problems, seizures, and altered level of consciousness should suggest the possibility of PRES, especially in high-risk group. Abnormalities on magnetic resonance imaging are often characteristic and may be the first clue to the diagnosis. PMID:28042366
Maddison, J E
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.
Green, Rebecca; Scott, L Keith; Minagar, Alireza; Conrad, Steven
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.
Whitaker, Claudia; Kavanaugh, Karen; Klima, Carrie
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.
Whitaker, Claudia; Kavanaugh, Karen; Klima, Carrie
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
van Roosmalen, J
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).
Kaplan, Peter W; Sutter, Raoul
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.
Patel, Kinjal Prahaladbhai; Makadia, Mayur Goradhanbhai; Patel, Vishwal Indravardan; Nilayangode, Haridas Neelakandan
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
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.
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
Kale, Alok; Joshi, Priscilla; Kelkar, A B
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
Larguía, A Miguel; González, María Aurelia; Dinerstein, Néstor Alejandro; Soto Conti, Constanza
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.).
Menke, J A; McClead, R E
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.
IJsseldijk, Lonneke L.; Leopold, Mardik F.; Bravo Rebolledo, Elisa L.; Deaville, Rob; Haelters, Jan; IJzer, Jooske; Jepson, Paul D.; Gröne, Andrea
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
Angeles, Danilyn M; Ashwal, Stephen; Wycliffe, Nathaniel D; Ebner, Charlotte; Fayard, Elba; Sowers, Lawrence; Holshouser, Barbara A
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.
Mavrogiorgou, P; Illes, F; Juckel, G
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.
Hirai, Nozomi; Yoshimaru, Daisuke; Moriyama, Yoko; Honda, Takafumi; Yasukawa, Kumi; Takanashi, Jun-Ichi
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.
Gopagondanahalli, Krishna Revanna; Li, Jingang; Fahey, Michael C.; Hunt, Rod W.; Jenkin, Graham; Miller, Suzanne L.; Malhotra, Atul
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
Soós, Zsuzsanna; Salamon, Mónika; Oláh, Roland; Czégeni, Anna; Salamon, Ferenc; Folyovich, András; Winkler, Gábor
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.
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.
Pearl, Phillip L.
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
Poli, D; Solarino, B; Di Vella, G; Tattoli, L; Strisciullo, G; Goldoni, M; Mutti, A; Gagliano-Candela, R
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).
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
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.
Quattrocchi, Carlo Cosimo; Fariello, Giuseppe; Longo, Daniela
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
Iadevaia, Maddalena Diana; Prete, Anna Del; Cesaro, Claudia; Gaeta, Laura; Zulli, Claudio; Loguercio, Carmelina
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
Erol, Ilknur; Saygi, Semra; Alehan, Füsun
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.
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
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.
Mallewa, Macpherson; Birbeck, Gretchen L
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.
Chamorro Fernández, A J; Marcos Martín, M; Laso Guzmán, F J
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?
López, Jorge; Fernández, Sarah N.; González, Rafael; Solana, María J.; Urbano, Javier; López-Herce, Jesús
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
Kang, Woo-Hyuk; Na, Ju-Young; Kim, Meyung-Kug; Yoo, Bong-Goo
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.
Mizock, B A
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.
Nardone, Raffaele; Taylor, Alexandra C; Höller, Yvonne; Brigo, Francesco; Lochner, Piergiorgio; Trinka, Eugen
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.
Smith, Gordon C S
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.
Fridinger, S E; Alper, Gulay
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.
Chamorro, Antonio J; Marcos-Martin, Miguel; Martin-Polo, Jorge; Garcia-Diez, Luis Carlos; Luna, Guillermo
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.
Nar Senol, Pelin; Bican Demir, Aylin; Bora, Ibrahim; Bakar, Mustafa
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
Byard, Roger W; Simpson, Ellie; Gilbert, John D
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.
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
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.
Guilheri, Juliana; Andronikof, Anne; Yazigi, Latife
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.
Vytautas, Basys; Nijolė, Drazdienė; Nijolė, Vezbergienė; Jelena, Isakova
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.
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...
Martínez-Pérez, R; Paredes, I; Munarriz, P M; Paredes, B; Alén, J F
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.
Chang, Jan-Shun; Chang, Tien-Chun
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.
Hindawy, A; Gouda, A; El-Ayyadi, A; Megahed, H; Bazaraa, H
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).
Kotha, V.K.; De Souza, A.
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
Khavinson, V Kh; Morozov, V G; Rybnikov, V Iu; Zakutskiĭ, N G
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.
... muscle or group of muscles), nystagmus (rapid, involuntary eye movement), tremor, muscle atrophy and weakness, dementia, seizures, and ... muscle or group of muscles), nystagmus (rapid, involuntary eye movement), tremor, muscle atrophy and weakness, dementia, seizures, and ...
Garvie, Patricia A.; Zeldow, Bret; Malee, Kathleen; Nichols, Sharon L.; Smith, Renee A.; Wilkins, Megan L.; Williams, Paige L.
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
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.
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
Kim, Tae Eun; Lee, Eun Ja; Young, Jeong Bo; Shin, Dong Jae; Kim, Ji Hoon
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.
Brausch, Amy M.; Decker, Kristina M.; Hadley, Andrea G.
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…
Vreeman, Rachel C; Scanlon, Michael L; McHenry, Megan S; Nyandiko, Winstone M
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
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…
Rodríguez, Charo; des Rivières-Pigeon, Catherine
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
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
Kennedy, Holly Powell
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
Henry, Dana; Gonzalez, Juan M; Harris, Ian, S.; Sparks, Teresa; Killion, Molly; Thiet, Mari-Paule; Bianco, Katherine
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
Mrelashvili, Anna; Bonifacio, Sonia L.; Rogers, Elizabeth E.; Shimotake, Thomas K.; Glass, Hannah C.
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
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…
Mocellin, Ramon; Walterfang, Mark; Velakoulis, Dennis
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
Mannix, Rebekah; Zafonte, Ross; Pascual-Leone, Alvaro
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
Meehan, William; Mannix, Rebekah; Zafonte, Ross; Pascual-Leone, Alvaro
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.
Sergeeva, Olga A
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.
Riggio, Oliviero; Ridola, Lorenzo; Pasquale, Chiara
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.
Fraile, Pilar; Cacharro, Luis Maria; Garcia-Cosmes, Pedro; Rosado, Consolacion; Tabernero, Jose Matias
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.
Saulle, Michael; Greenwald, Brian D.
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
Quan, Li; Ishikawa, Takaki; Michiue, Tomomi; Li, Dong-Ri; Zhao, Dong; Zhu, Bao-Li; Maeda, Hitoshi
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.
Salazar, R; Mehta, C; Zaher, N; Miller, D
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.
Matic, Vladimir; Cherian, Perumpillichira J.; Koolen, Ninah; Naulaers, Gunnar; Swarte, Renate M.; Govaert, Paul; Van Huffel, Sabine; De Vos, Maarten
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.
Wintermark, P.; Hansen, A.; Warfield, SK.; Dukhovny, D.; Soul, JS.
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
Blanco, D; García-Alix, A; Valverde, E; Tenorio, V; Vento, M; Cabañas, F
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.
Noh, Grace J.; Asher, Y. Jane Tavyev; Graham, John M.
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
Fridley, J.; Reddy, G.; Curry, D.; Agadi, S.
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
Zampieri, Fernando Godinho; Park, Marcelo; Machado, Fabio Santana; Azevedo, Luciano Cesar Pontes
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
Berdai, Mohamed Adnane; Labib, Smael; Harandou, Mustapha
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
de Pierrepont, C; Polomeno, V
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.
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...
Stein, Thor D.; Alvarez, Victor E.; McKee, Ann C.
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
Stein, Thor D; Alvarez, Victor E; McKee, Ann C
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.
Béranger, F; Mangé, A; Solassol, J; Lehmann, S
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.
Agrawal, Swastik; Umapathy, Sridharan; Dhiman, Radha K.
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
Kural, Zekiye; Ozer, Ali Fahir
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
Chen, H; Blackburn, W R; Wertelecki, W
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.
Brande, M.C.; Zimmerman, A.M.
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.
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
Englert, Yvon; Buekens, Pierre
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
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.
Bury, Danielle; Langlois, Neil; Byard, Roger W
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.
Waldura, Jess Fogler
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.
Boutaleb, Y; Mesbahi, M; Lahlou, D; Aderdour, M
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
Helbig, Ingo; Tayoun, Abou Ahmad N.
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
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...
Baluarte, H J; Gruskin, A B; Hiner, L B; Foley, C M; Grover, W D
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.
Behura, Sushree Sangita; Swain, Sarada Prasanna
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
Yang, Ting; Zhuang, Lei; Terrando, Niccolò; Wu, Xinmin; Jonhson, Mark R; Maze, Mervyn; Ma, Daqing
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.
Khoshdel, Abolfazl; Noormohammadi, Hajar; Kheiri, Soleiman; Reisi, Roya; Nourbakhsh, Seyed Mohammad-Kazem; Panahandeh, Gholam Reza; Heidarian, Esfandiar
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
Sánchez-Illana, Ángel; Solberg, Rønnaug; Lliso, Isabel; Pankratov, Leonid; Quintás, Guillermo; Saugstad, Ola Didrik; Vento, Máximo; Kuligowski, Julia
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
Harville, Emily W; Xiong, Xu; Buekens, Pierre
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.
Ounsted, C; Roberts, J C; Gordon, M; Milligan, B
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
Barger, Mary K
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.
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.
Hill, P M
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.
Bause, George S
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.
Morgan, Marsha Y; Amodio, Piero; Cook, Nicola A; Jackson, Clive D; Kircheis, Gerald; Lauridsen, Mette M; Montagnese, Sara; Schiff, Sami; Weissenborn, Karin
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
Forray, Ariadna; Foster, Dawn
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
Vasudevan, Chakrapani; Renfrew, Mary; McGuire, William
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.
Carter, Carolyn S.
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…
AWARD NUMBER: W81XWH-14-1-0399 TITLE: Molecular & Genetic Investigation of Tau in Chronic Traumatic Encephalopathy PRINCIPAL INVESTIGATOR: John F...Include area code) October 2015 Annual Report 30 Sep 2014 - 29 Sep 2015 Molecular & Genetic Investigation of Tau in Chronic Traumatic Encephalopathy John...encephalopathy (CTE), but the underlying molecular changes remain unclear. Here, biochemical and genetic studies that deepen our understanding of the
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
Riviello, J J; Halligan, G E; Dunn, S P; Widzer, S J; Foley, C M; Breningstall, G N; Grover, W D
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.
Kim, Young Ok; Korff, Christian M; Villaluz, Mel Michel G; Suls, Arvid; Weckhuysen, Sarah; De Jonghe, Peter; Scheffer, Ingrid E
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.
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
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.
Montagna, Giacomo; Imperiali, Mauro; Agazzi, Pamela; D'Aurizio, Federica; Tozzoli, Renato; Feldt-Rasmussen, Ulla; Giovanella, Luca
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.
Noto, Antonio; Pomero, Giulia; Barberini, Luigi; Fattuoni, Claudia; Palmas, Francesco; Dalmazzo, Cristina; Delogu, Antonio; Dessì, Angelica; Fanos, Vassilios; Gancia, Paolo
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
Lewis, Judith A
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.
Marín, E; Uribe, M
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.
Morgan, M. Hilary; Bolton, C. H.; Morris, J. S.; Read, A. E.
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
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.
Hughes, Adrienne; Brown, Alisha; Valento, Matthew
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.
Hughes, Adrienne; Brown, Alisha; Valento, Matthew
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
Agadi, Satish; Quach, Michael M.
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
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.
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
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
John, Elizabeth S; Sedhom, Ramy; Dalal, Ishita; Sharma, Ranita
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.
John, Elizabeth S; Sedhom, Ramy; Dalal, Ishita; Sharma, Ranita
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
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...
Essen, Birgitta; Bodker, Birgit; Sjoberg, N-O; Gudmundsson, Saemundur; Ostergren, P-O; Langhoff-Roos, Jens
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
Pastor Montero, Sonia María; Romero Sánchez, José Manuel; Hueso Montoro, César; Lillo Crespo, Manuel; Vacas Jaén, Ana Gema; Rodríguez Tirado, María Belén
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.
Chalak, Lina F; Tian, Fenghua; Tarumi, Takashi; Zhang, Rong
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
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.
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
Nagai, T.; Kato, R.; Hasegawa, T.
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.
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...
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...
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...
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.
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…
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
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
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
Jose, Annu; Matthai, John; Paul, Sarah
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
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
Savica, Rodolfo; Rabinstein, Alejandro A; Josephs, Keith A
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.
Bandyopadhyay, Sabyasachi; Mondal, Kanchan Kumar; Das, Somnath; Gupta, Anindya; Biswas, Jaya; Bhattacharyya, Subir Kumar; Biswas, Gautam
Cortical blindness is defined as visual failure with preserved pupillary reflexes in structurally intact eyes due to bilateral lesions affecting occipital cortex. Bilateral oedema and infarction of the posterior and middle cerebral arterial territory, trauma, glioma and meningioma of the occipital cortex are the main causes of cortical blindness. Posterior reversible encephalopathy syndrome (PRES) refers to the reversible subtype of cortical blindness and is usually associated with hypertension, diabetes, immunosuppression, puerperium with or without eclampsia. Here, 3 cases of PRES with complete or partial visual recovery following treatment in 6-month follow-up are reported.
Signaté, A; Olindo, S; Chausson, N; Cassinoto, C; Edimo Nana, M; Saint Vil, M; Cabre, P; Smadja, D
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.
Solomon, Gary S; Sills, Allen
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.
Montagnese, Sara; Turco, Matteo; Amodio, Piero
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
Gauthier, Angela C; Baehring, Joachim M
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.
McKee, Ann C; Alosco, Michael L; Huber, Bertrand R
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.
Simmonds, Anne H
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.
Yang, Irene; Hall, Lynne A
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.
Hands, Beth; Kendall, Garth; Larkin, Dawne; Parker, Helen
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,…
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.
Kusiako, T.; Ronsmans, C.; Van der Paal, L.
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
Liou, Kuang-Chung; Kuo, Shu-Fan; Chen, Lu-An
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.
Bigatello, L M; Broitman, S A; Fattori, L; Di Paoli, M; Pontello, M; Bevilacqua, G; Nespoli, A
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.
Butterworth, Roger F
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.
Wu, Xiujuan; Wu, Wei; Pan, Wei; Wu, Limin; Liu, Kangding; Zhang, Hong-Liang
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
Bhakoo, O N; Kumar, R
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.
Dumont, Dora M; Wildeman, Christopher; Lee, Hedwig; Gjelsvik, Annie; Valera, Pamela; Clarke, Jennifer G
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.
Benjamin, S.A.; Lee, A.C.; Angleton, G.M.; Saunders, W.J.; Miller, G.K.; Williams, J.S.; Brewster, R.D.; Long, R.I.
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.
Gentile, Salvatore; Fusco, Maria Luigia
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.
Yun, Byung Cheol; Kim, W Ray
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.
Zhao, Pan; Zhao, Yanling; Wei, Zhenman; Chen, Jing; Yan, Lilong
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
Stawicka, Agnieszka; Zbrzeźniak, Justyna; Świderska, Aleksandra; Kilisińska, Natalia; Świderska, Magdalena; Jaroszewicz, Jerzy; Flisiak, Robert
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.
Oliver, Karen L; Lukic, Vesna; Thorne, Natalie P; Berkovic, Samuel F; Scheffer, Ingrid E; Bahlo, Melanie
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.
Blom, H J; Ferenci, P; Grimm, G; Yap, S H; Tangerman, A
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.
Kennedy, Holly Powell
This analysis was conducted to describe the concept of optimality and its appropriateness for perinatal health care. The concept was identified in 24 scientific disciplines. Across all disciplines, the universal definition of optimality is the robust, efficient, and cost-effective achievement of best possible outcomes within a rule-governed framework. Optimality, specifically defined for perinatal health care, is the maximal perinatal outcome with minimal intervention placed against the context of the woman's social, medical, and obstetric history.
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
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.
Cobb, Aaron D.
Prenatal screening can lead to the detection and diagnosis of significantly life-limiting conditions affecting the unborn child. Recognizing the difficulties facing parents who decide to continue the pregnancy, some have proposed perinatal hospice as a new modality of care. Although the medical literature has begun to devote significant attention to these practices, systematic philosophical reflection on perinatal hospice has been relatively limited. Drawing on Alasdair MacIntyre’s account of the virtues of acknowledged dependence, I contend that perinatal hospice manifests and facilitates virtues essential to living well with human dependency and vulnerability. For this reason, perinatal hospice deserves broad support within society. PMID:26661051
Romero, J; Muñiz, J; Logica Tornatore, T; Holubiec, M; González, J; Barreto, G E; Guelman, L; Lillig, C H; Blanco, E; Capani, F
Perinatal asphyxia represents an important cause of severe neurological deficits including delayed mental and motor development, epilepsy, major cognitive deficits and blindness. However, at the moment, most of the therapeutic strategies were not well targeted toward the processes that induced the brain injury during perinatal asphyxia. Traditionally, experimental research focused on neurons, whereas astrocytes have been more related with the damage mechanisms of perinatal asphyxia. In this work, we propose to review possible protective as well as deleterious roles of astrocytes in the asphyctic brain with the aim to stimulate further research in this area of perinatal asphyxia still not well studied.
Cobb, Aaron D
Prenatal screening can lead to the detection and diagnosis of significantly life-limiting conditions affecting the unborn child. Recognizing the difficulties facing parents who decide to continue the pregnancy, some have proposed perinatal hospice as a new modality of care. Although the medical literature has begun to devote significant attention to these practices, systematic philosophical reflection on perinatal hospice has been relatively limited. Drawing on Alasdair MacIntyre's account of the virtues of acknowledged dependence, I contend that perinatal hospice manifests and facilitates virtues essential to living well with human dependency and vulnerability. For this reason, perinatal hospice deserves broad support within society.
Margolis, A.; VÃ¡zquez, R.; Mendoza, G.; Zignago, A.; LÃ³pez, A.; LuciÃ¡n, H.
In this article, new uses of the Perinatal Information System at the Uruguayan Social Security health care facilities are described. The perinatal information system has been in place for over 13 years, with about 40 thousand clinical records on electronic files. A newly created Web interface allows a distributed access to existing perinatal information within the National Social Security Wide Area a Network. Perinatal data is also exported to a management information system, allowing to dynamically answer questions and make managerial decisions, and eventually link these data with other sources. Future steps regarding clinical information systems are outlined. Images Figure 1 Figure 2 PMID:10566481
Luo, Zhong-Cheng; Zhao, Yan-Jun; Ouyang, Fengxiu; Yang, Zu-Jing; Guo, Yu-Na; Zhang, Jun
Background Diabetes in pregnancy has been associated with a paradoxically reduced risk of neonatal death in twin pregnancies. Risk “shift” may be a concern in that the reduction in neonatal deaths may be due to an increase in fetal deaths (stillbirths). This study aimed to clarify the impact of diabetes on the risk of perinatal death (neonatal death plus stillbirth) in twin pregnancies. Methods This was a retrospective cohort study of twin births using the largest available dataset on twin births (the U.S. matched multiple birth data 1995-2000; 19,676 neonates from diabetic pregnancies, 541,481 from non-diabetic pregnancies). Cox proportional hazard models were applied to estimate the adjusted hazard ratios (aHR) of perinatal death accounting for twin cluster-level dependence. Results Comparing diabetic versus non-diabetic twin pregnancies, overall perinatal mortality rate was counterintuitively lower [2.1% versus 3.3%, aHR 0.70 (95% confidence intervals 0.63-0.78)]. Individually, both stillbirth and neonatal mortality rates were lower in diabetic pregnancies, but we identified significant differences by gestational age and birth weight. Diabetes was associated with a survival benefit in pregnancies completed before 32 weeks [aHR 0.55 (0.48-0.63)] or with birth weight <1500 g [aHR 0.61 (0.53-0.69)]. In contrast, diabetes was associated with an elevated risk of perinatal death in pregnancies delivered between 32 and 36 weeks [aHR 1.38 (1.10-1.72)] or with birth weight >=2500 g [aHR 2.20 (1.55-3.13)]. Conclusions Diabetes in pregnancy appears to be “protective” against perinatal death in twin pregnancies ending in very preterm or very low birth weight births. Prospective studies are required to clarify whether these patterns of risk are real, or they are artifacts of unmeasured confounders. Additional data correlating these outcomes with the types of diabetes in pregnancy are also needed to distinguish the effects of pre-gestational vs. gestational diabetes
Vierling, John M
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.
Basu, P Patrick; Shah, Niraj James
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.
Leise, Michael D; Poterucha, John J; Kamath, Patrick S; Kim, W Ray
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
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.
Ong, Chin-Sing; McConnell, James R.; Chu, Wei-Kom
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.
Novy, Jan; Catarino, Claudia B; Chinthapalli, Krishna; Smith, Shelagh M; Clayton-Smith, Jill; Hennekam, Raoul C M; Hammond, Peter; Sisodiya, Sanjay M
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.
Martínez-Biarge, M; Blanco, D; García-Alix, A; Salas, S
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.
Harrison, Rebecca A; Vu, Trung; Hunter, Alan J
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
Cantu, Robert; Chin, Lawrence S.
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
Frijlink, Daphne W; Tilanus, Joachim J; Roks, Gerwin
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.
Dixon, Brandon J.; Reis, Cesar; Ho, Wing Mann; Tang, Jiping; Zhang, John H.
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
Sheasgreen, Christopher; Lu, Lucy; Patel, Ameen
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.
Atluri, Dileep K; Prakash, Ravi; Mullen, Kevin D
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
Peleman, Cedric; Camilleri, Michael
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
McGhee, A.S.; Kassouny, M.E.; Matthews, D.E.; Millikan, W.
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.
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
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.
Miller, D L; Ross, E M
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
Wicklund, Meredith R; Knopman, David S
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.
Lough, Mary E
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.
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.
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
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.
HASSANZADEH RAD, Afagh; AMINZADEH, Vahid
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
Maramattom, Boby Varkey; Raja, Rajat; Balagopal, Anuroop
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. 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
Jain, Puneet; Tripathi, Manjari
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
This report presented a brief overview of the literature on the perinatal asphyxia syndrome (PAS) in foals as a prelude to a description of the investigation and treatment of acute onset seizures in a 24-hour-old Thoroughbred colt foal. PAS can cause a wide variety of clinical abnormalities, of which seizures due to encephalopathy are the most significant. The structural and biochemical components of CNS neurones are disrupted by the shift from oxidative to anaerobic metabolism, with a resultant deficit in cellular energy. The cells succumb to the combined effects of acidosis, neurotoxic activities of glutamate, nitric oxide and free radicals, lipid peroxidation, accumulation of intracellular calcium and destructive overactivity of intracellular enzymes. Concurrently, the hypoxia affects other organ systems and management of foals presenting with CNS signs requires the veterinarian to undertake a thorough clinical examination and to institute appropriate therapy for the various derangements induced by the hypoxic-ischaemic episode. Diazepam (0.1 to 0.2 mg/kg bwt) can be used for short-term control of seizures; phenobarbital (2 to 10 mg/kg bwt) may be required for more prolonged treatment of recurrent seizures. The needs of the affected foal for nutrients, fluids and electrolytes, antimicrobial therapy and ancillary therapies were discussed in the literature review and illustrated in the case report. PMID:21851654
Misri, Shaila; Abizadeh, Jasmin; Sanders, Shawn; Swift, Elena
Perinatal generalized anxiety disorder (GAD) has a high prevalence of 8.5%-10.5% during pregnancy and 4.4%-10.8% postpartum. Despite its attendant dysfunction in the patient, this potentially debilitating mental health condition is often underdiagnosed. This overview will provide guidance for clinicians in making timely diagnosis and managing symptoms appropriately. A significant barrier to the diagnosis of GAD in the perinatal population is difficulty in distinguishing normal versus pathological worry. Because a perinatal-specific screening tool for GAD is nonexistent, early identification, diagnosis and treatment is often compromised. The resultant maternal dysfunction can potentially impact mother-infant bonding and influence neurodevelopmental outcomes in the children. Comorbid occurrence of GAD and major depressive disorder changes the illness course and its treatment outcome. Psychoeducation is a key component in overcoming denial/stigma and facilitating successful intervention. Treatment strategies are contingent upon illness severity. Cognitive behavior therapy (CBT), relaxation, and mindfulness therapy are indicated for mild GAD. Moderate/severe illness requires pharmacotherapy and CBT, individually or in combination. No psychotropic medications are approved by the FDA or Health Canada in pregnancy or the postpartum; off-label pharmacological treatment is instituted only if the benefit of therapy outweighs its risk. SSRIs/SNRIs are the first-line treatment for anxiety disorders due to data supporting their efficacy and overall favorable side effect profile. Benzodiazepines are an option for short-term treatment. While research on atypical antipsychotics is evolving, some can be considered for severe manifestations where the response to antidepressants or benzodiazepines has been insufficient. A case example will illustrate the onset, clinical course, and treatment strategies of GAD through pregnancy and the postpartum.
Dumont, Dora M.; Wildeman, Christopher; Lee, Hedwig; Gjelsvik, Annie; Valera, Pamela A.; Clarke, Jennifer G.
Background 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. Methods We analyzed 2006-2010 data from the Pregnancy Risk Assessment and Monitoring System (PRAMS) to assess the association between incarceration in the year prior to the birth of a child and perinatal maternal hardships and behaviors. Results Women reporting incarceration of themselves or their partners in the year before birth of a child had 0.86 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. Conclusions 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 U.S. simultaneously presents an underutilized public health opportunity and constitutes a social determinant of health that may contribute to disparities in early childhood development. PMID:24615355
Abizadeh, Jasmin; Sanders, Shawn; Swift, Elena
Abstract Perinatal generalized anxiety disorder (GAD) has a high prevalence of 8.5%–10.5% during pregnancy and 4.4%–10.8% postpartum. Despite its attendant dysfunction in the patient, this potentially debilitating mental health condition is often underdiagnosed. This overview will provide guidance for clinicians in making timely diagnosis and managing symptoms appropriately. A significant barrier to the diagnosis of GAD in the perinatal population is difficulty in distinguishing normal versus pathological worry. Because a perinatal-specific screening tool for GAD is nonexistent, early identification, diagnosis and treatment is often compromised. The resultant maternal dysfunction can potentially impact mother–infant bonding and influence neurodevelopmental outcomes in the children. Comorbid occurrence of GAD and major depressive disorder changes the illness course and its treatment outcome. Psychoeducation is a key component in overcoming denial/stigma and facilitating successful intervention. Treatment strategies are contingent upon illness severity. Cognitive behavior therapy (CBT), relaxation, and mindfulness therapy are indicated for mild GAD. Moderate/severe illness requires pharmacotherapy and CBT, individually or in combination. No psychotropic medications are approved by the FDA or Health Canada in pregnancy or the postpartum; off-label pharmacological treatment is instituted only if the benefit of therapy outweighs its risk. SSRIs/SNRIs are the first-line treatment for anxiety disorders due to data supporting their efficacy and overall favorable side effect profile. Benzodiazepines are an option for short-term treatment. While research on atypical antipsychotics is evolving, some can be considered for severe manifestations where the response to antidepressants or benzodiazepines has been insufficient. A case example will illustrate the onset, clinical course, and treatment strategies of GAD through pregnancy and the postpartum. PMID:26125602
Gillberg, Christopher; Cederlund, Mats
Objective: Study familial and pre- and perinatal factors in Asperger Syndrome (AS). Methods: Hundred boys with AS had their records reviewed. "Pathogenetic subgroups" were defined according to presence of medical syndromes/chromosomal abnormalities, indices of familiarity, and pre- and perinatal risk factors predisposing to brain damage. Results:…
Kotini, A; Anastasiadis, A N; Koutlaki, N; Tamiolakis, D; Anninos, P; Anastasiadis, P
This is a report on our experience in the application of biomagnetism in perinatal medicine. We provide a brief description of our research work in fetal magnetoencephalography and fetal magnetocardiography in normal, preeclamptic and IUGR pregnancies, together with hemodynamics of the umbilical cord and uterine arteries, providing a new approach to biomagnetism as a non invasive imaging modality in the investigation of perinatal complications.
Manderbacka, Kristiina; And Others
Examined association between mother's marital status and perinatal outcome among single births in Finland in 1987 (n=56,595 infants). Found that perinatal deaths, low birthweight, and preterm infants were more common among single mothers than among married mothers. Results for cohabiting mothers were more similar to those of married than to those…
Harris, Allyssa L
Depression is a significant health issue for women of reproductive age. A number of professional organizations have issued guidance regarding perinatal depression screening. However, some health care providers are reluctant to screen women. This column takes a second look at two recent research studies in which investigators examined the barriers to and facilitators of perinatal depression screening.
The state of Indiana took a unique approach to developing a statewide plan to improve perinatal health outcomes by engaging parents in a series of focus groups, called Community Conversations in Perinatal Care (CCPC), to hear directly from consumers about their health care experiences and needs. Recognizing that disparities exist among different…
Tillema, Jan-Mendelt; Byars, Anna W.; Jacola, Lisa M.; Schapiro, Mark B.; Schmithorst, Vince J.; Szaflarski, Jerzy P.; Holland, Scott K.
Objective: Functional MRI was used to determine differences in patterns of cortical activation between children who suffered perinatal left middle cerebral artery (MCA) stroke and healthy children performing a silent verb generation task. Methods: Ten children with prior perinatal left MCA stroke (age 6-16 years) and ten healthy age matched…
O'Mahen, Heather; Fedock, Gina; Henshaw, Erin; Himle, Joseph A.; Forman, Jane; Flynn, Heather A.
The evidence for the efficacy of CBT for depression during the perinatal period is mixed. This was a qualitative study that aimed to understand the perinatal-specific needs of depressed women in an effort to inform treatment modifications that may increase the relevance and acceptability of CBT during this period. Stratified purposeful sampling…
Harolds, Laura; Hurst, Helen
Many aspects of perinatal nursing put nurses at risk for injuries, including frequent repetitive bending, lifting of clients, and exposure to potentially large amounts of body fluids such as blood and amniotic fluid. Violence is also a potential risk with stressful family situations that may arise around childbirth. Workplace injuries put a health care facility at risk for staff turnover, decreases in the number of skilled nurses, client dissatisfaction, workers' compensation payouts, and employee lawsuits. Through the use of safety equipment, improved safety and violence training programs, "no manual lift" policies, reinforcement of personal protective equipment usage, and diligent staff training to improve awareness, these risks can be minimized.
Rombaldi, Renato L; Serafini, Eduardo P; Mandelli, Jovana; Zimmermann, Edineia; Losquiavo, Kamille P
The purpose was to study the perinatal transmission of human papillomavirus DNA (HPV-DNA) in 63 mother-newborn pairs, besides looking at the epidemiological factors involved in the viral DNA transmission. The following sampling methods were used: (1) in the pregnant woman, when was recruited, in cervix and clinical lesions of the vagina, vulva and perineal region; (2) in the newborn, (a) buccal, axillary and inguinal regions; (b) nasopharyngeal aspirate, and (c) cord blood; (3) in the children, buccal was repeated in the 4th week and 6th and 12th month of life. HPV-DNA was identified using two methodologies: multiplex PCR (PGMY09 and MY11 primers) and nested-PCR (genotypes 6/11, 16, 18, 31, 33, 42, 52 and 58). Perinatal transmission was considered when concordance was found in type-specific HPV between mother/newborn or mother/child. HPV-DNA genital was detected in 49 pregnant women submitted to delivery. Eleven newborns (22.4%, n = 11/49) were HPV-DNA positive. In 8 cases (16.3%, n = 8/49) there was type specific HPV concordance between mother/newborn samples. At the end of the first month of life three children (6.1%, n = 3/49) became HPV-DNA positive, while two remained positive from birth. In 3 cases (100%, n = 3/3) there was type specific HPV concordance between mother/newborn samples. In the 6th month, a child (2%, n = 1/49) had become HPV-DNA positive between the 1st and 6th month of life, and there was type specific HPV concordance of mother/newborn samples. All the HPV-DNA positive children (22.4%, n = 11/49) at birth and at the end first month of life (6.1%, n = 3/49) became HPV-DNA negative at the age of 6 months. The HPV-DNA positive child (2%, n = 1/49) from 1st to the 6th month of life became HPV-DNA negative between the 6th and 12th month of life and one child had anogenital warts. In the twelfth month all (100%, n = 49/49) the children studied were HPV-DNA negative. A positive and significant correlation was observed between perinatal transmission
Brausch, Amy M; Decker, Kristina M; Hadley, Andrea G
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.
Ilves, Pilvi; Laugesaar, Rael; Loorits, Dagmar; Kolk, Anneli; Tomberg, Tiiu; Lõo, Silva; Talvik, Inga; Kahre, Tiina; Talvik, Tiina
It is unknown why some infants with perinatal stroke present clinical symptoms late during infancy and will be identified as infants with presumed perinatal stroke. The risk factors and clinical and radiological data of 42 infants with presumed perinatal stroke (69% with periventricular venous infarction and 31% with arterial ischemic stroke) from the Estonian Pediatric Stroke Database were reviewed. Children with presumed perinatal stroke were born at term in 95% of the cases and had had no risk factors during pregnancy in 43% of the cases. Children with periventricular venous infarction were born significantly more often (82%) vaginally (P = .0213) compared to children with arterial stroke (42%); nor did they require resuscitation (P = .0212) or had any neurological symptoms after birth (P = .0249). Periventricular venous infarction is the most common type of lesion among infants with the presumed perinatal stroke. Data suggest that the disease is of prenatal origin.
Rip, M R; Keen, C S; Kibel, M A
An infant's weight at birth as well as its socio-economic environment are recognized as constituting two of the major risk factors associated with perinatal mortality. Spatial analyses of birth weight, socio-economic status and perinatal mortality in Metropolitan Cape Town for the year 1982 are presented in an attempt to assess the relationship between these variables at the suburb (or community) level. Variations in perinatal mortality for each suburb were found to be highly correlated with variations in the distribution of low birth weights. Overall, it would appear that the geography of the interrelationship between low birth weight and perinatal mortality tends, in part, to mirror long-standing gradients in socio-economic status--particularly for those coloured communities which show high perinatal death rates. To what extent these variations are associated with available antenatal and infant health care services can only be postulated. Points for possible community intervention are suggested.
Rhodes, Ann M; Segre, Lisa S
Accumulating research documenting the prevalence and negative effects of perinatal depression, together with highly publicized tragic critical incidents of suicide and filicide by mothers with postpartum psychosis, have fueled a continuum of legislation. Specialists in perinatal mental health should recognize how their work influences legislative initiatives and penal codes, and take this into consideration when developing perinatal services and research. Yet, without legal expertise, the status of legislative initiatives can be confusing. To address this shortfall, we assembled an interdisciplinary team of academics specializing in law, as well as perinatal mental health, to summarize these issues. This review presents the relevant federal and state legislation and summarizes the criminal codes that governed the court decisions on cases in which a mother committed filicide because of postpartum psychosis. Moreover, the review aims to help researchers and providers who specialize in perinatal depression understand their role in this legal landscape.
Smolich, Joseph J; Kenna, Kelly R; Cheung, Michael M
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.
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.
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
Kim, J Jo; Silver, Richard K; Elue, Rita; Adams, Marci G; La Porte, Laura M; Cai, Li; Kim, Jong Bae; Gibbons, Robert D
We assessed differential item functioning (DIF) based on computerized adaptive testing (CAT) to examine how perinatal mood disorders differ from adult psychiatric disorders. The CAT-Mental Health (CAT-MH) was administered to 1614 adult psychiatric outpatients and 419 perinatal women with IRB approval. We examined individual item-level differences using logistic regression and overall score differences by scoring the perinatal data using the original bifactor model calibration based on the psychiatric sample data and a new bifactor model calibration based on the perinatal data and computing their correlation. To examine convergent validity, we computed correlations of the CAT-MH with contemporaneously administered Edinburgh Postnatal Depression Scales (EPDS). The rate of major depression in the perinatal sample was 13 %. Rates of anxiety, mania, and suicide risk were 5, 6, and 0.4 %, respectively. One of 66 depression items, one of 69 anxiety items, and 15 of 53 mania items exhibited DIF (i.e., failure to discriminate between high and low levels of the disorder) in the perinatal sample based on the psychiatric sample calibration. Removal of these items resulted in correlations of the original and perinatal calibrations of r = 0.983 for depression, r = 0.986 for anxiety, and r = 0.932 for mania. The 91.3 % of cases were concordantly categorized as either "at-risk" or "low-risk" between the EPDS and the perinatal calibration of the CAT-MH. There was little evidence of DIF for depression and anxiety symptoms in perinatal women. This was not true for mania. Now calibrated for perinatal women, the CAT-MH can be evaluated for longitudinal symptom monitoring.
Kobtan, Abdelrahman A; El-Kalla, Ferial S; Soliman, Hanan H; Zakaria, Soha S; Goda, Mohamed A
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.
Finkelstein, Julia L; Layden, Alexander J; Stover, Patrick J
Vitamin B-12 deficiency (<148 pmol/L) is associated with adverse maternal and neonatal outcomes, including developmental anomalies, spontaneous abortions, preeclampsia, and low birth weight (<2500 g). The importance of adequate vitamin B-12 status periconceptionally and during pregnancy cannot be overemphasized, given its fundamental role in neural myelination, brain development, and growth. Infants born to vitamin B-12-deficient women may be at increased risk of neural tube closure defects, and maternal vitamin B-12 insufficiency (<200 pmol/L) can impair infant growth, psychomotor function, and brain development, which may be irreversible. However, the underlying causal mechanisms are unknown. This review was conducted to examine the evidence that links maternal vitamin B-12 status and perinatal outcomes. Despite the high prevalence of vitamin B-12 deficiency and associated risk of pregnancy complications, few prospective studies and, to our knowledge, only 1 randomized trial have examined the effects of vitamin B-12 supplementation during pregnancy. The role of vitamin B-12 in the etiology of adverse perinatal outcomes needs to be elucidated to inform public health interventions.
Development of animal models is a crucial issue in biological psychiatry for the search of novel drug targets as well as the screening of candidate compounds. Epidemiologic studies suggest that environmental insults, such as prenatal infection and perinatal complication, are involved in the development of schizophrenia. Recently, we have developed a novel mouse model of viral infection during the perinatal stage by injecting polyriboinosinic-polyribocytidilic acid (polyI:C) into neonatal mice. Neonatal treatment of mice with polyI:C, an inducer of innate immune responses via toll-like receptor 3, caused a significant increase in interferon-induced transmembrane protein 3 (IFITM3) levels in the astrocytes of the hippocampus, which resulted in long-lasting brain dysfunction, including cognitive and emotional impairments as well as a deficit in depolarization-evoked glutamate release in the hippocampus in adulthood. Neonatal polyI:C-induced neuronal impairments have not been observed in IFITM3-KO mice. These findings suggest that the induction of IFITM3 expression in astrocytes by the activation of the innate immune system during the early stages of neurodevelopment has non-cell autonomous effects that affect subsequent neurodevelopment, leading to neuropathological impairments and brain dysfunction, by impairing endocytosis in astrocytes.
Harville, EW; Xiong, X; Buekens, P
Background The empirical literature on the effects of disaster on pregnancy and the postpartum period is limited. The objective of this review was to examine the existing evidence on the effect of disasters on perinatal health. Methods A systematic review was conducted by searching electronic databases (MEDLINE, EMBASE, Cinahl, PsycInfo), including literature on disasters and pregnancy outcomes (e.g., preterm birth, low birthweight, congenital anomalies), mental health, and child development. 110 articles were identified, but many published reports were anecdotes or recommendations rather than systematic studies. The final review included 49 peer-reviewed studies that met inclusion criteria. Results Studies addressing the World Trade Center disaster of September 11th and other terrorist attacks, environmental/chemical disasters, and natural disasters such as hurricanes and earthquakes were identified. Disasters of various types may reduce fetal growth in some women, though there does not appear to be an effect on gestational age at birth. Severity of exposure is the major predictor of mental health issues among pregnant and postpartum women. The mother's mental health after a disaster may more strongly influence on child development than any direct effect of disaster-related prenatal stress. Conclusions There is evidence that disaster impacts maternal mental health and some perinatal health outcomes, particular among highly-exposed women. Future research should focus on under-studied outcomes such as spontaneous abortion. Relief workers and clinicians should concentrate on the most exposed women, particularly with respect to mental health. PMID:21375788
Steiner, H; Neligan, G
Steiner, H., and Neligan, G. (1975). Archives of Disease in Childhood, 50, 696. Perinatal cardiac arrest: quality of the survivors. Twenty-two consecutive survivors of perinatal cardiac arrest have been followed to a mean age of 4 1/4 years, using methods of neurological and developmental assessment appropriate to their ages. 4 showed evidence of gross, diffuse brain-damage (2 of these died before the age of 3 years). These were the only 4 survivors of the first month of life who took more than 30 minutes to establish regular, active respiration after their heartbeat had been restored. The arrest in these cases had occurred during or within 15 minutes of delivery, and followed antepartum haemorrhage, breech delivery, or prolapsed cord. The remaining 18 were free of any evidence of brain damage. In the majority of these the arrest had occurred during shoulder dystocia or exchange transfusion, or was unexplained; the heartbeat had been restored within 5 minutes in most cases, and regular, active respiration had been established within 30 minutes thereafter in all cases. PMID:1190819
Kuhn, C; Bero, L; Ignar, D; Lurie, S; Field, E
In summary, we have shown that marked acute responses as well as persistent changes in hypothalamopituitary responsivity to opiate challenge result from perinatal opioid addiction. We have also shown that different endocrine systems and opioid receptor subtypes develop at different rates, and that the responses of these systems depend upon the relative timing of the treatment regimen and the functional development of the particular opioid system involved. It should be emphasized that these studies have investigated only a single developmental window. The additional critical question of how opioid neuron function is affected by treatment during the period of active neuronal differentiation has not yet been answered. However, these studies do demonstrate the utility of this neuroendocrine model in assessing opioid function following chronic treatment regimens. By using neuroendocrine function as an end point, multiple systems can be studied simultaneously in the same animal. This has a particular advantage in studying the effects of chronic drug exposure on the developing nervous system, because hormone secretion is an easily quantifiable and early maturing functional index which can be used to identify vulnerable (and resistant) systems. Endogenous opioid systems appear to be particularly important in neuroendocrine regulation during the early phase of development, when other neural controls have not yet matured. Our preliminary results suggest that specific opioid systems that mature early may be especially important in the specific neuroendocrine effects of perinatal opiate addiction.
Massoni, F; Troili, G M; Pelosi, M; Ricci, S
Perinatal testicular torsion (PTT) is a very complex condition because of rarity of presentation and diagnostic and therapeutic difficulties. In presence of perinatal testicular torsion, the involvement of contralateral testis can be present also in absence of other indications which suggest the bilateral involvement; therefore, occurrences supported by literature do not exclude the use of surgery to avoid the risk of omitted or delayed diagnosis. The data on possible recovery of these testicles are not satisfactory, and treatment consists of an observational approach ("wait-and-see") or an interventional approach. The hypothesis of randomized clinical trials seems impracticable because of rarity of disease. The authors present a case of PTT, analyzing injuries due to clinical and surgical management of these patients, according to medicolegal profile. The delayed diagnosis and the choice of an incorrect therapeutic approach can compromise the position of healthcare professionals, defective in terms of skill, prudence and diligence. Endocrine insufficiency is an unfortunate event. The analysis of literature seems to support, because of high risk, a surgical approach aimed not only at resolution of unilateral pathology or prevention of a relapse, but also at prevention of contralateral testicular torsion.
Van Kerrebroeck, Philip
Perinatal testicular torsion is a relatively rare event that remains unrecognized in many patients or is suspected and treated accordingly only after an avoidable loss of time. The authors report their own experience with several patients, some of them quite atypical but instructive. Missed bilateral torsion is an issue, as are partial torsion, possible antenatal signs, and late presentation. These data are discussed together with the existing literature and may help shed new light on the natural course of testicular torsion and its treatment. The most important conclusion is that a much higher index of suspicion based on clinical findings is needed for timely detection of perinatal torsion. It is the authors’ opinion that immediate surgery is mandatory not only in suspected bilateral torsions but also in cases of possible unilateral torsions. There is no place for a more fatalistic “wait-and-see” approach. Whenever possible, even necrotic testes should not be removed during surgery because some endocrine function may be retained. PMID:19856186
Finkelstein, Julia L; Layden, Alexander J; Stover, Patrick J
Vitamin B-12 deficiency (<148 pmol/L) is associated with adverse maternal and neonatal outcomes, including developmental anomalies, spontaneous abortions, preeclampsia, and low birth weight (<2500 g). The importance of adequate vitamin B-12 status periconceptionally and during pregnancy cannot be overemphasized, given its fundamental role in neural myelination, brain development, and growth. Infants born to vitamin B-12-deficient women may be at increased risk of neural tube closure defects, and maternal vitamin B-12 insufficiency (<200 pmol/L) can impair infant growth, psychomotor function, and brain development, which may be irreversible. However, the underlying causal mechanisms are unknown. This review was conducted to examine the evidence that links maternal vitamin B-12 status and perinatal outcomes. Despite the high prevalence of vitamin B-12 deficiency and associated risk of pregnancy complications, few prospective studies and, to our knowledge, only 1 randomized trial have examined the effects of vitamin B-12 supplementation during pregnancy. The role of vitamin B-12 in the etiology of adverse perinatal outcomes needs to be elucidated to inform public health interventions. PMID:26374177
Savlan, Ilona; Liakina, Valentina; Valantinas, Jonas
Hepatic encephalopathy is a neuropsychiatric complication of liver cirrhosis the symptoms of which may vary from imperceptible to severe, invaliding, and even lethal. Minimal hepatic encephalopathy is also important because of its tendency to impair patients' cognitive functions and quality of life. The polyetiological pathogenesis of hepatic encephalopathy is intensively studied. A general consensus exists that not only excess of ammonia but also inflammatory, oxidative, and other processes are significant in the development of hepatic encephalopathy.
McKee, Ann C.; Stein, Thor D.; Kiernan, Patrick T.; Alvarez, Victor E.
Repetitive brain trauma is associated with a progressive neurological deterioration, now termed as chronic traumatic encephalopathy (CTE). Most instances of CTE occur in association with the play of sports, but CTE has also been reported in association with blast injuries and other neurotrauma. Symptoms of CTE include behavioral and mood changes, memory loss, cognitive impairment and dementia. Like many other neurodegenerative diseases, CTE is diagnosed with certainty only by neuropathological examination of brain tissue. CTE is a tauopathy characterized by the deposition of hyperphosphorylated tau (p-tau) protein as neurofibrillary tangles, astrocytic tangles and neurites in striking clusters around small blood vessels of the cortex, typically at the sulcal depths. Severely affected cases show p-tau pathology throughout the brain. Abnormalities in phosphorylated 43 kDa TAR DNA-binding protein are found in most cases of CTE; beta-amyloid is identified in 43%, associated with age. Given the importance of sports participation and physical exercise to physical and psychological health as well as disease resilience, it is critical to identify the genetic risk factors for CTE as well as to understand how other variables, such as stress, age at exposure, gender, substance abuse and other exposures, contribute to the development of CTE. PMID:25904048
Grover, Vijay PB; Tognarelli, Joshua M; Massie, Nicolas; Crossey, Mary ME; Cook, Nicola A; Taylor-Robinson, Simon D
Hepatic encephalopathy is a common neuropsychiatric abnormality, which complicates the course of patients with liver disease. It was probably first described by Hippocrates over 2000 years ago, who said that “those whose madness arises from phlegm are quiet and neither shout nor make a disturbance, while those whose madness arises from bile shout, play tricks and will not keep still, but are always up to some mischief ”. He was presumably describing the differences between patients with pneumonia and acute liver failure. Despite the fact that the syndrome was probably first recognized thousands of years ago, the exact pathogenesis still remains unclear. Furthermore, a precise definition of the syndrome is lacking, as are definitive methods of diagnosing this condition. It is important as both patients with cirrhosis and the general population with whom they interact may be affected as a consequence. At a minimum, the individual may be affected by impaired quality of life, impaired ability to work, and slowed reaction times, which are relevant to the population at large if affected individuals operate heavy machinery or drive a car. Pathogenic mechanisms, diagnostic tools, and treatment options are discussed. PMID:26719720
McKee, Ann C; Stein, Thor D; Kiernan, Patrick T; Alvarez, Victor E
Repetitive brain trauma is associated with a progressive neurological deterioration, now termed as chronic traumatic encephalopathy (CTE). Most instances of CTE occur in association with the play of sports, but CTE has also been reported in association with blast injuries and other neurotrauma. Symptoms of CTE include behavioral and mood changes, memory loss, cognitive impairment and dementia. Like many other neurodegenerative diseases, CTE is diagnosed with certainty only by neuropathological examination of brain tissue. CTE is a tauopathy characterized by the deposition of hyperphosphorylated tau (p-tau) protein as neurofibrillary tangles, astrocytic tangles and neurites in striking clusters around small blood vessels of the cortex, typically at the sulcal depths. Severely affected cases show p-tau pathology throughout the brain. Abnormalities in phosphorylated 43 kDa TAR DNA-binding protein are found in most cases of CTE; beta-amyloid is identified in 43%, associated with age. Given the importance of sports participation and physical exercise to physical and psychological health as well as disease resilience, it is critical to identify the genetic risk factors for CTE as well as to understand how other variables, such as stress, age at exposure, gender, substance abuse and other exposures, contribute to the development of CTE.
García-Martínez, Rita; Simón-Talero, Macarena; Córdoba, Juan
Hepatic encephalopathy (HE) is a common complication of liver failure that is associated with poor prognosis. However, the prognosis is not uniform and depends on the underlying liver disease. Acute liver failure is an uncommon cause of HE that carries bad prognosis but is potentially reversible. There are several prognostic systems that have been specifically developed for selecting patients for liver transplantation. In patients with cirrhosis the prognosis of the episode of HE is usually dictated by the underlying precipitating factor. Acute-on-chronic liver failure is the most severe form of decompensation of cirrhosis, the prognosis depends on the number of associated organ failures. Patients with cirrhosis that have experienced an episode of HE should be considered candidates for liver transplant. The selection depends on the underlying liver function assessed by the Model for End-stage Liver Disease (MELD) index. There is a subgroup that exhibits low MELD and recurrent HE, usually due to the coexistence of large portosystemic shunts. The recurrence of HE is more common in patients that develop progressive deterioration of liver function and hyponatremia. The bouts of HE may cause sequels that have been shown to persist after liver transplant. PMID:22045403
Patidar, Kavish R; Bajaj, Jasmohan S
The treatment of hepatic encephalopathy (HE) is complex and therapeutic regimens vary according to the acuity of presentation and the goals of therapy. Most treatments for HE rely on manipulating the intestinal milieu and therefore antibiotics that act on the gut form a key treatment strategy. Prominent antibiotics studied in HE are neomycin, metronidazole, vancomycin and rifaximin. For the management of the acute episode, all antibiotics have been tested. However the limited numbers studied, adverse effects (neomycin oto- and nephrotoxicity, metronidazole neurotoxicity) and potential for resistance emergence (vancomycin-resistant enterococcus) has limited the use of most antibiotics, apart from rifaximin which has the greatest evidence base. Rifaximin has also demonstrated, in conjunction with lactulose, to prevent overt HE recurrence in a multi-center, randomized trial. Despite its cost in the US, rifaximin may prove cost-saving by preventing hospitalizations for overt HE. In minimal/covert HE, rifaximin is the only systematically studied antibiotic. Rifaximin showed improvement in cognition, inflammation, quality-of-life and driving simulator performance but cost-analysis does not favor its use at the current time. Antibiotics, especially rifaximin, have a definite role in the management across the spectrum of HE.
Jung, Young-Chul; Chanraud, Sandra; Sullivan, Edith V
There is considerable evidence that neuroimaging findings can improve the early diagnosis of Wernicke's encephalopathy (WE) in clinical settings. The most distinctive neuroimaging finding of acute WE are cytotoxic edema and vasogenic edema, which are represented by bilateral symmetric hyperintensity alterations on T2-weighted MR images in the periphery of the third ventricle, periaqueductal area, mammillary bodies and midbrain tectal plate. An initial bout of WE can result in Korsakoff's syndrome (KS), but repeated bouts in conjunction with its typical comorbidity, chronic alcoholism, can result in signs of tissue degeneration in vulnerable brain regions. Chronic abnormalities identified with neuroimaging enable examination of brain damage in living patients with KS and have expanded the understanding of the neuropsychological deficits resulting from thiamine deficiency, alcohol neurotoxicity, and their comorbidity. Brain structure and functional studies indicate that the interactions involving the thalamus, mammillary bodies, hippocampus, frontal lobes, and cerebellum are crucial for memory formation and executive functions, and the interruption of these circuits by WE and chronic alcoholism can contribute substantially to the neuropsychological deficits in KS.
Sun, Bo; Shao, Xiaomei; Cao, Yun; Xia, Shiwen; Yue, Hongni
With an annual birth rate of 12‰, or 16 millions, of all population (1.34 billions), and an implementation of universal healthcare policy for all rural residents in recent years, China is undergoing a dramatic and profound transition in perinatal and neonatal healthcare as a part of the global campaign for reduction in mortality of children under 5 years old. This review describes recent development in neonatal–perinatal medicine, with special emphasis on general neonatal–perinatal care, respiratory and intensive care, neurological and infectious diseases, for a comprehensive view of the trend and challenge in relation with problems and solutions of the field. PMID:23759518
Simini, F; Fernández, A; Sosa, C; Díaz Rossello, J L
The Perinatal Information System (SIP) is a clinical record, local management and quality assurance software standard in Latin America and the Caribbean. The time to implement SIP in a Maternity Hospital is evaluated as well as the effect of statistics on perinatal health indicators in subsequent years. In the sample of 20 Maternity Hospitals (5 Countries, 40% Private and 60% Public) 85% had a reliable information system by the third year of use of SIP. 15% of hospitals still had problems at that time that were already clear during the second year, a time corrective measures can still be taken. The evaluation of the impact of yearly reports shows that 58% of recommendations were fulfilled, specially those regarding the complete filling-in of clinical records (62%) and to a lesser extent variables that reflect clinical practices and organization of services (52%). The conclusion is that Maternity Hospitals in Latin America and the Caribbean have the capacity to adopt a complex tool of computerized clinical records for quality assurance of perinatal care and monitoring of health indicators.
Wong-Kisiel, Lily C.; Nickels, Katherine
Epileptic encephalopathy syndromes are disorders in which the epileptiform abnormalities are thought to contribute to a progressive cerebral dysfunction. Characteristic electroencephalogram findings have an important diagnostic value in classification of epileptic encephalopathy syndromes. In this paper, we focus on electroencephalogram findings of childhood epileptic encephalopathy syndromes and provide sample illustrations. PMID:24024028
... RIN 0579-AC68 Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products AGENCY... live bovines and products derived from bovines with regard to bovine spongiform encephalopathy. This... with regard to bovine spongiform encephalopathy. Comments on the proposed rule were required to......
Milner, R D; De Gasparo, M
The development of the autonomic nervous system in relation to perinatal metabolism is reviewed with particular attention given to the adipocyte, hepatocyte and the A and B cells of the islets of Langerhans. Adrenergic receptors develop in the B cell independently of normal innervation and by the time of birth, in most species studied, the pancreas, liver and adipose tissue respond appropriately to autonomic signals. Birth is associated with a huge surge in circulating catecholamines which is probably responsible for the early postnatal rise in free fatty acids and glucagon concentrations in plasma. beta-Blocking drugs such as propranolol have an adverse effect on fetal growth and neonatal metabolism, being responsible for hypoglycemia and for impairing the thermogenic response to cold exposure. beta-Mimetic drugs are commonly used to prevent premature labour and may help the fetus in other ways, for example, by improving the placental blood supply and the delivery of nutrients by increasing maternal fat and carbohydrate mobilization.
Güngör, T; Funk, M; Linde, R; Jacobi, G; Horn, M; Kreuz, W
A 7 year old child perinatally infected with HIV who died from progressive muscular paralysis and central nervous respiratory failure is described. Cytomegalovirus (CMV) prophylaxis with a special intravenous CMV hyper-immunoglobulin had been successfully conducted for more than four years. Macroscopic and microscopic immunohistochemical examination of the spinal cord revealed a diffuse CMV infiltration of the entire myelon. CMV infected cells were identified as astrocytes, oligodendrocytes, neurons, macrophages, ependymal, endothelial, and Schwann cells. Other organs had no signs of CMV infection. Central nervous spinal CMV infection was most probably due to insufficient penetration of the blood-brain barrier by the CMV hyper-immunoglobulin. In suspicious cases early spinal magnetic resonance imaging (1.5 tesla) combined with an examination of urine and cerebrospinal fluid for CMV is recommended. Images Figure 1 Figure 2 Figure 3 PMID:8385439
Franklin, R C; Carpenter, L M; O'Grady, C M
Indices of thyroid function were measured in 229 healthy term neonates at birth and at 5, 10, and 15 days of age. Results were analysed to assess whether maternal diabetes mellitus, toxaemia of pregnancy, intrapartum fetal distress, duration of labour, method of delivery, asphyxia at birth, race, sex, birthweight, birth length, head circumference, or method of feeding influenced any index. Thyroxine, the free thyroxine index, and free thyroxine concentrations at birth correlated with birthweight. Method of delivery influenced mean thyroxine and free thyroxine index values at birth and at age 5 days. Mean values of triiodothyronine, reverse triiodothyronine, thyroxine binding globulin, and thyroid stimulating hormone were not affected by any of the perinatal factors studied. Birthweight and perhaps method of delivery should be taken into account when interpreting neonatal thyroxine parameters but determination of thyroid stimulating hormone as a screen for congenital hypothyroidism in healthy term neonates circumvents these considerations. PMID:3977386
Marks, Lucy; McConnell, Jennifer; Baker, Martyn
Health visitors' involvement in work with maternal depression has developed considerably over the last 10 years, with a focus upon problems in the postnatal period. In a paper last month we reported on research highlighting dilemmas that can arise for health visitors connected with a lack of an agreed conceptual framework. Conflicting roles they find themselves using when working with distressed mothers, and within a pressured organisational work setting, make it hard to give sufficient priority to such work. By focussing too narrowly on postnatal depression, other important perinatal psychological difficulties may be left unattended. We argue the need to create workplace time to address these issues by describing the provision of a training and supervision package to facilitate this. We give some informal indications of its success and some indicators of the broadening development of its membership and its content
In 2011 a national children's charity led a campaign that identified why help with postnatal depression (PND) needed to improve; however PND remains a huge problem. Numerous cases are still coming to light where a mother has not sought help or has not been given adequate support, with disastrous consequences. Why is this still happening and what can health professionals do? Former practising midwife and specialist PND counsellor at a charity supporting women and families suffering from PND, Joanne Morton discusses why increasing awareness, education and understanding of perinatal illness are vital to limit the devastating effects of antenatal depression (AND) and PND and how standards of care must improve to help mothers in need.
Ebringer, Alan; Rashid, Taha; Wilson, Clyde
"Bovine spongiform encephalopathy", "scrapie", as well as Creutzfeldt-Jakob disease and kuru belong to a group of related neurological conditions termed "transmissible spongiform encephalopathies". These diseases are based on the LD50 measurement whereby saline brain homogenates are injected into experimental animals and when 50% of them develop symptoms, this is considered as transmission of the disease, but the gold standard for diagnosis is autopsy examination. However, an untenable assumption is being made in that saline brain homogenates do not cause tissue damage but it is known since the time of Pasteur, that they give rise to "post-rabies vaccination allergic encephalomyelitis". This is the fundamental flaw in the diagnosis of these diseases. A way forward, however, is to examine infectious agents, such as Acinetobacter which show molecular mimicry with myelin and elevated levels of antibodies to this microbe are found in multiple sclerosis patients and animals affected by "bovine spongiform encephalopathy".
Patidar, Kavish R; Bajaj, Jasmohan S
Hepatic encephalopathy (HE) is part of a spectrum of neurocognitive changes in cirrhosis. HE is divided into 2 broad categories based on severity: covert hepatic encephalopathy (CHE) and overt hepatic encephalopathy (OHE). CHE has a significant impact on a patient's quality of life, driving performance, and recently has been associated with increased hospitalizations and death. Likewise, OHE is associated with increased rates of hospitalizations and mortality, and poor quality of life. Given its significant burden on patients, care takers, and the health care system, early diagnosis and management are imperative. In addition, focus also should be directed on patient and family member education on the disease progression and adherence to medications. Treatment strategies include the use of nonabsorbable disaccharides, antibiotics (ie, rifaximin), and, potentially, probiotics. Other therapies currently under further investigation include L-ornithine-L-aspartate, ornithine phenylacetate, glycerol phenylbutyrate, molecular adsorbent recirculating system, and albumin infusion.
Gul Mert, Gülen; Horoz, Ozden Ozgur; Herguner, M Ozlem; Incecik, Faruk; Yildizdas, R Dincer; Onenli Mungan, Neslihan; Yuksel, Bilgin; Altunbasak, Sakir
Hashimoto's encephalopathy is a rare clinically heterogenous condition consisting of encephalopathy, seizures and variable neurological and psychiatric manifestations, accompanied by high titres of serum antithyroid antibodies. We described the clinical and laboratory findings of four children (aged 8-17 years) with Hashimoto's encephalopathy. The clinical features of three patients at presentation included refractory epilepsy, and confusion, and one patient presented with behavioral and cognitive changes. During their presentation, two of them were in euthyroid, and the others were in hypothyroid status. All patients manifested increased antithyroid antibodies. Two patients improved with steroid treatment. The others responded to plasmapheresis instead of corticosteroid treatment. Physicians' awareness of this complication is of great importance because most patients respond dramatically to the treatment.
Pera, Maria Carmela; Randazzo, Giovanna; Masnada, Silvia; Dontin, Serena Donetti; De Giorgis, Valentina; Balottin, Umberto; Veggiotti, Pierangelo
Summary The aim of this retrospective study of children affected by epileptic encephalopathy was to evaluate seizure frequency, electroencephalographic pattern and neuropsychological status, before and after intravenous methylprednisolone therapy. Eleven children with epileptic encephalopathy were administered one cycle of intravenous methylprednisolone (15–30 mg/kg/day for three consecutive days, once a month for four months) in addition to constant dosages of their regular antiepileptic drugs. The treatment resulted in statistically significant reductions of generalized slow spike-and-wave discharges (p<0.0028) and seizure frequency (p<0.013), which persisted even after methylprednisolone pulse therapy was stopped. A globally positive outcome was noted in 9/11 patients (81.8%). This methylprednisolone treatment regimen did not cause significant or persistent adverse effects. We suggest that children with epileptic encephalopathy without an underlying structural lesion could be the best candidates for intravenous methylprednisolone pulse therapy. PMID:26910177
Wetzburger, C L; Van Regemorter, N; Szliwowski, H B; Abramowicz, M J; Van Bogaert, P
Trichothiodystrophy was diagnosed in a 3-year-old male presenting with speech delay, brittle hair, chronic neutropenia, and a history of febrile convulsions. Cranial magnetic resonance imaging revealed a focal subcortical and periventricular gray matter heterotopia. An acute encephalopathy with status epilepticus and coma occurred when he was 4 years of age during an upper respiratory tract infection. Magnetic resonance imaging revealed multifocal T2-weighted hypersignal lesions involving mainly the thalami, hippocampi, midbrain, and pons. Analysis of cerebrospinal fluid revealed hyperproteinorachia without pleocytosis. Results of an extensive metabolic evaluation of this acute brain injury, resembling the syndrome of acute necrotizing encephalopathy of childhood described in Japan, were negative. Focal neuronal migration disorder and acute encephalopathy with symmetric thalamic involvement are newly described neurologic manifestations of syndromes with trichothiodystrophy, which suggests that these conditions may have a common genetic background.
Torre Delgadillo, Aldo; Guerrero-Hernández, Ignacio; Uribe, Misael
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. This cirrhosis complication is generally not perceived by physician, and diagnosis can only be made by neuropsychological tests and other especial measurements like evoked potentials and image studies like positron emission tomography. 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.
Rath, Amitav; Naryanan, T Jaishree; Chowdhary, G V S; Murthy, J M K
Hyperammonemic encephalopathy with normal liver function is an uncommon serious adverse effect of valproate therapy. We retrospectively analyzed the case records of 5 patients of epilepsy on valproate with hyperammonemic encephalopathy. Of the 5 patients, 3 were on monotherapy. The mean valproate dose was 1250 mg/day and the duration of therapy ranged between 4 and 90 days. Alteration in the sensorium was the presenting clinical feature. The risk factors included high initial dose (2), long-term valproate therapy (1), and long-term valproate therapy with concomitant topiramate (1). There was good correlation between the fall in serum ammonia levels and clinical improvement. Hyperammonemic encephalopathy should be suspected in patients on valproate with altered sensorium. Response to treatment is rewarding.
Meltzer-Brody, Samantha; Jones, Ian
The perinatal period is a time of high risk for women with unipolar and bipolar mood disorders. We discuss treatment considerations for perinatal mood disorders, including unipolar and bipolar depression as well as postpartum psychosis. We further explore the unique issues faced by women and their families across the full trajectory of the perinatal period from preconception planning through pregnancy and following childbirth. Treatment of perinatal mood disorders requires a collaborative care approach between obstetrics practitioners and mental health providers, to ensure that a thoughtful risk : benefit analysis is conducted. It is vital to consider the risks of the underlying illness versus risks of medication exposure during pregnancy or lactation. When considering medication treatment, attention must be paid to prior medication trials that were most efficacious and best tolerated. Lastly, it is important to assess the impact of individual psychosocial stressors and lifestyle factors on treatment response.
Carter, Carolyn S
This article explores societal responses to perinatal drug abuse, including stigmatic attitudes and behaviors of health care workers that are directed toward women who abuse drugs during pregnancy. Health care providers' stigmatic responses can deter women from receiving perinatal care and place women and their unborn children at risk. Because poor women and women of color face a greater probability of being prosecuted or losing custody of their children for using drugs while they are pregnant, the article emphasizes societal responses to these client populations. 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 is provided.
Mavalankar, D. V.; Trivedi, C. R.; Gray, R. H.
To estimate levels and determinants of perinatal mortality, we conducted a hospital-based surveillance and case-control study, linked with a population survey, in Ahmedabad, India. The perinatal mortality rate was 79.0 per 1000, and was highest for preterm low-birth-weight babies. The case-control study of 451 stillbirths, 160 early neonatal deaths and 1465 controls showed that poor maternal nutritional status, absence of antenatal care, and complications during labour were independently associated with substantially increased risks of perinatal death. Multivariate analyses indicate that socioeconomic factors largely operate through these proximate factors and do not have an independent effect. Estimates of attributable risk derived from the prevalence of exposures in the population survey suggest that improvements in maternal nutrition and antenatal and intrapartum care could result in marked reductions of perinatal mortality. PMID:1934237
... heart rate by means of combining and coordinating uterine contraction and fetal heart monitors with... SERVICES (CONTINUED) MEDICAL DEVICES OBSTETRICAL AND GYNECOLOGICAL DEVICES Obstetrical and Gynecological Monitoring Devices § 884.2740 Perinatal monitoring system and accessories. (a) Identification. A...
... heart rate by means of combining and coordinating uterine contraction and fetal heart monitors with... SERVICES (CONTINUED) MEDICAL DEVICES OBSTETRICAL AND GYNECOLOGICAL DEVICES Obstetrical and Gynecological Monitoring Devices § 884.2740 Perinatal monitoring system and accessories. (a) Identification. A...
Meltzer-Brody, Samantha; Jones, Ian
The perinatal period is a time of high risk for women with unipolar and bipolar mood disorders. We discuss treatment considerations for perinatal mood disorders, including unipolar and bipolar depression as well as postpartum psychosis. We further explore the unique issues faced by women and their families across the full trajectory of the perinatal period from preconception planning through pregnancy and following childbirth. Treatment of perinatal mood disorders requires a collaborative care approach between obstetrics practitioners and mental health providers, to ensure that a thoughtful risk : benefit analysis is conducted. It is vital to consider the risks of the underlying illness versus risks of medication exposure during pregnancy or lactation. When considering medication treatment, attention must be paid to prior medication trials that were most efficacious and best tolerated. Lastly, it is important to assess the impact of individual psychosocial stressors and lifestyle factors on treatment response. PMID:26246794
Hua, Cong; Ju, Wei-na; Jin, Hang; Sun, Xin; Zhao, Gang
Hypoxic-ischemic encephalopathy (HIE) is a disease that occurs when the brain is subjected to hypoxia, resulting in neuronal death and neurological deficits, with a poor prognosis. The mechanisms underlying hypoxic-ischemic brain injury include excitatory amino acid release, cellular proteolysis, reactive oxygen species generation, nitric oxide synthesis, and inflammation. The molecular and cellular changes in HIE include protein misfolding, aggregation, and destruction of organelles. The apoptotic pathways activated by ischemia and hypoxia include the mitochondrial pathway, the extrinsic Fas receptor pathway, and the endoplasmic reticulum stress-induced pathway. Numerous treatments for hypoxic-ischemic brain injury caused by HIE have been developed over the last half century. Hypothermia, xenon gas treatment, the use of melatonin and erythropoietin, and hypoxic-ischemic preconditioning have proven effective in HIE patients. Molecular chaperones are proteins ubiquitously present in both prokaryotes and eukaryotes. A large number of molecular chaperones are induced after brain ischemia and hypoxia, among which the heat shock proteins are the most important. Heat shock proteins not only maintain protein homeostasis; they also exert anti-apoptotic effects. Heat shock proteins maintain protein homeostasis by helping to transport proteins to their target destinations, assisting in the proper folding of newly synthesized polypeptides, regulating the degradation of misfolded proteins, inhibiting the aggregation of proteins, and by controlling the refolding of misfolded proteins. In addition, heat shock proteins exert anti-apoptotic effects by interacting with various signaling pathways to block the activation of downstream effectors in numerous apoptotic pathways, including the intrinsic pathway, the endoplasmic reticulum-stress mediated pathway and the extrinsic Fas receptor pathway. Molecular chaperones play a key role in neuroprotection in HIE. In this review, we
Rai, Rahul; Saraswat, Vivek A.; Dhiman, Radha K.
Ammonia, a key factor in the pathogenesis of hepatic encephalopathy (HE), is predominantly derived from urea breakdown by urease producing large intestinal bacteria and from small intestine and kidneys, where the enzyme glutaminases releases ammonia from circulating glutamine. Non-culture techniques like pyrosequencing of bacterial 16S ribosomal ribonucleic acid are used to characterize fecal microbiota. Fecal microbiota in patients with cirrhosis have been shown to alter with increasing Child-Turcotte-Pugh (CTP) and Model for End stage Liver Disease (MELD) scores, and with development of covert or overt HE. Cirrhosis dysbiosis ratio (CDR), the ratio of autochthonous/good bacteria (e.g. Lachnospiraceae, Ruminococcaceae and Clostridiales) to non-autochthonous/pathogenic bacteria (e.g. Enterobacteriaceae and Streptococcaceae), is significantly higher in controls and patients with compensated cirrhosis than patients with decompensated cirrhosis. Although their stool microbiota do not differ, sigmoid colonic mucosal microbiota in liver cirrhosis patients with and without HE, are different. Linkage of pathogenic colonic mucosal bacteria with poor cognition and inflammation suggests that important processes at the mucosal interface, such as bacterial translocation and immune dysfunction, are involved in the pathogenesis of HE. Fecal microbiome composition does not change significantly when HE is treated with lactulose or when HE recurs after lactulose withdrawal. Despite improving cognition and endotoxemia as well as shifting positive correlation of pathogenic bacteria with metabolites, linked to ammonia, aromatic amino acids and oxidative stress, to a negative correlation, rifaximin changes gut microbiome composition only modestly. These observations suggest that the beneficial effects of lactulose and rifaximin could be associated with a change in microbial metabolic function as well as an improvement in dysbiosis. PMID:26041954
Boniol, Scott; Boyd, Molly; Koreth, Rachel; Burton, Gary V
Thiamine deficiency can occur in any disease that results in inadequate intake or excessive loss of vitamin B1. In addition to increased thiamine consumption secondary to high cell turnover, cancer patients frequently have reduced oral intake as a direct result of their cancer or from cancer treatments. However, Wernicke encephalopathy (cerebral Beriberi), a clinical manifestation of thiamine deficiency, has rarely been associated with cancer patients. We report a case of Wernicke encephalopathy in a nonalcoholic patient with lymphoma. Although thiamine deficiency rarely potentiates clinical sequelae in cancer patients, it is important to recognize the risk and the clinical signs and manifestations so that prompt therapy can be initiated to reverse morbidity.
Ataseven, Hilmi; Yüksel, Ilhami; Gültuna, Selcan; Köklü, Seyfettin; Uysal, Serkan; Basar, Omer; Sasmaz, Nurgül
Mucormycosis is an acutely fatal infection that occurs in immuncompromised patients. Cirrhosis is an acquired immune deficiency state and those patients are more prone to develop opportunistic infections. A 42-years-old cirrhotic man was admitted to our gastroenterology clinic with hepatic encephalopathy. Although he recovered from encephalopathy with supportive measurements, he developed paresthesia on the face. He was diagnosed with rhinocerebral mucormycosis and antifungal therapy was administered. Surgical treatment couldn.t be performed because of his bleeding diathesis and poor general condition. He succumbed on the 12th day of his admission.
Parvex, P; Pinsk, M; Bell, L E; O'Gorman, A M; Patenaude, Y G; Gupta, I R
Neurological complications post transplant have been described with the use of calcineurin inhibitors. Although tacrolimus may be a better immunosuppressant than cyclosporine, its neurological side effects may be worse. Two children, living-related kidney transplant recipients, were treated with antibody induction, mycophenolate mofetil, prednisone, and tacrolimus. Soon after transplant, they each developed an encephalopathy, which when visualized by magnetic resonance imaging showed that it affected both white and grey matter of the brain. Although the encephalopathy was associated with the use of tacrolimus, there was a complete neurological recovery without cessation of the drug.
Sahoo, Tanushree; Thukral, Anu; Agarwal, Ramesh; Sankar, Mari Jeeva
Galactosaemia is a disorder of galactose metabolism in which raised levels of galactose and galactose-1-phosphate damage various organs. Although galactosaemia is a common metabolic liver disease in childhood, it is a rare cause of neonatal hyperbilirubinemia requiring intervention. We report an unusual case of neonatal galactosaemia that at presentation had features of acute bilirubin encephalopathy requiring exchange transfusion and at discharge had features of chronic bilirubin encephalopathy. This case report emphasises the need for timely suspicion and diagnosis of this disease for prevention of chronic morbidity.
Lenz, V; Vargas, M I; Bin, J F; Bogorin, A; Grebici-Guessoum, M; Jacques, C; Marin, H; Zöllner, G; Dietemann, J L
Wernicke encephalopathy (Wernicke-Korsakoff encephalopathy) is related to thiamine deficiency. We report the MRI findings in four patients with visualization of bilateral and symmetrical hyperintense foci on T2W and FLAIR images involving the periaqueductal gray matter, the mamillary bodies and around the third ventricle. Diffusion weighted images obtained in two patients demonstrated mild hypersignal in the same areas. Contrast enhancement within the mamillary bodies was noted in one patient. Follow-up MRI obtained in three patients showed rapid regression of signal abnormalities without correlation with good clinical outcome.
BenHamida, Emira; Ayadi, Imene; Ouertani, Ines; Chammem, Maroua; Bezzine, Ahlem; BenTmime, Riadh; Attia, Leila; Mrad, Ridha; Marrakchi, Zahra
Perinatal-lethal Gaucher disease is very rare and is considered a variant of type 2 Gaucher disease that occurs in the neonatal period. The most distinct features of perinatal-lethal Gaucher disease are non-immune hydrops fetalis. Less common signs of the disease are hepatosplenomegaly, ichthyosis and arthrogryposis. We report a case of Gaucher's disease (type 2) diagnosed in a newborn who presented with Hydrops Fetalis. PMID:26327947
Sit, Dorothy; Perel, James M.; Wisniewski, Stephen R.; Helsel, Joseph C.; Luther, James F.; Wisner, Katherine L.
Objectives The authors explored the relationship of cord-maternal antidepressant concentration ratios and maternal depression with perinatal events and preterm birth. Method The investigators examined 21 mother-infant pairs with antidepressant exposure during pregnancy. The antidepressants included serotonergic reuptake inhibitors (SRI) and nortriptyline (noradrenergic inhibitor and mild SRI). The mothers were evaluated with the Structured Clinical Interview for DSM-IV. Depression ratings were repeated at 20, 30 and 36 weeks pregnancy. At delivery, investigators assessed cord and maternal antidepressant concentrations, neonatal outcomes on the Peripartum Events Scale (PES) and gestational weeks at birth. Results Mean cord-to-maternal concentration ratios were 0.52±0.35 (0.08–1.64) - parent drug and 0.54±0.17 (0.28–0.79) - metabolite. Nine of 21 mothers (43%) had a major depressive episode. From examining the maximum depression ratings, the mean SIGH-ADS score was 16.0±7.6. One-third (7/21) of infants had at least one perinatal event (PES≥1). The frequency of deliveries complicated by perinatal event(s) was similar in depressed and non-depressed mothers. There was no significant association between perinatal events and cord-to-maternal antidepressant concentration ratios or maternal depression levels. Exposure to short half-life antidepressants compared to fluoxetine resulted in more perinatal events (7/16 =44% vs 0/5=0%; p=0.06). Fourteen percent (3/21) of infants were preterm. Preterm birth was not associated with cord-to-maternal metabolite concentration ratios, depression levels or exposure to fluoxetine. Conclusion Antidepressant-exposed infants experienced a limited number of transient perinatal events. No association between cord-maternal concentration ratios or maternal depression and perinatal events could be identified. Contrary to other reports, we detected no increased risk for perinatal events with fluoxetine therapy compared to the short half
Henderson, Phillip K; Herrera, Jorge L
Hepatic encephalopathy exists along a continuum from abnormal neuropsychiatric testing in the absence of clinical findings to varying degrees of detectable clinical findings. The International Society for Hepatic Encephalopathy and Nitrogen Metabolism has endorsed the term "covert" to encompass minimal hepatic encephalopathy and grade I overt hepatic encephalopathy. Covert hepatic encephalopathy has been associated with poor quality of life, decreased employment, increased falls, and increased traffic accidents that significantly impact quality of life and health care expenditures. Probiotics, nonabsorbable dissacharides, rifaximin, and l-ornithine-l-aspartate have been evaluated with varying levels of success. Because of the lack of universally accepted diagnostic tools, optimal timing of testing and treatment remains controversial.
Suraweera, Duminda; Sundaram, Vinay; Saab, Sammy
Hepatic encephalopathy is a spectrum of neurocognitive manifestations often seen in patients with liver injury or rarely in patients with portosystemic shunting without liver injury. It can be divided into minimal (covert) hepatic encephalopathy and overt hepatic encephalopathy, depending on the severity. Patients with hepatic encephalopathy have compromised clinical outcomes, decreased quality of life, and increased healthcare utilization, often resulting in a heavy financial and personal burden on caregivers. The diagnosis remains largely clinical, with the exclusion of possible other causes for the altered mental status. Current treatment strategies include nonabsorbable disaccharides and antibiotics. This review will focus on the diagnosis, management and clinical impact of hepatic encephalopathy.
Suraweera, Duminda; Sundaram, Vinay; Saab, Sammy
Hepatic encephalopathy is a spectrum of neurocognitive manifestations often seen in patients with liver injury or rarely in patients with portosystemic shunting without liver injury. It can be divided into minimal (covert) hepatic encephalopathy and overt hepatic encephalopathy, depending on the severity. Patients with hepatic encephalopathy have compromised clinical outcomes, decreased quality of life, and increased healthcare utilization, often resulting in a heavy financial and personal burden on caregivers. The diagnosis remains largely clinical, with the exclusion of possible other causes for the altered mental status. Current treatment strategies include nonabsorbable disaccharides and antibiotics. This review will focus on the diagnosis, management and clinical impact of hepatic encephalopathy. PMID:27377741
Roberts, Drucilla J
The practice of perinatal pathology in much of the world suffers, as do all subspecialties of anatomic pathology, from inadequate resources (equipment, consumables, and both professional and technical personnel), from lack of education (not only of the pathologist but also of the clinicians responsible for sending the specimens, and the technicians processing the specimens), and from lack of appropriate government sector support. Perinatal pathology has significant public health-related utility and should be championing its service by providing maternal and fetal/infant mortality and morbidity data to governmental health ministries. It is with this pathologic data that informed decisions can be made on health-related courses of action and allocation of resources. These perinatal pathology data are needed to develop appropriate public health initiatives, specifically toward achieving the Millennium Developmental Goals as the best way to effectively decrease infant and maternal deaths and to determine causes of perinatal mortality and morbidity. The following overview will focus on the utility of perinatal pathology specifically as related to its public health function and will suggest methods to improve its service in resource-poor settings. This article is offered not as a critique of the current practice that most pathologists find themselves working in globally, but to provide suggestions for improving perinatal pathology services, which could be implemented with the limited available resources and manpower most pathology departments currently have. In addition, we offer suggestions for graded improvements ("ramping up") over time.
Taha, T. E.; Gray, R. H.
Hospital- and community-based studies were conducted in central Sudan to investigate the association between pesticide exposure and perinatal mortality. The cases were 197 stillbirths in the hospital and 36 perinatal deaths in the community; the controls were 812 liveborn, normal-birth-weight infants in the hospital, and 1505 liveborn infants who survived for the first 7 days after birth in the community. The odds ratio (OR) of perinatal death associated with pesticide exposure was estimated using multiple logistic regression. There was a consistent and significant association between pesticide exposure and perinatal mortality in the hospital (adjusted OR = 1.9; 95% confidence interval (CI): 1.3-2.8) and the community populations (adjusted OR = 2.7; 95% CI: 1.1-6.4). The OR was significantly higher among women engaged in farming (3.6; 95% CI: 1.6-8.0), but not among women in nonfarming occupations (1.6; 95% CI: 0.8-3.3). The estimated attributable risks of perinatal death owing to pesticide exposure were 22.6% for hospital stillbirths and 15.7% for community perinatal deaths; but among women engaged in farming in the hospital population the attributable risks were substantially higher (34.5%). PMID:8324850
Vitonis, Allison; Zou, Liying; Ruan, Yan; Wang, Xin; Zhang, Weiyuan
Background Cesarean delivery (CD) rates have risen globally with nearly 50% of the non-indicated CDs worldwide in China and Brazil. In China’s One Child Policy era (1979–2015) most deliveries were women having their only child. Family size is a major determinant of the safety of medically non-indicated CD or CD on maternal request. The goal of this study is to document CD rates, indications, and analyze the relative safety of non-indicated CD compared to SVD and intrapartum CD. Methods Univariate and multivariate logistic regression analyses of the association between mode of delivery and short-term maternal and perinatal outcomes were performed on a cross-section of all deliveries at 39 hospitals in 14 provinces of China in 2011, presented as adjusted odds ratio (aOR), 99% confidence intervals (CI). Findings Among 108,847 deliveries, 59,415 were CD (54.6%) with 20.8% of deliveries or 38.2% of all cesareans were non-indicated CD. Compared to SVD, antepartum non-indicated CD was associated with a decreased likelihood of post-partum hemorrhage (PPH) (aOR = 0.80, CI = 0.69–0.92) and was not associated with maternal death or combined severe outcomes (maternal death, transfusion, or hysterectomy). Intrapartum indicated CD was associated with an increased risk of PPH (aOR = 1.68, CI = 1.50–1.89) compared to SVD. Compared to SVD, antepartum non-indicated CD was associated with lower likelihood of neonatal death (aOR = 0.14, CI = 0.06–0.34), neonatal ICU admission (aOR = 0.50, CI = 0.36–0.69), 5-minute Apgar<4 (aOR = 0.06, CI = 0.10–0.36), and respiratory distress syndrome (RDS) (aOR = 0.31, CI = 0.16–0.58), but not significantly associated with changes in rates of infection, hypoxic ischemic encephalopathy (HIEE), birth trauma or meconium aspiration rates. Conclusions In 2011 when 81% of deliveries were women having their first child antepartum non-indicated CD had short-term maternal and perinatal outcomes as safe as SVD. Now that all Chinese women can
Professionals in many different occupations, from psychiatrists, obstetricians, and pediatricians to nurses, midwives, pharmacists, clinical psychologists, public health nurses, and psychiatric social workers, are involved in perinatal mental healthcare. In order to enhance the function of such healthcare, it is necessary both to provide specialized training in each occupation and form a system and to smoothly conduct medical collaboration between different occupations. A deficiency in the medical function of perinatal mental healthcare greatly influences the mother and child's health, mental hygiene, and social life later in life. Therefore, a demand is seen for specialized staff and system formation capable of the following: 1) responding with appropriate perinatal management of female patients taking psychotropic drugs; 2) providing support and pregnancy consultation to female patients who wish to have children; and 3) properly handling postpartum mental disorder management, possibility of breastfeeding, and various issues that arise in mother-child relationships during upbringing. In the UK, the clinical guideline (NICE Clinical Guideline 45) for perinatal mental healthcare, which was created by the National Institute for Clinical Excellence (NICE), provides important guidelines on how to handle perinatal mental health. Aside from the NICE guideline, the Maudsley Prescribing Guidelines in Psychiatry indicates basic guidelines on prescribing perinatal drug therapy. In Japan, however, the current situation of perinatal mental healthcare is such that it has yet to be systemically developed. In this paper, we introduce the basic content in these British guidelines that should be noted. In addition, we consider the current status and future disposition of Japan's perinatal mental healthcare, with consideration for the differences in healthcare circumstances between Japan and the UK.
Nguyen, Douglas L; Morgan, Timothy
Since the late nineteenth century, protein restriction has been shown to improve hepatic encephalopathy. However, malnutrition has been described in up to 60 % of cirrhotic patients and is associated with increased mortality. Furthermore, emerging clinical evidence has revealed that a large proportion of cirrhotic patients may tolerate normal protein intake. However, approximately one third of cirrhotic patients with hepatic encephalopathy may need a short course of protein restriction, in addition to maximum medical therapy, to ameliorate the clinical course of their hepatic encephalopathy. For patients with chronic hepatic encephalopathy who are protein-sensitive, modifying their sources of nitrogen by using more vegetable protein, less animal protein, and branched-chain amino acids may improve their encephalopathy without further loss of lean body mass. In conclusion, among cirrhotics with hepatic encephalopathy, modulation of normal protein intake must take into account the patient's hepatic reserve, severity of hepatic encephalopathy, and current nutritional status.
Bovine spongiform encephalopathy (BSE) had never been detected in Sweden until 2006, when the active surveillance identified a case in a 12-year-old cow. The case was an unusual form since several molecular features of the protease-resistant prion protein (PrP**res) were different from classical BSE...
Tartara, Elena; Fanucchi, Simona; D'Errico, Ignazio; Farina, Lisa M; Casoni, Francesca; Sinforiani, Elena; Micieli, Giuseppe; Costa, Alfredo
There have been several reports of disulfiram intoxication, but little evidence of neurologic conditions resulting from disulfiram-induced brain damage combined with Wernicke encephalopathy-associated lesions. We report a rare patient with both Wernicke encephalopathy and disulfiram intoxication. This 50-year-old woman, who was taking disulfiram for chronic alcohol abuse, presented with an acute confusional state, dysarthria, nystagmus, supranuclear ophthalmoplegia, and paraparesis. Biochemical serum and cerebrospinal fluid analyses were normal. An electromyogram detected a motor polyneuropathy. Cognitive assessment revealed severe impairment of memory, attention, and logical and executive abilities. Magnetic resonance imaging with gadolinium enhancement showed brain lesions consistent with Wernicke encephalopathy, but also symmetric hyperintensities on T2-weighted images in the globus pallidus. Stopping the disulfiram and treating with hydration, high-dose thiamine supplements, and benzodiazepines significantly improved the patient's consciousness and oculomotor function. A magnetic resonance imaging scan after 1 month of treatment showed complete disappearance of the brain lesions and the hyperintensities in the globus pallidus. After a further month of intensive neurorehabilitation, the patient was able to interact with the medical staff, and her neuropsychological tests showed only mild memory impairment. Patients with alcoholism who present at emergency departments are at high risk for misdiagnosis, especially because there is no specific routine laboratory test for detecting asymptomatic disulfiram intoxication. Although uncommon, the combination of Wernicke encephalopathy and disulfiram intoxication should be suspected in patients with alcoholism. The disorder can be detected through a careful history and prompt clinical evaluation, together with characteristic magnetic resonance imaging findings.
Matsushita, M; Yamamoto, T; Gemba, H
Thioacetamide (TAA), a hepatotoxin used to ascertain the role of astrocytes in hepatic encephalopathy, was administered to prepare four experimental groups of rats. (The TAA1D, TAA1.5D, TAA2D, and TAA2.5D group rats were perfusion fixated with formalin at 1, 1.5, 2, and 2.5 days, respectively, after initial administration of TAA. In addition, TAA was readministered to the TAA2D and TAA2.5D rats 24 h after the first dose.) Abnormalities of higher brain function and equilibrium that progressed with time were apparent in the rats receiving TAA. On the other hand, innate reflexes (e.g. pupillary reflex) were similar to those in the normal control group. Astrocyte cell areas in the hippocampus, neocortex, hypothalamus, cerebellum, and basal ganglia (striatum) from the TAA rats were significantly larger than in corresponding sites from the normal rats (maximum in TAA1D and TAA1.5D groups). However, there were no differences with respect to the midbrain. Any morphological difference was not observed in neurons between the hepatic encephalopathy and normal rats. Administration of TAA caused hepatic tissue injury that progressed over time. Surprisingly, encephalopathy was apparent even when hepatic injury was mild. These findings suggest that abnormalities in astrocytes, which precede any abnormal change in neurons, play a role in the development of hepatic encephalopathy.
Ransing, Ramdas Sarjerao; Mishra, Kshirod Kumar; Sarkar, Dipayan
Hashimoto's encephalopathy is usually underdiagnosed and untreated because of complex neuropsychiatric manifestation. We report a case of an adolescent female with Hashimoto's encephalopathy who responded well to a combination of aspirin and levothyroxine. A 16-year-old girl presented at psychiatric emergency services with a depressive episode, menstrual irregularities, and a 5-month past history of thyroid swelling. On clinical examination, she was in a euthyroid state with insignificant neurological history. However, her previous investigation revealed a hypothyroid state. Her magnetic resonance imaging findings demonstrated infarcts in the bilateral gangliocapsular region and left frontal periventricular deep white matter lesion. Ultrasonography of the thyroid and fine needle aspiration cytology confirmed lymphocytic thyroiditis. Anti-thyroid peroxidase (289 IU/ml) antibody titer was elevated (289 IU/mL). Her depressive symptoms responded well to antidepressants, mood stabilizers, nonsteroidal anti-inflammatory drugs, and levothyroxine. She remained in the euthyroid state and then in the euthymic state for 3 years. Hashimoto's encephalopathy is steroid-responsive encephalopathy. Most researchers have observed a dramatic response to steroids with or without levothyroxine. A clinician may consider aspirin as an alternative to a steroid in long-term management to avoid steroid-related side effects and contraindications. PMID:27570351
Park, So Won; Yi, Yoon Young; Han, Jung Woo; Kim, Heung Dong; Lee, Joon Soo
Wernicke's encephalopathy is an acute neurological disorder characterized by mental confusion, oculomotor dysfunction, and ataxia. It has been reported in individuals with alcohol dependence, hyperemesis gravidarum, and prolonged parenteral nutrition without vitamin supplementation. Here we present the case of a 13-year-old male patient with neuroblastoma and a history of poor oral intake and nausea for 3 months. After admission, he showed gait disturbances, nystagmus, and excessive dizziness; his mental state, however, indicated he was alert, which did not fit the classical triad of Wernicke's encephalopathy. A diagnosis of Wernicke's encephalopathy was made only after brain magnetic resonance imaging and serum thiamine level analyses were performed. The patient's symptoms remained after 5 days of treatment with 100-mg thiamine once daily; thus, we increased the dosage to 500 mg 3 times daily, 1,500 mg per day. His symptoms then improved after 20 days of replacement therapy. This case report describes a pediatric patient who was promptly diagnosed with Wernicke's encephalopathy, despite only 2 suspicious symptoms, and who completely recovered after high doses of thiamine were given intravenously. PMID:25550705
Salpietro, Vincenzo; Mankad, Kshitij; Polizzi, Agata; Sugawara, Yuji; Granata, Francesca; David, Emanuele; Ferraù, Valeria; Gallizzi, Romina; Tortorella, Gaetano; Ruggieri, Martino
Hashimoto encephalopathy is a syndrome of encephalopathy associated with elevated concentration of circulating serum anti-thyroid antibodies usually responsive to steroid therapy. We report a 13-year-old girl with Hashimoto encephalopathy and peripheral nervous system involvement. The child had experienced high-grade pyrexia, global headache and sleeplessness. After admission she had an ileus with a distended urinary bladder, hallucinations and cognitive impairment. She had reduced deep tendon reflexes and distal sensory deficiency. Anti-thyroglobulin antibodies were raised at 2121 IU/mL (normal, 0-40) and the anti-thyroperoxidase was high at 886 IU/mL (normal, 0-50). Progressive neurological and psychiatric remission was noted after i.v. methylprednisolone. Follow-up magnetic resonance imaging showed complete resolution of the foci of signal abnormality previously yielded. This case report is the first, to the best of our knowledge, to describe peripheral nervous system involvement in a child with a diagnosis of Hashimoto's encephalopathy.
Boogerd, W; Zoetmulder, F A; Moffie, D
A patient is described with a severe encephalopathy and hyperammonemia in absence of liver dysfunction, attributed to urine absorption into the systemic circulation due to suture line breakdown after bladder dome resection. At autopsy characteristic Alzheimer type II astrocytes were found in the basal ganglia.
Biacabe, Anne-Gaëlle; Morignat, Eric; Vulin, Johann; Calavas, Didier; Baron, Thierry G M
In France, through exhaustive active surveillance, approximately 17.1 million adult cattle were tested for bovine spongiform encephalopathy from July 2001 through July 2007; approximately 3.6 million were >8 years of age. Our retrospective Western blot study of all 645 confirmed cases found that 7 were H-type and 6 were L-type.
Emeksiz, Serhat; Kutlu, Nurettin Onur; Çaksen, Hüseyin; Alkan, Gülsüm; Yıkmaz, Hülya Şeker; Tokgöz, Hüseyin
Posterior reversible encephalopathy syndrome is characterized by hypertension, seizure, headache, clouding of consciousness, and visual disturbance, and is diagnosed in the presence of typical lesions on magnetic resonance imaging. We retrospectively evaluated five patients who were diagnosed as having posterior reversible encephalopathy syndrome and followed up in Meram Medical Faculty, Pediatric Intensive Care and Hematology wards, between January 2010 and January 2014. We reviewed the demographic and clinical data, and neuroimaging findings. The primary diseases of the subjects included acute lymphocytic leukemia (n=2), Henoch-Schönlein purpura (n=1), systemic lupus erythematous (n=1), and acute poststreptococcal glomerulonephritis (n=1). The mean age was 10±4.58 years (range, 5–14 years). Acute elevation of blood pressure was found in all patients (n=5). Initial neurologic manifestations included seizure, clouding of consciousness, headache, and visual disturbance. After the diagnosis was made through clinical evaluations and magnetic resonance imaging, complete clinical recovery was obtained in all patients with the appropriate therapeutic approach. In conclusion, posterior reversible encephalopathy syndrome should be considered in the differential diagnosis of patients who present with encephalopathy and underlying diseases such as nephritis, vasculitis, malignancy accompanied by hypertension, and a history of use of medication. PMID:28123335
cumulative head trauma. All operational aspects of this study have been accomplished including local IRB approval, identification of potential... head injuries sustained in battle have been associated with the development of chronic traumatic encephalopathy (CTE). Pathological series have...will examine whether FDDNP PET imaging correlates with, and/or can predict, decline in cognitive function in those exposed to cumulative head trauma
Ransing, Ramdas Sarjerao; Mishra, Kshirod Kumar; Sarkar, Dipayan
Hashimoto's encephalopathy is usually underdiagnosed and untreated because of complex neuropsychiatric manifestation. We report a case of an adolescent female with Hashimoto's encephalopathy who responded well to a combination of aspirin and levothyroxine. A 16-year-old girl presented at psychiatric emergency services with a depressive episode, menstrual irregularities, and a 5-month past history of thyroid swelling. On clinical examination, she was in a euthyroid state with insignificant neurological history. However, her previous investigation revealed a hypothyroid state. Her magnetic resonance imaging findings demonstrated infarcts in the bilateral gangliocapsular region and left frontal periventricular deep white matter lesion. Ultrasonography of the thyroid and fine needle aspiration cytology confirmed lymphocytic thyroiditis. Anti-thyroid peroxidase (289 IU/ml) antibody titer was elevated (289 IU/mL). Her depressive symptoms responded well to antidepressants, mood stabilizers, nonsteroidal anti-inflammatory drugs, and levothyroxine. She remained in the euthyroid state and then in the euthymic state for 3 years. Hashimoto's encephalopathy is steroid-responsive encephalopathy. Most researchers have observed a dramatic response to steroids with or without levothyroxine. A clinician may consider aspirin as an alternative to a steroid in long-term management to avoid steroid-related side effects and contraindications.
Wedisinghe, Lilantha; Jayakody, Kaushadh; Arambage, Kirana
Wernicke's encephalopathy is a rare cause of maternal death. It is a difficult diagnosis to make but prevention and treatment is straightforward. Severe thiamine deficiency causes Wernicke-Korsakoff syndrome. Correct diagnosis and treatment with thiamine will decrease the case fatality rate.
Aravamuthan, Bhooma R.; Shoykhet, Michael
BACKGROUND The basal ganglia are vulnerable to injury during cardiac arrest. Movement disorders are a common morbidity in survivors. Yet, neuronal motor network changes post-arrest remain poorly understood. METHODS We compared function of the motor network in adult rats that, during postnatal week 3, underwent 9.5 min of asphyxial cardiac arrest (n = 9) or sham intervention (n = 8). Six months after injury, we simultaneously recorded local field potentials (LFP) from the primary motor cortex (MCx) and single neuron firing and LFP from the rat entopeduncular nucleus (EPN), which corresponds to the primate globus pallidus pars interna. Data were analyzed for firing rates, power, and coherence between MCx and EPN spike and LFP activity. RESULTS Cardiac arrest survivors display chronic motor deficits. EPN firing rate is lower in cardiac arrest survivors (19.5 ± 2.4 Hz) compared with controls (27.4 ± 2.7 Hz; P < 0.05). Cardiac arrest survivors also demonstrate greater coherence between EPN single neurons and MCx LFP (3—100 Hz; P < 0.001). CONCLUSIONS This increased coherence indicates abnormal synchrony in the neuronal motor network after cardiac arrest. Increased motor network synchrony is thought to be antikinetic in primary movement disorders. Characterization of motor network synchrony after cardiac arrest may help guide management of post-hypoxic movement disorders. PMID:26083760
Combs, Vélvá M; Crispell, Heather D; Drew, Kelly L
Stimulation of N-methyl-D-aspartate receptors (NMDAR) contributes to regenerative neuroplasticity following the initial excitotoxic insult during cerebral ischemia. Stimulation of NMDAR with the partial NMDAR agonist D-cycloserine (DCS) improves outcome and restores hippocampal synaptic plasticity in models of closed head injury. We thus hypothesized that DCS would improve outcome following restoration of spontaneous circulation (ROSC) from cardiac arrest (CA). DCS (10 mg/kg, IP) was administered to Sprague-Dawley rats (male, 250-330 g; 63-84 days old) 24 and 48 hours after 6 or 8 minutes of asphyxial CA. Heart rate and blood pressure declined similarly in all groups. Animals showed neurological deficits after 6 and 8 minutes CA (P<0.05, Tukey) and these deficits recovered more quickly after 6 minutes than after 8 minutes of CA. CA decreased the number of healthy neurons within CA1 with no difference between 6 and 8 minutes duration of CA (180.8±27.6 (naïve, n=5) versus 46.3±33.8 (all CA groups, n=27) neurons per mm CA1). DCS had no effect on neurological deficits or CA1 hippocampal cell counts (P>0.05, Tukey).
Shaik, Jafar Sadik B; Poloyac, Samuel M; Kochanek, Patrick M; Alexander, Henry; Tudorascu, Dana L; Clark, Robert Sb; Manole, Mioara D
Vasoconstrictive and vasodilatory eicosanoids generated after cardiac arrest (CA) may contribute to cerebral vasomotor disturbances and neurodegeneration. We evaluated the balance of vasodilator/vasoconstrictor eicosanoids produced by cytochrome P450 (CYP) metabolism, and determined their role on cortical perfusion, functional outcome, and neurodegeneration after pediatric asphyxial CA. Cardiac arrest of 9 and 12 minutes was induced in 16- to 18-day-old rats. At 5 and 120 minutes after CA, we quantified the concentration of CYP eicosanoids in the cortex and subcortical areas. In separate rats, we inhibited 20-hydroxyeicosatetraenoic acid (20-HETE) synthesis after CA and assessed cortical cerebral blood flow (CBF), neurologic deficit score, neurodegeneration, and edema. After 9 minutes of CA, vasodilator eicosanoids markedly increased versus sham. Conversely, after 12 minutes of CA, vasoconstrictor eicosanoid 20-HETE increased versus sham, without compensatory increases in vasodilator eicosanoids. Inhibition of 20-HETE synthesis after 12 minutes of CA decreased cortical 20-HETE levels, increased CBF, reduced neurologic deficits at 3 hours, and reduced neurodegeneration and edema at 48 hours versus vehicle-treated rats. In conclusion, cerebral vasoconstrictor eicosanoids increased after a pediatric CA of 12 minutes. Inhibition of 20-HETE synthesis improved cortical perfusion and short-term neurologic outcome. These results suggest that alterations in CYP eicosanoids have a role in cerebral hypoperfusion and neurodegeneration after CA and may represent important therapeutic targets.
Markus, Hugh Stephen
Background Migraine is common in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) but its treatment responses are not well described, and its relationship to stroke risk unknown. Encephalopathy is a less common presentation; it has been suggested it is related to migraine. We characterised migraine patterns and treatment responses in CADASIL, and examined associations between migraine and both stroke risk and encephalopathy. Methods 300 symptomatic CADASIL patients were prospectively recruited from a national referral clinic over a nineteen year period, from 1996 to 2015. Data was collected using a standardised questionnaire. Migraine was classified according to the International Classification of Headache Disorders, 3rd edition (beta version). A cross-sectional analysis was carried out on the data collected. Results Migraine was present in 226 (75.3%), and the presenting feature in 203 (67.7%). It was usually accompanied by aura (89.8%). Patients showed variable responses to a variety of drugs for migraine. Of 24 given triptans, 45.5% had consistent or partial responses. None had complications following triptans. Thirty-three (11.0%) patients experienced encephalopathy lasting on average 8.1 ± 3.4 days. Patients with migraine with aura had higher odds of encephalopathy (OR = 5.4; 95%CI 1.6–28.4; p = 0.002). Patients with confusional aura had higher odds of encephalopathy than those with other aura types (OR = 2.5, 95%CI = 1.0–5.8, p = 0.04). There was also no increase in risk of encephalopathy with sex or age at onset of migraine. Migraineurs had a lower stroke risk than non-migraineurs (HR = 0.46, 95%CI 0.3–0.6, p = 2.1x10-6). Conclusions Migraine with aura is a prominent feature of CADASIL. Treatment responses are similar to those seen in the general migraine population and no complications were observed with triptans. Migraine with aura was associated with increased risk of encephalopathy suggesting
Guo, Lili; Wang, Dehang; Bo, Genji; Zhang, Hui; Tao, Weijing; Shi, Ying
Brain damage following a perinatal hypoxic-ischemic encephalopathy (HIE) can be diagnosed by different techniques. The aim of the present study was to combine magnetic resonance (MR) imaging with proton MR spectroscopy in HIE diagnosis and to evaluate their correlation with outcome. A prospective observational cohort study was performed between February 2012 and February 2013. Consecutive newborns, 24 full-term neonates with HIE (mild to moderate and severe group) and 5 normal neonates, were included. Two sequential MR studies were performed; a conventional MR imaging for observation in T1 weighted image (WI) and T2WI, and proton MR spectroscopy for observation in the left or right basal ganglia and thalamus. MR images were assessed and scored by two neuroradiologists who were blinded to the clinical condition of the infants. The mild to moderate group (n=13) and severe group (n=11) were similar in the visualization of punctate hyperintensity lesions on T1WI and brain edema on T2WI. The differences of N-acetylaspartate/creatine (Cr), choline/Cr and lactate/Cr in the basal ganglia and thalamus in the HIE group were significantly different (P<0.05) compared with the control group, while no significant difference was identified between the mild to moderate and severe group (P>0.05). In conclusion, MR spectroscopy is a complementary tool for the diagnosis of HIE. PMID:27882082
Bale, Gemma; Mitra, Subhabrata; de Roever, Isabel; Chan, Marcus; Caicedo-Dorado, Alexander; Meek, Judith; Robertson, Nicola; Tachtsidis, Ilias
Perinatal hypoxic ischaemic encephalopathy (HIE) is associated with severe neurodevelopmental problems and mortality. There is a clinical need for techniques to provide cotside assessment of the injury extent. This study aims to use non-invasive cerebral broadband near-infrared spectroscopy (NIRS) in combination with systemic physiology to assess the severity of HIE injury. Broadband NIRS is used to measure the changes in haemodynamics, oxygenation and the oxidation state of cytochrome c oxidase (oxCCO). We used canonical correlation analysis (CCA), a multivariate statistical technique, to measure the relationship between cerebral broadband NIRS measurements and systemic physiology. A strong relationship between the metabolic marker, oxCCO, and systemic changes indicated severe brain injury; if more than 60 % of the oxCCO signal could be explained by the systemic variations, then the neurodevelopmental outcome was poor. This boundary has high sensitivity and specificity (100 and 83 %, respectively). Broadband NIRS measured concentration changes of the oxidation state of cytochrome c oxidase has the potential to become a useful cotside tool for assessment of injury severity following hypoxic ischaemic brain injury.
Guo, Lili; Wang, Dehang; Bo, Genji; Zhang, Hui; Tao, Weijing; Shi, Ying
Brain damage following a perinatal hypoxic-ischemic encephalopathy (HIE) can be diagnosed by different techniques. The aim of the present study was to combine magnetic resonance (MR) imaging with proton MR spectroscopy in HIE diagnosis and to evaluate their correlation with outcome. A prospective observational cohort study was performed between February 2012 and February 2013. Consecutive newborns, 24 full-term neonates with HIE (mild to moderate and severe group) and 5 normal neonates, were included. Two sequential MR studies were performed; a conventional MR imaging for observation in T1 weighted image (WI) and T2WI, and proton MR spectroscopy for observation in the left or right basal ganglia and thalamus. MR images were assessed and scored by two neuroradiologists who were blinded to the clinical condition of the infants. The mild to moderate group (n=13) and severe group (n=11) were similar in the visualization of punctate hyperintensity lesions on T1WI and brain edema on T2WI. The differences of N-acetylaspartate/creatine (Cr), choline/Cr and lactate/Cr in the basal ganglia and thalamus in the HIE group were significantly different (P<0.05) compared with the control group, while no significant difference was identified between the mild to moderate and severe group (P>0.05). In conclusion, MR spectroscopy is a complementary tool for the diagnosis of HIE.
Capitulo, Kathleen Leask
The purpose of this article is to explore the concept of perinatal grief and evidence-based healing interventions for it. The loss of a pregnancy or death of an infant causes profound grief, yet society has long minimized or ignored this grief, which is among the most painful of bereavement experiences. Throughout the last century, research on grief and the special needs of bereaved parents has changed the context of professional intervention from protective to supportive. The central focus of bereavement interventions is to assist families in healing by helping them make meaning of their losses. The use of symbols, spirituality, and rituals has been shown to help bring meaning. Research has shown that memories are key to healing, and that gender, age, and relationships bring different grief expressions and experiences. While children's understanding of loss and grief differs with developmental age, they should also be given the opportunity to participate in grief rituals and practices. Professionals who care for bereaved parents have a unique opportunity to offer support by validating their grief, facilitating rituals, providing mementos, and letting the bereaved tell their stories. While no intervention can bring back their beloved children, appropriate intervention can promote healing.
Bobić, Mirna Vuković; Habek, Dubravko; Habek, Jasna Čerkez
In the present study, the impact of the potential perinatal epidemiological factors on preeclampsia development was assessed. This clinical study included 55 pregnant women with preeclampsia and control group of 50 healthy pregnant women. Positive family history of cardiovascular disease, diabetes mellitus or thromboembolic disease was recorded in 50% of women with preeclampsia versus 28% of control group women. Positive personal history of this disease was recorded in 15% of women with preeclampsia, whereas all control group women had negative personal history of preeclampsia. Dietary habits, i.e. the intake of meat and meat products, fruit and vegetables, coffee and alcohol drinks were similar in the two groups, without statistically significant differences. The women with preeclampsia and control women reported comparable habits; there was no difference in the consumption of meat, fruit, vegetables, coffee and alcohol, smoking, use of folate and oral hormonal contraception before pregnancy, or in physical activity as the potential risk factors for preeclampsia in current pregnancy. However, personal and family history of vascular disease proved to be significant risk factors for the occurrence of preeclampsia, emphasizing the need of lifestyle and dietary modifications with healthy dietary habits, while avoiding adverse habits in pregnancy.
Mercuri, E; Atkinson, J; Braddick, O; Anker, S; Nokes, L; Cowan, F; Rutherford, M; Pennock, J; Dubowitz, L
AIMS: To evaluate the visual function of infants with perinatal cerebral infarction in whom the site and size of the lesion has been determined using magnetic resonance imaging (MRI). METHODS: Twelve infants with cerebral infarction on MRI were studied with a battery of tests specifically designed to evaluate visual function in infancy. This included tests: for visual attention (fixation shifts); of cerebral asymmetry (optokinetic nystagmus, visual fields); for assessment of acuity (forced choice preferential looking); and neurophysiological measures of vision (phase reversal and orientation reversal visual evoked potential). RESULTS: A considerable incidence of abnormalities on at least one of the tests for visual function used was observed. The presence or severity of visual abnormalities could not always be predicted by the site and extent of the lesion seen on imaging. CONCLUSIONS: Early focal lesions affecting the visual pathway can, to some extent, be compensated for by the immature developing brain. These data suggest that all the infants presenting with focal lesions need to be investigated with a detailed assessment of various aspects of vision. Images PMID:8949687
Fodor, Anna; Tímár, Júlia; Zelena, Dóra
Opioids are among the world's oldest known drugs used mostly for pain relief, but recreational use is also widespread. A particularly important problem is opioid exposure in females, as their offspring can also be affected. Adverse intrauterine and postnatal environments can affect offspring development and may lead to various disabilities later in life. It is clear that repetitive painful experiences, such as randomly occurring invasive procedures during neonatal intensive care, can permanently alter neuronal and synaptic organization and therefore later behavior. At the same time, analgesic drugs can also be harmful, inducing neuronal apoptosis or withdrawal symptoms in the neonate and behavioral alterations in adulthood. Hence, risk-benefit ratios should be taken into consideration when pain relief is required during pregnancy or in neonates. Recreational use of opioids can also alter many aspects of life. Intrauterine opioid exposure has many toxic effects, inducing poor pregnancy outcomes due to underdevelopment, but it is believed that later negative consequences are more related to environmental factors such as a chaotic lifestyle and inadequate prenatal care. One of the crucial components is maternal care, which changes profoundly in addicted mothers. In substance-dependent mothers, pre- and postnatal care has special importance, and controlled treatment with a synthetic opioid (e.g., methadone) could be beneficial. We aimed to summarize and compare human and rodent data, as it is important to close the gap between scientific knowledge and societal policies. Special emphasis is given to gender differences in the sensitivity of offspring to perinatal opioid exposure.
Meisler, Miriam H; Helman, Guy; Hammer, Michael F; Fureman, Brandy E; Gaillard, William D; Goldin, Alan L; Hirose, Shinichi; Ishii, Atsushi; Kroner, Barbara L; Lossin, Christoph; Mefford, Heather C; Parent, Jack M; Patel, Manoj; Schreiber, John; Stewart, Randall; Whittemore, Vicky; Wilcox, Karen; Wagnon, Jacy L; Pearl, Phillip L; Vanderver, Adeline; Scheffer, Ingrid E
On April 21, 2015, the first SCN8A Encephalopathy Research Group convened in Washington, DC, to assess current research into clinical and pathogenic features of the disorder and prepare an agenda for future research collaborations. The group comprised clinical and basic scientists and representatives of patient advocacy groups. SCN8A encephalopathy is a rare disorder caused by de novo missense mutations of the sodium channel gene SCN8A, which encodes the neuronal sodium channel Nav 1.6. Since the initial description in 2012, approximately 140 affected individuals have been reported in publications or by SCN8A family groups. As a result, an understanding of the severe impact of SCN8A mutations is beginning to emerge. Defining a genetic epilepsy syndrome goes beyond identification of molecular etiology. Topics discussed at this meeting included (1) comparison between mutations of SCN8A and the SCN1A mutations in Dravet syndrome, (2) biophysical properties of the Nav 1.6 channel, (3) electrophysiologic effects of patient mutations on channel properties, (4) cell and animal models of SCN8A encephalopathy, (5) drug screening strategies, (6) the phenotypic spectrum of SCN8A encephalopathy, and (7) efforts to develop a bioregistry. A panel discussion of gaps in bioregistry, biobanking, and clinical outcomes data was followed by a planning session for improved integration of clinical and basic science research. Although SCN8A encephalopathy was identified only recently, there has been rapid progress in functional analysis and phenotypic classification. The focus is now shifting from identification of the underlying molecular cause to the development of strategies for drug screening and prioritized patient care.
Dávalos Moscol, Milagros; Bustios Sanchez, Carla
Hepatic encephalopathy (HE) is a neuropsychiatric syndrome characterized by changes in cognitive function, behavior, and personality, as well as by transient neurological symptoms and electroencephalographic changes, which occur in the context of acute or chronic liver failure. Cirrhosis is the main disease associated to HE, and it is known that its incidence is increasing worldwide. As a cause of mortality, cirrhosis is ranked 14 worldwide, but 10 in developed countries. It has been demonstrated that the incidence of liver disease is increasing, in part because of the ascending prevalence of NAFLD, HCV, HCC, as well of alcohol consumption. The real incidence of cirrhosis in Latin America is unknown, although in some Latin American countries that provided national data, cirrhosis death rates were between 5 and 17/100,000 for men and 3 and 5/100,000 for women. Disability, quality of life, and social aspects should be considered when assessing the impact of a disease. In this context, preliminary estimates of the global burden of disease attributable to chronic liver disease seem to be substantial. Hepatic encephalopathy, a main complication of liver failure, occurs in 30-45% of patients as overt encephalopathy, but when subclinical or minimal hepatic encephalopathy (MHE) is considered, estimates of the incidence of encephalopathy vary from 20 to 60%. In USA, the 2009 NIH Report on the Costs of Digestive Diseases stated that liver disease was the second most costly disease in direct and indirect costs (13.1 billion dollars). Although the economic cost of HE has not been assessed, it is obvious that the economic impact of HE on daily activities of living is extremely high, as the costs of diminished work performance and lost wages are substantial.
Fogler, Jessica A; Weber, Shannon; Goldschmidt, Ronald H; Mahoney, Megan R; Cohan, Deborah
This study evaluates the consultation needs of clinicians who provide perinatal human immunodeficiency virus (HIV) care in the United States. The Perinatal Hotline (1-888-448-8765) is a telephone consultation service for providers who treat HIV-infected pregnant women and their infants. Hotline calls were analyzed for demographics about callers and their patients and information about consultation topics. There were 430 calls to the hotline from January 1, 2005, through June 30, 2006. Most calls (59.5%) were related to pregnant patients; 5.1% of the calls pertained to women currently in labor. The most common topic was HIV care in pregnancy (49.1%), particularly antiretroviral drug use (42.1%). HIV testing was discussed in 21.9%, and intrapartum treatment was discussed in 24.0%. Callers most often requested help choosing antiretroviral drug regimens; many of the discussions were about drug toxicities and viral resistance. Although the hotline received few calls about women in labor, the need for these consultations is expected to increase with the expanding use of rapid HIV testing. Access to 24-hour consultation can help ensure that state-of-the-art care is provided.
Farr, Sherry L; Ko, Jean Y; Burley, Kim; Gupta, Seema
Women's lack of knowledge on symptoms of perinatal depression and treatment resources is a barrier to receiving care. We sought to estimate the prevalence and predictors of discussing depression with a prenatal care provider. We used the 2011 population-based data from 24 sites participating in the Pregnancy Risk Assessment Monitoring System (n = 32,827 women with recent live births) to examine associations between maternal characteristics and report that a prenatal care provider discussed with her what to do if feeling depressed during or after pregnancy. Overall, 71.9 % of women reported discussing perinatal depression with their prenatal care provider (range 60.7 % in New York City to 85.6 % in Maine). Women were more likely to report a discussion on perinatal depression with their provider if they they were 18-29 years of age than over 35 years of age compared to older (adjusted prevalence ratio [aPR] 18 to 19 y = 1.08, 20 to 24 y = 1.10, 25 to 29 y = 1.09), unmarried (aPR = 1.07) compared to married, had <12 years of education (aPR = 1.05) compared to > 12 years, and had no previous live births (aPR = 1.03) compared to ≥ 1 live births. Research is needed on effective ways to educate women about perinatal depression and whether increased knowledge on perinatal depression results in higher rates of treatment and shorter duration of symptoms.
Akalin, M Z; Maine, D; de Francisco, A; Vaughan, R
In recent years, the perinatal mortality rate (PNMR) has been proposed as a proxy measure of maternal mortality, because perinatal deaths are more frequent and potentially more easily measured. This report assesses evidence for an association between these two statistics. This study, based upon data from Matlab, Bangladesh, shows that the maternal mortality ratio (MMR) and the PNMR do not vary together over time, and that the PNMR does not reliably indicate either the magnitude or the direction of change in the MMR from year to year. Statistical analysis shows that the correlation between the PNMR and the MMR is not significantly different from zero. An examination of the major causes of maternal and perinatal deaths indicates that the two measures cannot be expected to vary together. Almost half of perinatal deaths result from causes that do not pose a threat to the mother's life, and almost half of maternal deaths result from causes that do not lead to perinatal death. Monitoring of the PNMR can give an inaccurate picture of maternal mortality and should not be used as a proxy.
Kendig, Susan; Keats, John P; Hoffman, M Camille; Kay, Lisa B; Miller, Emily S; Simas, Tiffany A Moore; Frieder, Ariela; Hackley, Barbara; Indman, Pec; Raines, Christena; Semenuk, Kisha; Wisner, Katherine L; Lemieux, Lauren A
Perinatal mood and anxiety disorders are among the most common mental health conditions encountered by women of reproductive age. When left untreated, perinatal mood and anxiety disorders can have profound adverse effects on women and their children, ranging from increased risk of poor adherence to medical care, exacerbation of medical conditions, loss of interpersonal and financial resources, smoking and substance use, suicide, and infanticide. Perinatal mood and anxiety disorders are associated with increased risks of maternal and infant mortality and morbidity and are recognized as a significant patient safety issue. In 2015, the Council on Patient Safety in Women's Health Care convened an interdisciplinary work group to develop an evidence-based patient safety bundle to address maternal mental health. The focus of this bundle is perinatal mood and anxiety disorders. The bundle is modeled after other bundles released by the Council on Patient Safety in Women's Health Care and provides broad direction for incorporating perinatal mood and anxiety disorder screening, intervention, referral, and follow-up into maternity care practice across health care settings. This commentary provides information to assist with bundle implementation.
Luo, Ma; Czarnecki, Chris; Ramdahin, Suzie; Embree, Joanne; Plummer, Francis A
Transplacental passage is a well-known phenomenon in HIV infection and immune responses at the maternal-fetal interface play a critical role in perinatal mother-to-child HIV transmission (MCHT). The high expression of HLA-G at the maternal-fetal interface and its role in mediating immune tolerance suggest that it could play an important role in MCHT. We investigated the role of HLA-G polymorphism in perinatal HIV transmission in 348 ART naïve mother-child pairs enrolled in a mother-child HIV transmission cohort, established in Nairobi, Kenya in 1986. Among the 348 children born to 266 HIV+ mothers, 258 were uninfected and 90 became infected perinatally. HLA-G exons 2 and 3 of 266 mothers and 251 children were sequenced and genotyped. Among 14 HLA-G alleles identified, only 4 alleles have a phenotype frequency above 10%. Correlation analysis showed that HLA-G(∗)01:03+ mothers were less likely to perinatally transmit HIV-1 to their children (p=0.038, Odds ratio:0.472, 95%CI:0.229-0.973). Mother-child HLA-G concordance was not associated with the increased perinatal HIV transmission. There was no significant difference in the general health between the transmitting mothers and the mothers who did not transmit HIV to their children.
Hill, Pamela D.; DeBackere, Katrina; Kavanaugh, Karen L.
Objective To review the research literature on the parental experience of pregnancy, primarily maternal, subsequent to perinatal loss. Data Sources Computerized searches on CINAHL and PubMed databases. Study Selection Articles from indexed journals relevant to the objective were reviewed from January 1997 to December 2007. Only research-based studies in English were included. Data Extraction The review was performed using the methodology of Whittemore and Knafl (2005). Data were extracted and organized under headings: author/year/setting; purpose; sample; design/instruments; results; and nursing implications for parents during a pregnancy following a perinatal loss. Data Synthesis Depression and anxiety are frequently seen in pregnant women subsequent to a perinatal loss. The parental experience is filled with intense and conflicting emotions as parents balance being hopeful while worrying about another potential loss. Conclusions It is important for health care providers to evaluate the woman's obstetric history, acknowledge and validate previous perinatal loss, and discuss with her what would be helpful during the prenatal period with respect to the previous perinatal loss. PMID:18811772
Marnes, Joanne; Hall, Pauline
The establishment of the National Perinatal Depression Initiative (NPDI, 2008-2013) has brought a focus across Australia for the need to identify women at risk of perinatal mental health disorders, suggesting that routine screening by relevant health professionals may aid earlier detection, better care and improved outcomes. Midwives are frequently the primary point of contact in the perinatal period and thus ideally placed to identify, interpret and manage complex situations, including screening for perinatal mental health disorders. This paper offers strategies that could be implemented into daily midwifery practice in order to achieve the goals consistent with the National Perinatal Depression Initiative. A case study (Jen) and discussion, guided by recommendations from the Australian Nursing and Midwifery Competency standards and beyondblue Clinical Practice Guidelines, are used to demonstrate how midwifery care can be provided. In accordance with her legal obligations, the midwife should act within her scope of practice to undertake a series of psychosocial and medical assessments in order to best determine how midwifery care and support can be of benefit to Jen, her infant and her family. Suggestions described include administration of validated screening questionnaires, clinical interview, physical assessment, discussion with partner, awareness of the mother-infant interactions and questioning around baby's sleep and feeding. Based on evaluation of the information gained from a bio-psycho-social assessment, suggestions are made as to the midwifery care options that could be applied.
Kendig, Susan; Keats, John P; Hoffman, M Camille; Kay, Lisa B; Miller, Emily S; Moore Simas, Tiffany A; Frieder, Ariela; Hackley, Barbara; Indman, Pec; Raines, Christena; Semenuk, Kisha; Wisner, Katherine L; Lemieux, Lauren A
Perinatal mood and anxiety disorders are among the most common mental health conditions encountered by women of reproductive age. When left untreated, perinatal mood and anxiety disorders can have profound adverse effects on women and their children, ranging from increased risk of poor adherence to medical care, exacerbation of medical conditions, loss of interpersonal and financial resources, smoking and substance use, suicide, and infanticide. Perinatal mood and anxiety disorders are associated with increased risks of maternal and infant mortality and morbidity and are recognized as a significant patient safety issue. In 2015, the Council on Patient Safety in Women's Health Care convened an interdisciplinary workgroup to develop an evidence-based patient safety bundle to address maternal mental health. The focus of this bundle is perinatal mood and anxiety disorders. The bundle is modeled after other bundles released by the Council on Patient Safety in Women's Health Care and provides broad direction for incorporating perinatal mood and anxiety disorder screening, intervention, referral, and follow-up into maternity care practice across health care settings. This commentary provides information to assist with bundle implementation.
Kendig, Susan; Keats, John P; Hoffman, M Camille; Kay, Lisa B; Miller, Emily S; Moore Simas, Tiffany A; Frieder, Ariela; Hackley, Barbara; Indman, Pec; Raines, Christena; Semenuk, Kisha; Wisner, Katherine L; Lemieux, Lauren A
Perinatal mood and anxiety disorders are among the most common mental health conditions encountered by women of reproductive age. When left untreated, perinatal mood and anxiety disorders can have profound adverse effects on women and their children, ranging from increased risk of poor adherence to medical care, exacerbation of medical conditions, loss of interpersonal and financial resources, smoking and substance use, suicide, and infanticide. Perinatal mood and anxiety disorders are associated with increased risks of maternal and infant mortality and morbidity and are recognized as a significant patient safety issue. In 2015, the Council on Patient Safety in Women's Health Care convened an interdisciplinary workgroup to develop an evidence-based patient safety bundle to address maternal mental health. The focus of this bundle is perinatal mood and anxiety disorders. The bundle is modeled after other bundles released by the Council on Patient Safety in Women's Health Care and provides broad direction for incorporating perinatal mood and anxiety disorder screening, intervention, referral, and follow-up into maternity care practice across health care settings. This commentary provides information to assist with bundle implementation.
Wiegand, Samantha L; Beamon, Carmen J; Chescheir, Nancy C; Stamilio, David
Objective To estimate the association between the severity of idiopathic polyhydramnios and adverse outcomes. Study Design Retrospective cohort study of deliveries at one hospital from 2000 to 2012 with an amniotic fluid index (AFI) measurement ≥24 + 0 weeks' gestation. Pregnancies complicated by diabetes, multiples, or fetal anomalies were excluded. Exposure was the degree of polyhydramnios: normal (AFI 5-24 cm), mild (≥ 24-30 cm), and moderate-severe (> 30 cm). Primary outcomes were perinatal mortality, neonatal intensive care unit (NICU) admission, and postpartum hemorrhage. Results There were 10,536 pregnancies: 10,188 with a normal AFI, 274 mild (78.74%), and 74 moderate-severe polyhydramnios (21.26%). Adverse outcomes were increased with idiopathic polyhydramnios: NICU admission (adjusted odds ratio [AOR] 3.71, 95% confidence interval [CI] 2.77-4.99), postpartum hemorrhage (AOR 15.81, 95% CI 7.82-31.96), macrosomia (AOR 3.41, 95% CI 2.61-4.47), low 5-minute Apgar score (AOR 2.60, 95% CI 1.57-4.30), and cesarean (AOR 2.16, 95% CI 1.74-2.69). There were increasing odds of macrosomia (mild: AOR 3.19, 95% CI 2.36-4.32; moderate-severe: AOR 4.44, 95% CI 2.53-7.79) and low 5-minute Apgar score (mild: AOR 2.24, 95% CI 1.23-4.08; moderate-severe: AOR 3.93, 95% CI 1.62-9.55) with increasing severity of polyhydramnios. Conclusion Idiopathic polyhydramnios is independently associated with increased risks of morbidity. There appears to be a dose-response relationship for neonatal macrosomia and low 5-minute Apgar score risks.
Craswell, Alison; Moxham, Lorna; Broadbent, Marc
This paper presents research undertaken as part of a larger research project to examine the factors that influence midwives when entering perinatal data. A grounded theory methodology was used to undertake qualitative interviews with 15 participants from 12 different hospitals across Queensland, Australia using three different systems for perinatal data collection. The findings surrounding accountability are presented revealing that a shift in governance relating to responsibility and accountability is not occurring in midwifery units across Queensland. Without assignation of responsibility for entries and accountability for mistakes or omissions, perinatal data records can be left incomplete or inaccurate. Increasing use of electronic health records and creation of digital hospitals indicates these issues are highly relevant in planning for these services.
Deligiannidis, Kristina M.; Freeman, Marlene P.
Complementary and Alternative Medicine (CAM) therapies are increasingly sought out by patients with psychiatric disorders. This article provides a review of the evidence for several commonly utilized CAM therapies (i.e. omega-3 fatty acids, folate, S-adenosyl-methionine (SAMe), St. John’s Wort, bright light therapy, exercise, massage, and acupuncture) in the treatment of perinatal depression. A number of these treatments may be reasonable to consider for women during pregnancy or the postpartum, but the safety and efficacy of these relative to standard treatments must still be systematically determined. Evidence based use of CAM treatments for perinatal depression is discussed. Adequately powered systematic studies are necessary to determine the role of CAM in the treatment of perinatal depression. PMID:24041861
Azad, Meghan B.; Kozyrskyj, Anita L.
Perinatal programming, a dominant theory for the origins of cardiovascular disease, proposes that environmental stimuli influence developmental pathways during critical periods of prenatal and postnatal development, inducing permanent changes in metabolism. In this paper, we present evidence for the perinatal programming of asthma via the intestinal microbiome. While epigenetic mechanisms continue to provide new explanations for the programming hypothesis of asthma development, it is increasingly apparent that the intestinal microbiota plays an independent and potentially interactive role. Commensal gut bacteria are essential to immune system development, and exposures disrupting the infant gut microbiota have been linked to asthma. This paper summarizes the recent findings that implicate caesarean delivery, breastfeeding, perinatal stress, probiotics, and antibiotics as modifiers of infant gut microbiota in the development of asthma. PMID:22110540
Curtis, Colleen; Mineyko, Aleksandra; Massicotte, Patricia; Leaker, Michael; Jiang, Xiu Yan; Floer, Amalia; Kirton, Adam
Perinatal stroke causes cerebral palsy and lifelong disability. Specific diseases are definable but mechanisms are poorly understood. Evidence suggests possible associations between arterial perinatal stroke and prothrombotic disorders but population-based, controlled, disease-specific studies are limited. Understanding thrombophilia in perinatal stroke informs pathogenesis models and clinical management. We conducted a population-based, prospective, case-control study to determine the association of specific perinatal stroke diseases with known thrombophilias. Children with idiopathic, MRI-classified neonatal arterial ischemic stroke (NAIS), arterial presumed perinatal ischemic stroke (APPIS), or fetal periventricular venous infarction (PVI) were recruited. Standardized thrombophilia evaluations were performed after 12 months of age on stroke cases and controls including quantified protein C and S, antithrombin, factors VIII/IX/XI, fibrinogen, lipoprotein(a), homocysteine, lupus anticoagulant, anticardiolipin antibodies and genotyping of factor V Leiden (FVL), factor II G20210A (FII), and MTHFR C677T. A total of 212 children were studied: 46 NAIS, 34 APPIS, 55 PVI, and 77 controls (53% male, median 4.8 years). Of 14 parameters, no differences were observed in 12 including all common thrombophilias. Mean prothrombin time was shorter in arterial strokes (p<0.001). Rates of antiphospholipid antibodies were low, comparable to controls, and resolved on repeat testing. FVL and FII rates were comparable to population norms. Total number of possible abnormalities did not differ between cases and controls. Our prospective, population-based, controlled, disease-specific study suggests minimal association between perinatal stroke and thrombophilia. This does not exclude the possibility of disordered coagulation at the time of stroke but suggests testing in childhood is not indicated.
Zerwas, Stephanie; Leserman, Jane; Holle, Ann Von; Regis, Taylor; Bulik, Cynthia
Abstract Objective Although the prevalence of perinatal depression (depression occurring during pregnancy and postpartum) is 10%, little is known about psychiatric comorbidity in these women. We examined the prevalence of comorbid eating disorders (ED) and trauma history in women with perinatal depression. Methods A research questionnaire was administered to 158 consecutive patients seen in a perinatal psychiatry clinic during pregnancy (n=99) or postpartum (n=59). Measures included Structured Clinical Interview for DSM (SCID) IV-based questions for lifetime eating psychopathology and assessments of comorbid psychiatric illness including the State/Trait Anxiety Inventory (STAI), Patient Health Questionnaire (PHQ-9), Edinburgh Postnatal Depression Scale (EPDS), and Trauma Inventory. Results In this cohort, 37.1% reported a putative lifetime ED history; 10.1% reported anorexia nervosa (AN), 10.1% reported bulimia nervosa (BN), 10.1% reported ED not otherwise specified-purging subtype (EDNOS-P), and 7.0% reported binge eating disorder (BED). Women with BN reported more severe depression (EPDS score, 19.1, standard deviation [SD 4.3], p=0.02; PHQ-severity 14.5, SD 7.4, p=0.02) than the referent group of women with perinatal depression and no ED history (EPDS 13.3, SD=6.1; PHQ 9.0, SD=6.2). Women with AN were more likely to report sexual trauma history than the referent group (62.5% vs. 29.3%, p<0.05), and those with BN were more likely report physical (50.0%, p<0.05) and sexual (66.7%, p<0.05) trauma histories. Conclusions ED histories were present in over one third of admissions to a perinatal psychiatry clinic. Women with BN reported more severe depression and histories of physical and sexual trauma. Screening for histories of eating psychopathology is important in women with perinatal depression. PMID:21671774
Gow, Adam G; Frowde, Polly E; Elwood, Clive M; Burton, Carolyn A; Powell, Roger M; Tappin, Simon W; Foale, Rob D; Duncan, Andrew; Mellanby, Richard J
Hypermanganesemia is commonly recognized in human patients with hepatic insufficiency and portosystemic shunting. Since manganese is neurotoxic, increases in brain manganese concentrations have been implicated in the development of hepatic encephalopathy although a direct causative role has yet to be demonstrated. Evaluate manganese concentrations in dogs with a naturally occurring congenital shunt before and after attenuation as well as longitudinally following the changes in hepatic encephalopathy grade. Our study demonstrated that attenuation of the shunt resolved encephalopathy, significantly reduced postprandial bile acids, yet a hypermanganasemic state persisted. This study demonstrates that resolution of hepatic encephalopathy can occur without the correction of hypermanganesemia, indicating that increased manganese concentrations alone do not play a causative role in encephalopathy. Our study further demonstrates the value of the canine congenital portosystemic shunt as a naturally occurring spontaneous model of human hepatic encephalopathy.
Slagle, T A
Today clinical information is used for a multitude of purposes beyond patient care documentation including quality review and improvement processes, allocation of resources, budgetary and long-term planning, productivity measurement, and justification to payers for services provided. Providers in perinatal medicine are faced with the challenge of finding methods to meet these information needs. Case examples of the different approaches to collecting and using obstetric and neonatal information are described. The role of computer-based patient records is outlined and solutions available to perinatal medicine are reviewed.
Busse, H; Harrop, T
Objective To assess the prevalence of engagement in self-asphyxial (risk-taking) behaviour (SAB) (‘choking game’) and associated morbidity and mortality in children and young people up to age 20. Design Systematic literature review. Search strategy Electronic database search of MEDLINE, Embase, PsycINFO, CINAHL, PubMed, Web of Science Core Collection, BIOSIS citation index and the Cochrane register with no language or date limits applied. References of key papers were reviewed, and experts were contacted to identify additional relevant papers. Eligibility criteria Systematic reviews, cross-sectional, cohort and case–control studies, and case reports examining SAB with regard to individuals aged 0–20 years, without explicitly stated autoerotic, suicidal or self-harm intentions were included. Results Thirty-six relevant studies were identified, and SAB was reported in 10 countries. In North America, France and Colombia, awareness of SAB ranged from 36% to 91% across studies/settings, and the median lifetime prevalence of engagement in SAB was 7.4%. Six studies identified the potential for SAB to be associated with engagement in other risk behaviours. Ninety-nine fatal cases were reported. Of the 24 cases described in detail, most occurred when individuals engaged in SAB alone and used a ligature. Conclusions The current evidence on SAB among young people is limited, and stems predominantly from North America and France. Awareness of SAB among young people is high, and engagement varies by setting. Further research is needed to understand the level of risk and harm associated with SAB, and to determine the appropriate public health response. PMID:26111816
Shetty, Teena; Raince, Avtar; Manning, Erin; Tsiouris, Apostolos John
Context: The diagnosis of chronic traumatic encephalopathy (CTE) can only be made pathologically, and there is no concordance of defined clinical criteria for premorbid diagnosis. The absence of established criteria and the insufficient imaging findings to detect this disease in a living athlete are of growing concern. Evidence Acquisition: The article is a review of the current literature on CTE. Databases searched include Medline, PubMed, JAMA evidence, and evidence-based medicine guidelines Cochrane Library, Hospital for Special Surgery, and Cornell Library databases. Study Design: Clinical review. Level of Evidence: Level 4. Results: Chronic traumatic encephalopathy cannot be diagnosed on imaging. Examples of imaging findings in common types of head trauma are discussed. Conclusion: Further study is necessary to correlate the clinical and imaging findings of repetitive head injuries with the pathologic diagnosis of CTE. PMID:26733590
Pillitteri, C A; Craig, L E
Hepatic encephalopathy has been listed as a differential for llamas displaying neurologic signs, but it has not been histopathologically described. This report details the neurologic histopathologic findings associated with 3 cases of hepatic lipidosis with concurrent neurologic signs and compares them to 3 cases of hepatic lipidosis in the absence of neurologic signs and 3 cases without hepatic lipidosis. Brain from all 3 llamas displaying neurologic signs contained Alzheimer type II cells, which were not detected in either subset of llamas without neurologic signs. Astrocytic immunohistochemical staining intensity for glial fibrillary acid protein was decreased in llamas with neurologic signs as compared to 2 of 3 llamas with hepatic lipidosis and without neurologic signs and to 2 of 3 llamas without hepatic lipidosis. Immunohistochemical staining for S100 did not vary between groups. These findings suggest that hepatic encephalopathy may be associated with hepatic lipidosis in llamas.
Jethava, Ashif; Dasanu, Constantin A
Health-care professionals must be aware of the mandatory vitamin supplementation in patients status post bariatric surgery. A recent increase in the number of gastric bypass surgeries in US has been associated with a proportional increase in Wernicke encephalopathy reports. Subtle or atypical neurologic features are not uncommon. Our report is of a female patient with acute Wernicke encephalopathy accompanied by sensorineural hearing loss six weeks after bariatric surgery. The patient had only a partial recovery of her neurologic symptoms eightweeks after vigorous therapy for this condition. Symptomatic thiamine (vitamin B1) and vitamin B12 deficiencies are particularly concerning effects of bariatric procedures, as neurologic and cognitive deficits may be long lasting or even permanent despite aggressive replacement therapy.
Naeini, Alireza E.; Daneshmand, Dana; Khorvash, Farzin; Chitsaz, Ahmad
Vogt-Koyanagi-Harada (VKH) is a rare syndrome affecting tissues containing melanocytes. The possibility of its autoimmune pathogenesis is supported by high frequent HLA-DR4 presentation, commonly associated with other autoimmune diseases. Eyes are the main affected organs, resulting in blindness. Brain disease is a late-onset event, and is extremely rare. Here, we are reporting a 57-year-old woman, a known case of VKH syndrome, presenting with brain encephalopathy several decades after the initial presentation. We think this long period between initial presentation and presentation of encephalopathy due to VKH syndrome has not been described before. She was treated with corticosteroids and discharged home with a good general condition. PMID:24753681
Albadareen, Rawan; Thornton, Stephen; Heshmati, Arezou; Gerona, Roy; Lowry, Jennifer
The availability and use of novel psychoactive substances has risen dramatically over the last decade. The unpredictability of their toxicity constitutes a real challenge. We report a case of an adolescent who developed prolonged encephalopathy after ingesting "Hot Molly," which was found to contain the novel psychoactive substance, methylenedioxybenzylpiperazine when analyzed by high resolution mass spectrometry assay. This is the first case of human toxicity from methylenedioxybenzylpiperazine ingestion in the medical literature confirmed by body fluid analysis presenting with significant and prolonged encephalopathy. The prolonged course may be due to CYP2D6 inhibition from a combination of the methylenedioxyphenyl moiety and the patient's ultrarapid metabolizer pharmacokinetics. The response to high dose corticosteroids suggests a possible inflammatory effect that warrants further investigation.
Malhotra, R C; Ghia, D K; Cordato, D J; Beran, R G
Glyphosate-surfactant (GlySH) is a commonly used herbicide that has been used in attempted suicide. Most reports of GlySH toxicity in patients have followed ingestion of the commercial product "Round-up" (Monsanto Ltd; Melbourne, Victoria, Australia), which consists of a mixture of glyphosate (as a isopropylanine salt) and a surfactant (polyoxyethyleneamine). Ingestion of Round-up is reported to cause significant toxicity including nausea, vomiting, oral and abdominal pain. Renal and hepatic impairment and pulmonary oedema may also occur. Impaired consciousness and encephalopathy have been reported as sequelae but there are limited data on the central nervous system (CNS) effects of Round-up toxicity. We report a 71-year-old male who attempted suicide with GlySH and developed a prolonged but reversible encephalopathy suggestive of acute CNS toxicity.
Hepatic encephalopathy is a frequent and serious complication of liver cirrhosis; the pathophysiology of this complication is not fully understood although great efforts have been made during the last years. There are few prospective studies on the epidemiology of this complication; however, it is known that it confers with high short-term mortality. Hepatic encephalopathy has been classified into different groups depending on the degree of hepatic dysfunction, the presence of portal-systemic shunts, and the number of episodes. Due to the large clinical spectra of overt EH and the complexity of cirrhotic patients, it is very difficult to perform quality clinical trials for assessing the efficacy of the treatments proposed. The physiopathology, clinical manifestation, and the treatment of HE is a challenge because of the multiple factors that converge and coexist in an episode of overt HE. PMID:27335836
Rivera-Espinosa, Liliana; Floriano-Sánchez, Esaú; Pedraza-Chaverrí, José; Coballase-Urrutia, Elvia; Sampieri, Aristides; Ortega-Cuellar, Daniel; Cárdenas-Rodríguez, Noemí; Carmona-Aparicio, Liliana
Hepatic encephalopathy (HE) is a common complication of cirrhosis, of largely reversible impairment of brain function occurring in patients with acute or chronic liver failure or when the liver is bypassed by portosystemic shunts. The mechanisms causing this brain dysfunction are still largely unclear. The need to avoid complications caused by late diagnosis has attracted interest to understand the mechanisms underlying neuronal damage in order to find markers that will allow timely diagnosis and to propose new therapeutic alternatives to improve the care of patients. One of the experimental approaches to study HE is microdialysis; this technique allows evaluation of different chemical substances in several organs through the recollection of samples in specific places by semi-permeable membranes. In this review we will discuss the contributions of microdialysis in the understanding of the physiological alterations in human hepatic encephalopathy and experimental models and the studies to find novel alternative therapies for this disease. PMID:23921686
Rivera-Espinosa, Liliana; Floriano-Sánchez, Esaú; Pedraza-Chaverrí, José; Coballase-Urrutia, Elvia; Sampieri, Aristides Iii; Ortega-Cuellar, Daniel; Cárdenas-Rodríguez, Noemí; Carmona-Aparicio, Liliana
Hepatic encephalopathy (HE) is a common complication of cirrhosis, of largely reversible impairment of brain function occurring in patients with acute or chronic liver failure or when the liver is bypassed by portosystemic shunts. The mechanisms causing this brain dysfunction are still largely unclear. The need to avoid complications caused by late diagnosis has attracted interest to understand the mechanisms underlying neuronal damage in order to find markers that will allow timely diagnosis and to propose new therapeutic alternatives to improve the care of patients. One of the experimental approaches to study HE is microdialysis; this technique allows evaluation of different chemical substances in several organs through the recollection of samples in specific places by semi-permeable membranes. In this review we will discuss the contributions of microdialysis in the understanding of the physiological alterations in human hepatic encephalopathy and experimental models and the studies to find novel alternative therapies for this disease.
Sanchez-Delgado, Jordi; Miquel, Mireia
Hepatic encephalopathy (HE) is a frequent and serious complication of liver cirrhosis. In addition to correction of the precipitating factors, the most commonly used treatments are non-absorbable disaccharides and rifaximin. Many of the recommendations are based on current clinical practice and there are few randomized controlled trials. Currently, rifaximin should be initiated during an episode of EH if, after 24-48 hours of non-absorbable disaccharide therapy, there is no clinical improvement. In recurrent EH, it is advisable to add rifaximin in patients under non-absorbable disaccharide therapy who develop a new episode. Currently, standard treatment with rifaximin for minimal EH is not recommended. Rifaximin is effective in the acute treatment of overt encephalopathy and in preventing recurrence.
Mirabelli-Badenier, M; Biancheri, R; Morana, G; Fornarino, S; Siri, L; Celle, M E; Veneselli, E; Vincent, A; Gaggero, R; Mancardi, M M
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a well-defined autoimmune disorder. Hashimoto's encephalopathy (HE) is a still controversial entity, lacking definite diagnostic criteria. We described a 14-year-old-girl presenting with a clinical picture consistent with the diagnosis of anti-NMDAR encephalitis, confirmed by NMDAR antibody testing. Four years earlier, she had presented a similar episode of acute encephalopathy diagnosed as HE. Anti-NMDAR encephalitis and HE share similar clinical features so that the differential diagnosis can be difficult if specific antibodies are not tested. The correct diagnosis of anti-NMDAR encephalitis is crucial to plan the appropriate management and follow-up, namely in term of oncological screening, since it can be paraneoplastic in origin. We suggest to re-evaluate the clinical history of all subjects with previous HE diagnosis in order to evaluate the possible diagnosis of anti-NMDAR encephalitis and plan the appropriate management of these patients.
Tassinari, Carlo A; Cantalupo, Gaetano; Rios-Pohl, Loreto; Giustina, Elvio Della; Rubboli, Guido
ESES (encephalopathy with status epilepticus during sleep) is an epileptic encephalopathy with heterogeneous clinical manifestations (cognitive, motor, and behavioral disturbances in different associations, and various seizure types) related to a peculiar electroencephalography (EEG) pattern characterized by paroxysmal activity significantly activated during slow sleep-that is, a condition of continuous spikes and waves, or status epilepticus, during sleep. The pathophysiologic mechanisms underlying this condition are still incompletely understood; recent data suggest that the abnormal epileptic EEG activity occurring during sleep might cause the typical clinical symptoms by interfering with sleep-related physiologic functions, and possibly neuroplasticity processes mediating higher cortical functions such as learning and memory consolidation. As in the myth of Penelope, the wife of Odysseus, what is weaved during the day will be unraveled during the night.
Leunda, Amaya; Van Vaerenbergh, Bernadette; Baldo, Aline; Roels, Stefan; Herman, Philippe
Since the appearance in 1986 of epidemic of bovine spongiform encephalopathy (BSE), a new form of neurological disease in cattle which also affected human beings, many diagnostic and research activities have been performed to develop detection and therapeutic tools. A lot of progress was made in better identifying, understanding and controlling the spread of the disease by appropriate monitoring and control programs in European countries. This paper reviews the recent knowledge on pathogenesis, transmission and persistence outside the host of prion, the causative agent of transmissible spongiform encephalopathies (TSE) in mammals with a particular focus on risk (re)assessment and management of biosafety measures to be implemented in diagnostic and research laboratories in Belgium. Also, in response to the need of an increasing number of European diagnostic laboratories stopping TSE diagnosis due to a decreasing number of TSE cases reported in the last years, decontamination procedures and a protocol for decommissioning TSE diagnostic laboratories is proposed. PMID:24055928
Ville, Dorothée; Chiron, Catherine; Laschet, Jacques; Dulac, Olivier
Hormonal therapy or ketogenic diet often permits overcoming the challenging periods of many epileptic encephalopathies (West and Lennox-Gastaut syndromes and encephalopathy with continuous spike-waves in slow sleep), but relapse affects over 20% of patients. We report here a monocenter pilot series of 42 consecutive patients in whom we combined oral steroids with the ketogenic diet for corticosteroid-resistant or -dependent epileptic encephalopathy. We retrospectively evaluated the effect on seizure frequency, interictal spike activity, neuropsychological course, and steroid treatment course. Twenty-three patients had West syndrome (WS), 13 had encephalopathy with continuous spike-waves in slow sleep (CSWS), and six others had miscellaneous epileptic encephalopathies. All patients succeeded to reach 0.8 to 1.6g/l ketone bodies in the urine following the usual KD regimen. For at least 6 months, 14/42 responded to the addition of the ketogenic diet: 4/23 with WS, 8/13 with CSWS, and 2/6 with miscellaneous epileptic encephalopathies. The addition of the KD allowed withdrawing steroids in all responders. Among them, 10/15 had been patients with steroid-dependent epileptic encephalopathy and 4/27 patients with steroid-resistant epileptic encephalopathy. Therefore, the ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies. Patients presenting with steroid-dependent CSWS seem to be the best candidates.
Alayón-Laguer, Diógenes; Alsina, Melissa; Ochoa-Bayona, Jose L.; Ayala, Ernesto
We report a case of a female patient with Durie-Salmon stage 3A/ISS stage I IgG kappa multiple myeloma (MM) who developed encephalopathy after high-dose melphalan and hematopoietic stem cell transplant (HSCT). The most common etiologies for encephalopathy such as infection, narcotic medications, metabolic-electrolyte disturbance, stroke, and central nervous system (CNS) hemorrhages were ruled out. The patient recovered from the altered mental status spontaneously. The possibilities of melphalan-induced encephalopathy versus critical-state delirium versus hypercytokinemia induce encephalopathy were contemplated. PMID:23259145
Latt, N; Dore, G
Wernicke encephalopathy is an acute, reversible neuropsychiatric emergency due to thiamine deficiency. Urgent and adequate thiamine replacement is necessary to avoid death or progression to Korsakoff syndrome with largely irreversible brain damage. Wernicke Korsakoff syndrome refers to a condition where features of Wernicke encephalopathy are mixed with those of Korsakoff syndrome. Although thiamine is the cornerstone of treatment of Wernicke encephalopathy, there are no universally accepted guidelines with regard to its optimal dose, mode of administration, frequency of administration or duration of treatment. Currently, different dose recommendations are being made. We present recommendations for the assessment and treatment of Wernicke encephalopathy based on literature review and our clinical experience.
Wernicke's encephalopathy is a fatal neurological disease caused by thiamine deficiency. Many reports indicate that Wernicke's encephalopathy is caused by malnutrition. We report the case of a 79-year-old female patient who had a left masticator space and parapharyngeal space abscess who was diagnosed with Wernicke's encephalopathy. She reported problems while eating due to the presence of the abscess, but the true quantities of food she was ingesting were never assessed. Clinicians have a responsibility to provide adequate nutritional support by ensuring that patients receive adequate nutrition. Clinicians should also keep in mind that Wernicke's encephalopathy may occur in patients who experienced prolonged periods of malnutrition. PMID:27162754
Tang, Ji-Hong; Tian, Jian-Mei; Sheng, Mao; Hu, Shao-Yan; Li, Yan; Zhang, Li-Ya; Gu, Qing; Wang, Qi
Increasing occurrence of posterior reversible encephalopathy syndrome has been reported in children with acute lymphoblastic leukemia. However, the etiology of posterior reversible encephalopathy syndrome is not clear. To study the possible pathogenetic mechanisms and treatment of this complication, we reported 11 cases of pediatric acute lymphoblastic leukemia who developed posterior reversible encephalopathy syndrome after induction chemotherapy. After appropriate treatment, the clinical symptoms of posterior reversible encephalopathy syndrome in most cases disappeared even though induction chemotherapy continued. During the 1-year follow-up, no recurrence of posterior reversible encephalopathy syndrome was observed. Although the clinical and imaging features of posterior reversible encephalopathy syndrome may be diverse, posterior reversible encephalopathy syndrome should be recognized as a possible important complication of acute lymphoblastic leukemia when neurologic symptoms appear. In line with previous reports, our study also indicated that posterior reversible encephalopathy syndrome was reversible when diagnosed and treated at an early stage. Thus, the occurrence of posterior reversible encephalopathy syndrome should be considered and investigated to optimize the early induction scheme of acute lymphoblastic leukemia treatment.
Sampaio, Mafalda; Rocha, Ruben; Biskup, Saskia; Leão, Miguel
The authors describe 2 patients with early infantile epileptic encephalopathy caused by 2 novel mutations involving the STXBP1 gene. The authors suggest that in spite of the rarity of STXBP1 mutations, molecular analysis of STXBP1 gene should be performed in patients with early infantile epileptic encephalopathy, after exclusion of ARX mutations in male patients and CDKL5 mutations in female patients. The potential mechanisms explaining the variable clinical phenotypes caused by STXBP1 mutations are discussed and the designation of early-onset epileptic encephalopathies, including an updated genetic classification, is proposed to encompass the epileptic encephalopathies beginning in the first 6 months of life.
Flamm, Steven L
Covert hepatic encephalopathy is a common problem in cirrhosis, affecting up to 80% of patients. It is defined as test-dependent brain dysfunction with clinical consequences in the setting of cirrhosis in patients who are not disoriented. Because it is not apparent clinically, and diagnostic testing has not been standardized, the issue has often been ignored in clinical practice. Yet, the clinical consequences are notable, including impaired quality of life, diminished work productivity, and poor driving skills.
Caranci, Ferdinando; Belfiore, Maria Paola; Manzi, Francesca; Pagliano, Pasquale; Cirillo, Sossio
Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological condition, generally observed in conjunction with severe and acute hypertension, that involves mainly the posterior head areas (occipital and temporal lobes) and anterior “watershed” areas. In this syndrome it is rare to observe a predominant involvement of the brainstem. We describe the clinical and radiological findings in a patient with brainstem involvement, discussing its pathophysiological features and possible differential diagnosis. PMID:26515750
Tortora, Fabio; Caranci, Ferdinando; Belfiore, Maria Paola; Manzi, Francesca; Pagliano, Pasquale; Cirillo, Sossio
Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological condition, generally observed in conjunction with severe and acute hypertension, that involves mainly the posterior head areas (occipital and temporal lobes) and anterior "watershed" areas. In this syndrome it is rare to observe a predominant involvement of the brainstem. We describe the clinical and radiological findings in a patient with brainstem involvement, discussing its pathophysiological features and possible differential diagnosis.
Gharabawy, Ramez; Pothula, Vijayasimha R; Rubinshteyn, Vladimir; Silverberg, Michael; Gave, Asaf A
Posterior reversible encephalopathy syndrome (PRES) is a rare disorder that is usually associated with hypertensive crises. It is often missed but may be diagnosed by head computed tomographic (CT) scan or magnetic resonance imaging. An adolescent man presented for elective right shoulder arthroscopic bankart repair. Arthroscopy was performed using a solution of normal saline with 3.3 mg/L of epinephrine for irrigation. Postoperatively, the patient presented with hypertension and epileptiform activity. A CT scan of the head showed PRES.
Anand, Anil C; Garg, Hitendra K
A large number of patients present with jaundice and encephalopathy in tropical country like India and acute liver failure is the usual cause. Clinical presentation like ALF is also a complication of many tropical infections, and these conditions may mimic ALF but may have subtle differences from ALF. Moreover, what hepatologists see as acute liver failure in tropics is different from what is commonly described in Western Textbooks. Paracetamol overdose, which is possibly the commonest cause of ALF in UK and USA, is hardly ever seen in India. Most common etiology here is viral hepatitis (hepatitis E > hepatitis B> hepatitis A). Apart from ALF, one may also come across subacute hepatic failure (SAHF) as well as acute-on-chronic liver failure (ACLF) due to viral hepatitis. Interestingly, a host of other conditions can mimic ALF because clinical presentation in these conditions can be dominated by jaundice and encephalopathy. Malarial hepatopathy is possibly the best-known condition out of these and is not an uncommon manifestation of severe malaria. A similar presentation can also be seen in other common infections in tropics such as dengue fever, typhoid fever, leptospirosis, scrub typhus, amoebic liver abscesses, tuberculosis and other bacterial and fungal infections with or without human immunodeficiency virus (HIV) related disease. In many of these conditions, liver failure may not be underlying pathophysiology. Some pregnancy related liver diseases could also present with jaundice and encephalopathy. This review summarizes the commonly seen presentations in tropical country like India, where jaundice and encephalopathy dominate the clinical picture.
Martinello, Kathryn; Hart, Anthony R; Yap, Sufin; Mitra, Subhabrata; Robertson, Nicola J
This review discusses an approach to determining the cause of neonatal encephalopathy, as well as current evidence on resuscitation and subsequent management of hypoxic-ischaemic encephalopathy (HIE). Encephalopathy in neonates can be due to varied aetiologies in addition to hypoxic-ischaemia. A combination of careful history, examination and the judicious use of investigations can help determine the cause. Over the last 7 years, infants with moderate to severe HIE have benefited from the introduction of routine therapeutic hypothermia; the number needed to treat for an additional beneficial outcome is 7 (95% CI 5 to 10). More recent research has focused on optimal resuscitation practices for babies with cardiorespiratory depression, such as delayed cord clamping after establishment of ventilation and resuscitation in air. Around a quarter of infants with asystole at 10 min after birth who are subsequently cooled have normal outcomes, suggesting that individualised decision making on stopping resuscitation is needed, based on access to intensive treatment unit and early cooling. The full benefit of cooling appears to have been exploited in our current treatment protocols of 72 hours at 33.5°C; deeper and longer cooling showed adverse outcome. The challenge over the next 5-10 years will be to assess which adjunct therapies are safe and optimise hypothermic brain protection in phase I and phase II trials. Optimal care may require tailoring treatments according to gender, genetic risk, injury severity and inflammatory status.
Direkze, Shamindra; Jalan, Rajiv
Minimal hepatic encephalopathy (mHE) is common among patients with cirrhotic liver disease and causes significant morbidity and mortality. It may present as cognitive impairment, behavioural changes and, less frequently, with neurological symptoms which make diagnosis of the disease challenging. A history of falls and accidents may also be suggestive of mHE. Diagnosis primarily relies on at least two positive psychometric tests of which the psychometric hepatic encephalopathy score (PHES) is essential. Alternatively, PHES and an electroencephalogram may be used to establish a diagnosis. Biochemical markers of encephalopathy currently have no role in the diagnosis of mHE. Treatment is not always advocated for a diagnosis of mHE but is dependent on the degree of impairment caused by the symptoms. After treatment of other metabolic abnormalities and co-morbidities associated with cirrhosis, more specific treatment for mHE largely relies on therapies used to lower ammonia levels. Laxatives and rifaximin are commonly used in treatment and work through decreasing ammonia absorption from the gut. Other therapies, such as BCAA, LOLA, L-carnitine and phenylbutyrate, modify responses to ammonia as well as enhancing metabolism and excretion. mHE resulting from spontaneous portosystemic shunts or transhepatic intraportal systemic shunts may require ablation or reduction of the shunt. Early detection and appropriate treatment of mHE is important to prevent significant cognitive impairments and progression to overt HE.
García-Moreno, L M; Conejo, N M; González-Pardo, H; Aller, M A; Nava, M P; Arias, J; Arias, J L
The serious neuropsychological repercussions of hepatic encephalopathy have led to the creation of several experimental models in order to better understand the pathogenesis of the disease. In the present investigation, two possible causes of hepatic encephalopathy, cholestasis and portal hypertension, were chosen to study the behavioral impairments caused by the disease using an object recognition task. This working memory test is based on a paradigm of spontaneous delayed non-matching to sample and was performed 60 days after surgery. Male Wistar rats (225-250 g) were divided into three groups: two experimental groups, microsurgical cholestasis (N = 20) and extrahepatic portal hypertension (N = 20), and a control group (N = 20). A mild alteration of the recognition memory occurred in rats with cholestasis compared to control rats and portal hypertensive rats. The latter group showed the poorest performance on the basis of the behavioral indexes tested. In particular, only the control group spent significantly more time exploring novel objects compared to familiar ones (P < 0.001). In addition, the portal hypertension group spent the shortest time exploring both the novel and familiar objects (P < 0.001). These results suggest that the existence of portosystemic collateral circulation per se may be responsible for subclinical encephalopathy.
Gamal, Maha; Abdel Wahab, Zainab; Eshra, Mohamed; Rashed, Laila; Sharawy, Nivin
Objective. Encephalopathy and brain edema are serious complications of acute liver injury and may lead to rapid death of patients. The present study was designed to investigate the role of the inflammatory mediators and oxidative stress in the cytotoxic brain oedema and the neuroprotective effects of both minocycline and dexamethasone. Methods. 48 male albino rats were divided into 4 groups: control group, acute liver injury (ALI) group, minocycline pretreated ALI group, and dexamethasone pretreated ALI group. 24 hours after acute liver injury serum ammonia, liver enzymes, brain levels of heme oxygenase-1 gene, iNOS gene expression, nitrite/nitrate, and cytokines were measured. In addition, the grades of encephalopathy and brain water content were assessed. Results. ALI was associated with significant increases in all measured inflammatory mediators, oxidative stress, iNOS gene expression, and nitrite/nitrate. Both minocycline and dexamethasone significantly modulated the inflammatory changes and the oxidative/nitrosative stress associated with ALI. However, only minocycline but not dexamethasone significantly reduced the cytotoxic brain oedema. Conclusion. Both minocycline and dexamethasone could modulate inflammatory and oxidative changes observed in brain after ALI and could be novel preventative therapy for hepatic encephalopathy episodes.
Kim, Jihye; Park, Jae Yong; Hong, Seung Wook; Lee, Joo Young; Kang, Jin Woo; Hwang, Seongjun; Ko, Sang-Bae; Im, Jong Pil; Kim, Joo Sung
Metronidazole is a widely used antibiotic for the treatment of anaerobic bacterial infections. Metronidazole-induced encephalopathy (MIEP) is a rare but potentially reversible disease. The mechanism of MIEP remains unclear, and differences in the neurotoxic effects of oral versus intravenous (IV) metronidazole administration have not yet been determined. We report the case of a Crohn's disease (CD) patient who experienced encephalopathy immediately after a single IV dose of metronidazole following long-term exposure to the oral form of the drug. The 64-year-old man with intractable CD experienced a sudden change in mental status, aphasia, and muscle weakness after IV administration of metronidazole. He had previously taken metronidazole orally for 13 years and received intermittent IV metronidazole treatments for CD exacerbation. Brain magnetic resonance imaging (MRI) showed high-intensity signals in the bilateral medial thalamus and the midbrain and pontine tegmentum on fluid-attenuated inversion recovery images. After discontinuation of metronidazole, the high-intensity brain MRI signals resolved and the patient's mental status dramatically improved; however, the patient exhibited mild cognitive dysfunction 2 months after the onset of encephalopathy. PMID:28239323
Dalla Bernardina, B; Dulac, O; Fejerman, N; Dravet, C; Capovilla, G; Bondavalli, S; Colamaria, V; Roger, J
The authors describe the electroclinical aspects and evolution of nine cases of myoclonic epileptic encephalopathy which began between two days and ten weeks of life. At onset it is associated with: myoclonic jerks, partial fits and periodic paroxysmal EEG abnormalities. Repeated spasms coexisting with partial fits and 'suppression-bursts' (both appearing later) complete the electroclinical picture. The neurological status (initially normal) progressively deteriorates leading within a few months to a decerebrate posture with opisthotonos. In spite of thorough neuroradiological, biochemical, cytological, metabolic, and ultrastructural investigations, the etiology remained unknown. However, the electroclinical and evolutive patterns are similar to those of some metabolic diseases (Polyodystrophy, Non-Ketotic Hyperglycinemia, etc.). All these observations display a homogeneous electroclinical pattern for which the authors propose the name of Early Myoclonic Epileptic Encephalopathy. This type deserves to be classified as a particular electroclinical entity among the epileptic encephalopathies of the first year of life; since its course is regularly downhill in all cases there may be a familial recurrence due to the possibility of a metabolic etiology.
Kwee, Janelle L; McBride, Hillary L
Women's experiences of pregnancy, birth, and postpartum adjustment are often characterized by feelings of disempowerment, trauma, and emotional pain. Psychosocial perinatal care has not kept up with medical advances in perinatal care. Access to psychosocial care appears to be inadequate because of the following: (a) perinatal health care providers are insufficiently prepared to address emotional aspects of maternal care and (b) traditional, compartmentalized psychological services benefit only a subsection of perinatal women, often in an untimely manner. Practical and innovative psychosocial services, integrated into routine perinatal care, can provide widespread access to psychosocial resources for mothers and supports providers in delivering optimal care.
Xu, Yajuan; Ren, Lidan; Zhai, Shanshan; Luo, Xiaohua; Hong, Teng; Liu, Rui; Ran, Limin; Zhang, Yingying
BACKGROUND To evaluate the association between the isolated single umbilical artery (iSUA) and perinatal outcomes, including pregnancy outcomes and perinatal complications. MATERIAL AND METHODS We performed a meta-analysis of 15 eligible studies regarding the relationship between the iSUA and perinatal outcomes, including gestational age at delivery, nuchal cord, placental weight, small for gestational age (SGA), oligohydramnios, polyhydramnios, pregnancy-induced hypertension (PIH), gestational diabetes mellitus (GDM), preeclampsia, and perinatal mortality. The overall odds ratios (OR) or standardized mean difference (SMD) were calculated. RESULTS The occurrence of nuchal cord was not found to be different between an iSUA and a three-vessel cord (TVC) fetus. For perinatal complications, the SGA, oligohydramnios, polyhydramnios, GDM, and perinatal mortality showed dramatic difference between women with an iSUA and women with a TVC fetus, which implied that the presence of iSUA significantly increased the risk of perinatal complications. For other perinatal complications, such as PIH and preeclampsia, no significant association was detected. CONCLUSIONS Our meta-analysis suggests that the presence of iSUA would increase the risk of perinatal complications such as SGA, oligohydramnios, polyhydramnios, GDM, and perinatal mortality. Therefore, pregnant women with an iSUA fetus have poorer perinatal outcomes and more attention should be given to the management of their pregnancy compared to women with a TVC fetus.
Kurth, Lisa; Haussmann, Robert
Objective: To investigate a potential relationship between coincidental increases in perinatal Pitocin usage and subsequent childhood ADHD onset in an attempt to isolate a specific risk factor as an early biomarker of this neurodevelopmental disorder. Method: Maternal labor/delivery and corresponding childbirth records of 172 regionally diverse,…
Dean, Raymond S.; Davis, Andrew S.
Perinatal complications have been associated with a myriad of later-developing behavioral, neurological, and psychological disorders. These have included school-related disorders such as attention-deficit/hyperactivity disorder, autism, mood and anxiety disorders, and learning disabilities. This article reviews the research that considers the…
Ribeiro, Christiane Fernandes; Lopes, Vânia Glória Silami; Brasil, Patrícia; Coelho, Janice; Muniz, Adriana Gouveia; Nogueira, Rita Maria Ribeiro
Perinatal transmission of dengue virus was confirmed by the evidence of virus in fetal tissue, newborn serum, and placenta of pregnant women. Abortion, several different clinical findings, and placental inflammatory findings were documented. No association was seen between severity of maternal dengue and disease of the newborn.
Jones, Melanie M.
This article reviews some of the literature on educational approaches for drug-exposed children. Common effects of prenatal and perinatal drug use on the female user, the developing fetus, and the neonate are reviewed. It is noted that female drug users have an increased incidence of medical complications during pregnancy; that the specific…
Zhang, Xin; Lv, Cong-Chao; Tian, Jiang; Miao, Ru-Juan; Xi, Wei; Hertz-Picciotto, Irva; Qi, Lihong
We conducted a case-control study using 190 Han children with and without autism to investigate prenatal and perinatal risk factors for autism in China. Cases were recruited through public special education schools and controls from regular public schools in the same region (Tianjin), with frequency matching on sex and birth year. Unadjusted…
McConnell, Edwina A.
Survey responses from 48 perinatal nurses found that most learned about medical devices by reading manuals; 75% had received inservice training; and 95% learned from other staff. Inadequate knowledge was related to fear of causing patient harm. Initial learning method influenced what was learned, and hands-on experience was considered efficacious.…
Bilder, Deborah A.; Pinborough-Zimmerman, Judith; Bakian, Amanda V.; Miller, Judith S.; Dorius, Josette T.; Nangle, Barry; McMahon, William M.
Prenatal and perinatal risk factors associated with intellectual disability (ID) were studied in 8-year-old Utah children from a 1994 birth cohort (N = 26,108) using broad ascertainment methods and birth records following the most current recording guidelines. Risk factor analyses were performed inclusive and exclusive of children with a known or…
For developing countries, especially in remote rural areas, measures of maternal and perinatal health may be difficult to obtain because it is not systematically collected and/or electronic data is not available. We assisted the public health officials of Bayingnormen (BaMen), In...
... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Perinatal monitoring system and accessories. 884.2740 Section 884.2740 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN... heart rate by means of combining and coordinating uterine contraction and fetal heart monitors...
... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Perinatal monitoring system and accessories. 884.2740 Section 884.2740 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN... heart rate by means of combining and coordinating uterine contraction and fetal heart monitors...
... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Perinatal monitoring system and accessories. 884.2740 Section 884.2740 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN... heart rate by means of combining and coordinating uterine contraction and fetal heart monitors...
Fenichel, Emily, Ed.
"Zero to Three is a single focus bulletin of the National Center for Infants, Toddlers, and Families providing insight from multiple disciplines on the development of infants, toddlers, and their families. Noting that because the perinatal periodfrom the later stages of pregnancy through the first 6 months of the infants lifeis a period of…
The experience of perinatal mental illness (mental illness occurring around the time of pregnancy) currently affect 1 in 10 women and can have adverse effects on the mother and her child (Massie and Szajnberg, 2002; O'Connor et al., 2002). The care and effective management of women experiencing perinatal mental illness is therefore an important issue for health care staff, managers, psychiatrists, commissioners and campaigners. Midwives play a significant part in caring for women throughout their pregnancies, during labour and up to the first month after birth. Midwives are in a unique position to assess a woman's well-being and to offer appropriate support. However, previous research has revealed that midwives often have poor understanding and knowledge of perinatal mental health issues and require improved training (Ross-Davie et al, 2006; McCann and Clark, 2010). This research project aims to systematically assess student midwives awareness of perinatal mental illness. The findings of this study will inform curriculum development for graduate and post-graduate midwifery students therefore improving the care and support women with mental illness receive from antenatal services. The findings from this study will also be used for the formation of an educational web-based programme for student and qualified midwives.
Bányász, Ilona; Bokodi, Géza; Vásárhelyi, Barna; Treszl, András; Derzbach, László; Szabó, András; Tulassay, Tivadar; Vannay, Adám
Low birth weight (LBW) infants have increased susceptibility to perinatal complications. An immature and impaired vascular system may possibly participate in these complications. There is evidence that supports the notion that vascular endothelial growth factor (VEGF), which is an essential regulator of embryonic angiogenesis, plays a central role in the pathogenesis of perinatal complications. We aimed to test whether functional genetic polymorphisms of VEGF are associated with the risk of preterm birth or perinatal morbidity. We enrolled 128 LBW infants (< or = 1500 grams). VEGF T-460C, VEGF C-2578A and VEGF G+405C polymorphisms were determined by real-time PCR or PCR-RFLP, respectively. Their genotypes were compared with VEGF genotypes of 200 healthy, term neonates. The prevalence of the VEGF+405 C allele was higher in LBW infants than in healthy, term neonates (OR [95% CI]: 1.29 [1.01-1.65]). Carrier state for the VEGF -2578A allele was an independent risk factor for enterocolitis necrotisans (NEC) (adjusted OR [95% CI]: 2.77 [1.00-7.65]). The carrier state for the VEGF -2578AA genotype was associated with a decreased risk of acute renal failure (ARF) (adjusted OR [95% CI]: 0.2 [0.05-0.78]). These results suggest that VEGF G+405C polymorphism might be associated with a higher risk of preterm birth and that VEGF C-2578A polymorphism may participate in the development of perinatal complications such as NEC and ARF.
Cannon, Daniel T; Liu, Jie; Sakurai, Reiko; Rossiter, Harry B; Rehan, Virender K
Perinatal smoke/nicotine exposure predisposes to chronic lung disease and morbidity. Mitochondrial abnormalities may contribute as the PPARγ pathway is involved in structural and functional airway deficits after perinatal nicotine exposure. We hypothesized perinatal nicotine exposure results in lung mitochondrial dysfunction that can be rescued by rosiglitazone (RGZ; PPARγ receptor agonist). Sprague-Dawley dams received placebo (CON), nicotine (NIC, 1 mg kg(-1)), or NIC + RGZ (3 mg kg(-1)) daily from embryonic day 6 to postnatal day 21. Parenchymal lung (~10 mg) was taken from adult male offspring for mitochondrial assessment in situ. ADP-stimulated O2 consumption was less in NIC and NIC + RGZ compared to CON (F[2,14] = 17.8; 4.5 ± 0.8 and 4.1 ± 1.4 vs. 8.8 ± 2.5 pmol s mg(-1); p < 0.05). The respiratory control ratio for ADP, an index of mitochondrial coupling, was reduced in NIC and remediated in NIC + RGZ (F[2,14] = 3.8; p < 0.05). Reduced mitochondrial oxidative capacity and abnormal coupling were evident after perinatal nicotine exposure. RGZ improved mitochondrial function through tighter coupling of oxidative phosphorylation.
Poulsen, Marie Kanne
This report, which is based on the work of the Perinatal Substance Exposure Think Tanks, establishes priorities for statewide services in California to young children who are prenatally exposed to alcohol and drugs. Although the report focuses on the developmental needs of children, it also examines efforts to provide prevention and treatment…
Objective To compare perinatal outcomes, maternal outcomes, and interventions in labour by planned place of birth at the start of care in labour for women with low risk pregnancies. Design Prospective cohort study. Setting England: all NHS trusts providing intrapartum care at home, all freestanding midwifery units, all alongside midwifery units (midwife led units on a hospital site with an obstetric unit), and a stratified random sample of obstetric units. Participants 64 538 eligible women with a singleton, term (≥37 weeks gestation), and “booked” pregnancy who gave birth between April 2008 and April 2010. Planned caesarean sections and caesarean sections before the onset of labour and unplanned home births were excluded. Main outcome measure A composite primary outcome of perinatal mortality and intrapartum related neonatal morbidities (stillbirth after start of care in labour, early neonatal death, neonatal encephalopathy, meconium aspiration syndrome, brachial plexus injury, fractured humerus, or fractured clavicle) was used to compare outcomes by planned place of birth at the start of care in labour (at home, freestanding midwifery units, alongside midwifery units, and obstetric units). Results There were 250 primary outcome events and an overall weighted incidence of 4.3 per 1000 births (95% CI 3.3 to 5.5). Overall, there were no significant differences in the adjusted odds of the primary outcome for any of the non-obstetric unit settings compared with obstetric units. For nulliparous women, the odds of the primary outcome were higher for planned home births (adjusted odds ratio 1.75, 95% CI 1.07 to 2.86) but not for either midwifery unit setting. For multiparous women, there were no significant differences in the incidence of the primary outcome by planned place of birth. Interventions during labour were substantially lower in all non-obstetric unit settings. Transfers from non-obstetric unit settings were more frequent for nulliparous women (36% to
Herrera, María Inés; Udovin, Lucas Daniel; Kusnier, Carlos; Kölliker-Frers, Rodolfo; de Souza, Wanderley
Birth asphyxia also termed perinatal asphyxia is an obstetric complication that strongly affects brain structure and function. Central nervous system is highly susceptible to oxidative damage caused by perinatal asphyxia while activation and maturity of the proper pathways are relevant to avoiding abnormal neural development. Perinatal asphyxia is associated with high morbimortality in term and preterm neonates. Although several studies have demonstrated a variety of biochemical and molecular pathways involved in perinatal asphyxia physiopathology, little is known about the synaptic alterations induced by perinatal asphyxia. Nearly 25% of the newborns who survive perinatal asphyxia develop neurological disorders such as cerebral palsy and certain neurodevelopmental and learning disabilities where synaptic connectivity disturbances may be involved. Accordingly, here we review and discuss the association of possible synaptic dysfunction with perinatal asphyxia on the basis of updated evidence from an experimental model. PMID:28326198
Herrera, María Inés; Otero-Losada, Matilde; Udovin, Lucas Daniel; Kusnier, Carlos; Kölliker-Frers, Rodolfo; de Souza, Wanderley; Capani, Francisco
Birth asphyxia also termed perinatal asphyxia is an obstetric complication that strongly affects brain structure and function. Central nervous system is highly susceptible to oxidative damage caused by perinatal asphyxia while activation and maturity of the proper pathways are relevant to avoiding abnormal neural development. Perinatal asphyxia is associated with high morbimortality in term and preterm neonates. Although several studies have demonstrated a variety of biochemical and molecular pathways involved in perinatal asphyxia physiopathology, little is known about the synaptic alterations induced by perinatal asphyxia. Nearly 25% of the newborns who survive perinatal asphyxia develop neurological disorders such as cerebral palsy and certain neurodevelopmental and learning disabilities where synaptic connectivity disturbances may be involved. Accordingly, here we review and discuss the association of possible synaptic dysfunction with perinatal asphyxia on the basis of updated evidence from an experimental model.
Hamir, Amir N; Kehrli, Marcus E; Kunkle, Robert A; Greenlee, Justin J; Nicholson, Eric M; Richt, Jürgen A; Miller, Janice M; Cutlip, Randall C
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. The emergence of BSE and its spread to human beings in the form of variant Creutzfeldt-Jakob disease (vCJD) resulted in interest in susceptibility of cattle to CWD, TME and scrapie. Experimental cross-species transmission of TSE agents provides valuable information for potential host ranges of known TSEs. Some interspecies transmission studies have been conducted by inoculating disease-causing prions intracerebrally (IC) rather than orally; the latter is generally effective in intraspecies transmission studies and is considered a natural route by which animals acquire TSEs. The "species barrier" concept for TSEs resulted from unsuccessful interspecies oral transmission attempts. Oral inoculation of prions mimics the natural disease pathogenesis route whereas IC inoculation is rather artificial; however, it is very efficient since it requires smaller dosage of inoculum, and typically results in higher attack rates and reduces incubation time compared to oral transmission. A species resistant to a TSE by IC inoculation would have negligible potential for successful oral transmission. To date, results indicate that cattle are susceptible to IC inoculation of scrapie, TME, and CWD but it is only when inoculated with TME do they develop spongiform lesions or clinical disease similar to BSE. Importantly, cattle are resistant to oral transmission of scrapie or CWD; susceptibility of cattle to oral transmission of TME is not yet determined.
Background Women of reproductive age are vulnerable to psychosocial problems, but these have remained largely unexplored in Muslim women in developing countries. The aim of this study was to explore and describe psychosocial impact and social support following perinatal loss among Muslim women. Methods A qualitative study was conducted in a specialist centre among Muslim mothers who had experienced perinatal loss. Purposive sampling to achieve maximum variation among Muslims in relation to age, parity and previous perinatal death was used. Data was collected by focus group discussion and in-depth unstructured interview until the saturation point met. Sixteen mothers who had recent perinatal loss of wanted pregnancy, had received antenatal follow up from public or private health clinics, and had delivery in our centre participated for the study. All of them had experienced psychological difficulties including feelings of confusion, emptiness and anxiety over facing another pregnancy. Results Two out of sixteen showed anger and one felt guilt. They reported experiencing a lack of communication and privacy in the hospital during the period of grief. Family members and friends play an important role in providing support. The majority agreed that the decision makers were husbands and families instead of themselves. The respondents felt that repetitive reminder of whatever happened was a test from God improved their sense of self-worth. They appreciated this reminder especially when it came from husband, family or friends closed to them. Conclusion Muslim mothers who had experienced perinatal loss showed some level of adverse psychosocial impact which affected their feelings. Husbands and family members were the main decision makers for Muslim women. Health care providers should provide psychosocial support during antenatal, delivery and postnatal care. On-going support involving husband should be available where needed. PMID:22708998
Okoroh, Ekwutosi M.; Kroelinger, Charlan D.; Smith, Alexander M.; Goodman, David A.; Barfield, Wanda D.
BACKGROUND Perinatal regionalization is a system of maternal and neonatal risk-appropriate health care delivery in which resources are ideally allocated for mothers and newborns during pregnancy, labor and delivery, and postpartum, in order to deliver appropriate care. Typically, perinatal risk-appropriate care is provided in-person, but with the advancement of technologies, the opportunity to provide care remotely has emerged. Telemedicine provides distance-based care to patients by consultation, diagnosis, and treatment in rural or remote US jurisdictions (states and territories). OBJECTIVE We sought to summarize the telemedicine policies of states and territories and assess if maternal and neonatal risk-appropriate care is specified. STUDY DESIGN We conducted a 2014 systematic World Wide Web–based review of publicly available rules, statutes, regulations, laws, planning documents, and program descriptions among US jurisdictions (N=59) on telemedicine care. Policies including language on the topics of consultation, diagnosis, or treatment, and those specific to maternal and neonatal risk-appropriate care were categorized for analysis. RESULTS Overall, 36 jurisdictions (32 states; 3 territories; and District of Columbia) (61%) had telemedicine policies with language referencing consultation, diagnosis, or treatment; 29 (49%) referenced consultation, 30 (51%) referenced diagnosis, and 35 (59%) referenced treatment. In all, 26 jurisdictions (22 states; 3 territories; and District of Columbia) (44%), referenced all topics. Only 3 jurisdictions (3 states; 0 territories) (5%), had policy language specifically addressing perinatal care. CONCLUSION The majority of states have published telemedicine policies, but few specify policy language for perinatal risk-appropriate care. By ensuring that language specific to the perinatal population is included in telemedicine policies, access to maternal and neonatal care can be increased in rural, remote, and resource
Yousefian, Omid; Ballantyne, Angela O.; Doo, Alex; Trauner, Doris A.
BACKGROUND Children with peri-natal stroke may show evidence of contralateral spatial neglect. The goal of this study was to determine whether a clock drawing task commonly used in adults to identify neglect would be effective in detecting neglect in children with peri-natal stroke. METHODS Thirty-eight individuals (age range 6–21 years) with left hemisphere (LH) or right hemisphere (RH) peri-natal onset unilateral lesions and one hundred seventy-nine age-matched controls were given the free-drawn Clock Drawing Task (CDT) in a cross-sectional design. An adapted scoring system that evaluated right- and left-sided errors separately was developed as part of the investigation. RESULTS Children with LH lesions made a greater number of errors on both the right and left sides of the clock drawings in all age subgroups (6–8 years, 9–14 years, and 15–21 years) compared to controls. Children with RH lesions showed greater left and right errors in the younger groups compared to controls, with significantly poorer performance on the left at 6–8 years, suggestive of contralateral neglect. However, by ages 15–21 years, the RH lesion subjects no longer differed from controls. CONCLUSIONS Clock drawing can identify spatial neglect in children with early hemispheric damage. However, brain development is a dynamic process, and as children age, spatial neglect may no longer be evident. These findings demonstrate the limitations of predicting long-term outcome after peri-natal stroke from early neuro-cognitive data. Children with peri-natal stroke may require different neural pathways to accomplish specific skills or to overcome deficits, but ultimately they may have “typical” outcomes. PMID:26002051
McAdams, Ryan M; McPherson, Ronald J; Kapur, Raj P; Juul, Sandra E
Worldwide, hypoxic-ischemic encephalopathy (HIE) is a major cause of neonatal mortality and morbidity. To better understand the mechanisms contributing to brain injury and improve outcomes in neonates with HIE, better preclinical animal models that mimic the clinical situation following birth asphyxia in term newborns are needed. In an effort to achieve this goal, we modified our nonhuman primate model of HIE induced by in utero umbilical cord occlusion (UCO) to include postnatal hypoxic episodes, in order to simulate apneic events in human neonates with HIE. We describe a cohort of 4 near-term fetal Macaca nemestrina that underwent 18 min of in utero UCO, followed by cesarean section delivery, resuscitation, and subsequent postnatal mechanical ventilation, with exposure to intermittent daily hypoxia (3 min, 8% O2 3-8 times daily for 3 days). After delivery, all animals demonstrated severe metabolic acidosis (pH 7 ± 0.12; mean ± SD) and low APGAR scores (<5 at 10 min of age). Three of 4 animals had both electrographic and clinical seizures. Serial blood samples were collected and plasma metabolites were determined by 2-dimensional gas chromatography coupled with time-of-flight mass spectrometry (GC × GC-TOFMS). The 4 UCO animals and a single nonasphyxiated animal (delivered by cesarean section but without exposure to UCO or prolonged sedation) underwent brain magnetic resonance imaging (MRI) on day 8 of life. Thalamic injury was present on MRI in 3 UCO animals, but not in the control animal. Following necropsy on day 8, brain histopathology revealed neuronal injury/loss and gliosis in portions of the ventrolateral thalamus in all 4 UCO, with 2 animals also demonstrating putamen/globus pallidus involvement. In addition, all 4 UCO animals demonstrated brain stem gliosis, with neuronal loss present in the midbrain, pons, and lateral medulla in 3 of 4 animals. Transmission electron microscopy imaging of the brain tissues was performed, which demonstrated
Laszewski, Audrey; Wichman, Christina L.; Doering, Jennifer J.; Maletta, Kristyn; Hammel, Jennifer
Early childhood professionals do many things to support young families. This is true now more than ever, as researchers continue to discover the long-term benefits of early, healthy, nurturing relationships. This article provides an overview of the development of an advanced practice perinatal depression algorithm created as a step-by-step guide…
... swine fever and bovine spongiform encephalopathy. 96.2 Section 96.2 Animals and Animal Products ANIMAL... Prohibition of casings due to African swine fever and bovine spongiform encephalopathy. (b) Casings from... spongiform encephalopathy. (a) Swine casings. The importation of swine casings that originated in or...
Bovine spongiform encephalopathy is a transmissible spongiform encephalopathy of domestic cattle. The disorder was reported in the United Kingdom in the late 1980s and was associated with recycling of ruminant byproducts in cattle feed. In 1996, the bovine disease was reported to be the cause of a...
... ``Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products,'' Docket No. APHIS-2008... regions for bovine spongiform encephalopathy (BSE) risk. Section 92.5 of the regulations provides that all... Animal and Plant Health Inspection Service Concurrence With OIE Risk Designations for Bovine...
Tzoulis, Charalampos; Neckelmann, Gesche; Mork, Sverre J.; Engelsen, Bernt E.; Viscomi, Carlo; Moen, Gunnar; Ersland, Lars; Zeviani, Massimo; Bindoff, Laurence A.
Mutations in the catalytic subunit of the mitochondrial DNA-polymerase gamma cause a wide spectrum of clinical disease ranging from infantile hepato-encephalopathy to juvenile/adult-onset spinocerebellar ataxia and late onset progressive external ophthalmoplegia. Several of these syndromes are associated with an encephalopathy that…
Transmissible Spongiform Encephalopathies (TSEs), including scrapie in sheep, chronic wasting disease (CWD) in cervids, and bovine spongiform encephalopathy (BSE), are fatal diseases of the nervous system associated with accumulation of misfolded prion protein (PrPSc). Different strains of BSE exist...
To determine if Spiroplasma mirum would be capable of producing lesions of spongiform encephalopathy in raccoons (Procyon lotor), 5 groups (n = 5) of raccoon kits were inoculated intracerebrally with either S. mirum and/or transmissible mink encephalopathy (TME). Two other groups (n = 5) of raccoon...
van Coeverden de Groot, H A
In 1980, a community perinatal service (CPS) facility was developed by the University of Cape Town in South Africa. This Peninsula Maternal and Neonatal Service (PMNS) is a model for understanding the objectives and essential requirements for the provision of a CPS. The goals of a CPS are to 1) use a single authority to provide integrated perinatal and family planning (FP) services for all women in a defined geographic area, 2) reduce infant morbidity and mortality to acceptable levels, 3) promote FP and a 2-child norm, and 4) provide education to staff, patients, and the community. A CPS must have a tiered system of perinatal care which has midwife obstetric units (MOUs) as the first level, secondary hospitals as the second, and tertiary hospitals as the third. The MOUs are centered around midwives, with a doctor available as a consultant to the midwives and as a provider of continuing education (CE) to the staff and patients. Staff CE takes place in perinatal mortality meetings, case discussions, orientation classes, refresher courses, outreach programs, and formal CE programs and journals. A loose-leaf, self-instructional perinatal education program is being developed to provide up-to-date information for every midwife and doctor. The midwives also may attend triennial congresses in Cape Town. The objective of patient education is to develop mothers as monitors of their own health and that of their fetuses and newborns. Patient education is achieved through the use of posters, lectures, and audiovisual programs. A CPS also needs appropriate equipment (a list is available from the World Health Organization). The CPS comprehensive referral system must cover all criteria and be respected throughout the region. The criteria must be updated regularly. Adequate communication channels and transportation facilities are also necessary to insure that a patient is transferred under the best conditions. Regular audits are essential and require accurate record keeping
Xu, Fenglian; Austin, Marie-Paule; Reilly, Nicole; Hilder, Lisa; Sullivan, Elizabeth A
This study aims to investigate hospital admission of major depressive disorders (MDD) before and after birth. Population data for all primiparous women admitted to the hospital with depressive disorders before and after birth were used. The comparison group consisted of 10 % of primiparous women not admitted to the hospital with a diagnosis of a psychiatric disorder or substance use. A total of 728 women had a first admission with depressive disorders (501 in the first postpartum year). The rate of first hospital admission for depressive disorders decreased during pregnancy and increased markedly in the first three months after birth (peaking in the second month with a rate of 10.74/1,000 person year and rate ratio of 12.56) compared with the 6 months prior to pregnancy. Admission remained elevated in the second postpartum year. Older maternal age, smoking, elective caesarian section and admission to a neonatal intensive care unit or special care nursery were associated with a higher rate of admission. Women born outside Australia and those most socioeconomically disadvantaged were less likely to be admitted to the hospital in the first postpartum year. Overall risk of hospital admission with depressive disorders rose significantly across the entire first postpartum year. This has significant implications for policy and service planning for women with mood disorders in the perinatal period.
Sivolap, Iu P; Damulin, I V
Wernicke's encephalopathy and Korsakoff's psychosis are severe unfavorable forms of alcoholic brain damage with poor prognosis. Thiamine deficiency represents a common cause of both diseases. In many cases, Korsakoff's psychosis develops in the outcome of Wernicke's encephalopathy, which, along with the general etiology, lets talk about a single disease - Wernicke-Korsakoff syndrome, acute (usually reversible) stage of which is Wernicke's encephalopathy and a chronic one (often irreversible) is Korsakoff psychosis. The dramatic paradox of Wernicke's encephalopathy is that in most cases it is difficult to detect, but early diagnosed cases are quite easy to cure. Unrecognized and therefore go untreated Wernicke's encephalopathy is a serious threat to the health and lives of patients, worsens the processes of brain aging and increases the risk of Alzheimer's disease in later life. The basic approach to the treatment of Wernicke-Korsakoff syndrome is long-term parenteral administration of thiamine, often in high doses. As an adjuvant means of therapy memantine is considered.
Vos, Amber A; van Voorst, Sabine F; Steegers, Eric A P; Denktaş, Semiha
Relatively high perinatal mortality and morbidity rates(2) in the Netherlands resulted in a process which induced policy changes regarding the Dutch perinatal healthcare system. Aims of this policy analysis are (1) to identify actors, context and process factors that promoted or impeded agenda setting and formulation of policy regarding perinatal health care reform and (2) to present an overview of the renewed perinatal health policy. The policy triangle framework for policy analysis by Walt and Gilson was applied(3). Contents of policy, actors, context factors and process factors were identified by triangulation of data from three sources: a document analysis, stakeholder analysis and semi-structured interviews with key stakeholders. Analysis enabled us to chronologically reconstruct the policy process in response to the perinatal mortality rates. The quantification of the perinatal mortality problem, the openness of the debate and the nature of the topic were important process factors. Main theme of policy was that change was required in the entire spectrum of perinatal healthcare. This ranged from care in the preconception phase through to the puerperium. Furthermore emphasis was placed on the importance of preventive measures and socio-environmental determinants of health. This required involvement of the preventive setting, including municipalities. The Dutch tiered perinatal healthcare system and divergent views amongst curative perinatal health care providers were important context factors. This study provides lessons which are applicable to health care professionals and policy makers in perinatal care or other multidisciplinary fields.
Bhutta, Zulfiqar A; Lassi, Zohra S; Blanc, Ann; Donnay, France
Some interventions in women before and during pregnancy may reduce perinatal and neonatal deaths, and recent research has established linkages of reproductive health with maternal, perinatal, and early neonatal health outcomes. In this review, we attempted to analyze the impact of biological, clinical, and epidemiologic aspects of reproductive and maternal health interventions on perinatal and neonatal outcomes through an elucidation of a biological framework for linking reproductive, maternal and newborn health (RHMNH); care strategies and interventions for improved perinatal and neonatal health outcomes; public health implications of these linkages and implementation strategies; and evidence gaps for scaling up such strategies. Approximately 1000 studies (up to June 15, 2010) were reviewed that have addressed an impact of reproductive and maternal health interventions on perinatal and neonatal outcomes. These include systematic reviews, meta-analyses, and stand-alone experimental and observational studies. Evidences were also drawn from recent work undertaken by the Child Health Epidemiology Reference Group (CHERG), the interconnections between maternal and newborn health reviews identified by the Global Alliance for Prevention of Prematurity and Stillbirth (GAPPS), as well as relevant work by the Partnership for Maternal, Newborn and Child Health. Our review amply demonstrates that opportunities for assessing outcomes for both mothers and newborns have been poorly realized and documented. Most of the interventions reviewed will require more greater-quality evidence before solid programmatic recommendations can be made. However, on the basis of our review, birth spacing, prevention of indoor air pollution, prevention of intimate partner violence before and during pregnancy, antenatal care during pregnancy, Doppler ultrasound monitoring during pregnancy, insecticide-treated mosquito nets, birth and newborn care preparedness via community-based intervention
Terada, Norihiko; Kinoshita, Kensuke; Taguchi, Shijima; Tokuda, Yasuharu
Deficiency of multiple vitamins can be identified in alcoholic and malnourished patients. We report a patient with Wernicke encephalopathy, a B1 deficiency and pellagra, a niacin deficiency. A 61-year-old Japanese man presented with generalised weakness. He had drunk alcohol heavily for more than a year without eating adequate meals. Physical examination showed disorientation, eye movement impairment, muscle wasting and a rash over the limbs. Multivitamin supplementations improved all the symptoms. Pellagra causes dementia, diarrhoea, or dermatitis, and can mimic non-specific erythaema in alcoholics. The differential diagnosis between pellagra and non-specific erythaema is important because of the treatability of pellagra by niacin supplementation.
Hepatic encephalopathy (HE) represents a broad continuum of neuropsychological dysfunction in patients with acute or chronic liver disease and/or portosystemic shunting of blood flow. The pathophysiology of this disease is quite complex, as it involves overproduction and reduced metabolism of various neurotoxins, particularly ammonia. Recent hypotheses implicate low-grade cerebral edema as a final common pathway for the pathophysiology of HE. Management of this condition is multifaceted and requires several steps: elimination of precipitating factors; removal of toxins, both by reducing them at their source and by augmenting scavenging pathways; modulation of resident fecal flora; proper nutritional support; and downregulation of systemic and gut-derived inflammation. PMID:21857820
Korngold, Caleb; Farrell, Helen M; Fozdar, Manish
The growing awareness of chronic traumatic encephalopathy (CTE) has the potential to change the public perception and on-field rules of the National Football League (NFL). More than 3,000 ex-NFL players or their relatives are engaged in litigation alleging that the NFL failed to acknowledge and address the neuropsychiatric risks associated with brain injuries that result from playing in the NFL. This article explores the intersection between the medical and legal aspects of CTE in the NFL from a forensic psychiatry perspective.
Ban, Vin Shen; Madden, Christopher J; Bailes, Julian E; Hunt Batjer, H; Lonser, Russell R
Recently, the pathobiology, causes, associated factors, incidence and prevalence, and natural history of chronic traumatic encephalopathy (CTE) have been debated. Data from retrospective case series and high-profile media reports have fueled public fear and affected the medical community's understanding of the role of sports-related traumatic brain injury (TBI) in the development of CTE. There are a number of limitations posed by the current evidence that can lead to confusion within the public and scientific community. In this paper, the authors address common questions surrounding the science of CTE and propose future research directions.
Kiemeneij, I M; de Leeuw, F-E; Ramos, L M P; van Gijn, J
A 24 year old woman presented with a sudden excruciating headache mimicking an acute vascular event. She had undergone a lung transplantation because of cystic fibrosis and was receiving maintenance treatment with tacrolimus and prednisone. Ancillary investigation excluded vascular causes. Magnetic resonance imaging demonstrated hyperintense lesions in the infratentorial and parieto-occipital regions consistent with posterior leucencephalopathy syndrome. Both her clinical condition improved and the lesions disappeared completely after withdrawal of tacrolimus, suggesting that her condition could be explained by a tacrolimus encephalopathy.
Naeini, Alireza E.; Daneshmand, Dana; Khorvash, Farzin; Chitsaz, Ahmad
VogtKoyanagi-Harada (VKH) is a rare syndrome affecting tissues with melanocytes. The possibility that VKH syndrome has an autoimmune pathogenesis is supported by the high frequency of human leukocyte antigen-DR4 commonly associated with other autoimmune diseases. Eyes are the main affected organ, resulting in blindness. Brain disease as a late onset event is extremely rare. Here, we are reporting a 57-year-old woman with previously diagnosed VKH syndrome, presenting with a late-onset brain encephalopathy. She was treated with corticosteroids and discharged from hospital with good general condition. PMID:23956579
Lucero, Catherine; Verna, Elizabeth C
Normal regulation of total body and circulating ammonia requires a delicate interplay in ammonia formation and breakdown between several organ systems. In the setting of cirrhosis and portal hypertension, the decreased hepatic clearance of ammonia leads to significant dependence on skeletal muscle for ammonia detoxification; however, cirrhosis is also associated with muscle depletion and decreased functional muscle mass. Thus, patients with diminished muscle mass and sarcopenia may have a decreased ability to compensate for hepatic insufficiency and a higher likelihood of developing physiologically significant hyperammonemia and hepatic encephalopathy.
Parekh, Parth J; Balart, Luis A
Hepatic encephalopathy (HE) is a commonly encountered sequela of chronic liver disease and cirrhosis with significant associated morbidity and mortality. Although ammonia is implicated in the pathogenesis of HE, the exact underlying mechanisms still remain poorly understood. Its role in the urea cycle, astrocyte swelling, and glutamine and gamma-amino-n-butyric acid systems suggests that the pathogenesis is multifaceted. Greater understanding in its underlying mechanism may offer more targeted therapeutic options in the future, and thus further research is necessary to fully understand the pathogenesis of HE.
Kodali, Sudha; McGuire, Brendan M
Hepatic encephalopathy (HE) is associated with cerebral edema (CE), increased intracranial pressure (ICP), and subsequent neurologic complications; it is the most important cause of morbidity and mortality in fulminant hepatic failure. The goal of therapy should be early diagnosis and treatment of HE with measures to reduce CE. A combination of clinical examination and diagnostic modalities can aid in prompt diagnosis. ICP monitoring and transcranial Doppler help diagnose and monitor response to treatment. Transfer to a transplant center and intensive care unit admission with airway management and reduction of CE with hypertonic saline, mannitol, hypothermia, and sedation are recommended as a bridge to liver transplantation.
Bosoi, Cristina R; Rose, Christopher F
Hepatic encephalopathy (HE), a complex neuropsychiatric syndrome, is a frequent complication of liver failure/disease. Increased concentrations of lactate are commonly observed in HE patients, in the systemic circulation, but also in the brain. Traditionally, increased cerebral lactate is considered a marker of energy failure/impairment however alterations in lactate homeostasis may also lead to a rise in brain lactate and result in neuronal dysfunction. The latter may involve the development of brain edema. This review will target the significance of increased cerebral lactate in the pathogenesis of HE.
Hörnlimann, B; Guidon, D; Griot, C
Since the occurrence of bovine spongiform encephalopathy (BSE) in Switzerland in 1990, extensive epidemiological investigations and risk factor analyses were carried out. In this study, statistical data on meat and bone meal traded from 1985 to 1989 were analysed addressing the following questions: i) what amount of meat and bone meal was exported from Great Britain (GB) and where to and ii) what amount of meat and bone meal was imported into Switzerland and where from? The findings led to the hypothesis that imported material potentially infected with scrapie-like agents was the cause for BSE in Switzerland.
Foley, C M; Polinsky, M S; Gruskin, A B; Baluarte, H J; Grover, W D
The examination of five pediatric patients with encephalopathy secondary to chronic renal failure has indicated a stereotyped sequence of neurologic signs and symptoms including ataxia, loss of motor abilities, myoclonus, seizures, dementia, and bulbar dysfunction. Both the patients with CNS dysfunction and a control group selected for a similar degree of renal failure had increased levels of serum phosphate, alkaline phosphatase, and parathyroid hormone. Serial EEGs in the affected group revealed progressive slowing and an increase in paroxysmal features. No specific neuropathologic findings were noted in one patient.
Orsolini, Laura; Valchera, Alessandro; Vecchiotti, Roberta; Tomasetti, Carmine; Iasevoli, Felice; Fornaro, Michele; De Berardis, Domenico; Perna, Giampaolo; Pompili, Maurizio; Bellantuono, Cesario
Perinatal period may pose a great challenge for the clinical management and treatment of psychiatric disorders in women. In fact, several mental illnesses can arise during pregnancy and/or following childbirth. Suicide has been considered a relatively rare event during the perinatal period. However, in some mental disorders (i.e., postpartum depression, bipolar disorder, postpartum psychosis, etc.) have been reported a higher risk of suicidal ideation, suicide attempt, or suicide. Therefore, a complete screening of mothers’ mental health should also take into account thoughts of suicide and thoughts about harming infants as well. Clinicians should carefully monitor and early identify related clinical manifestations, potential risk factors, and alarm symptoms related to suicide. The present paper aims at providing a focused review about epidemiological data, risk factors, and an overview about the main clinical correlates associated with the suicidal behavior during the pregnancy and postpartum period. Practical recommendations have been provided as well. PMID:27570512
Meuti, Valentina; Aceti, Franca; Giacchetti, Nicoletta; Carluccio, Giuseppe Mattia; Zaccagni, Michela; Marini, Isabella; Giancola, Orazio; Ciolli, Paola; Biondi, Massimo
Background. This study aims to verify if the presence and severity of perinatal depression are related to any particular pattern of attachment. Methods. The study started with a screening of a sample of 453 women in their third trimester of pregnancy, who were administered a survey data form, the Edinburgh Postnatal Depression Scale (EPDS) and the Experience in Close Relationship (ECR). A clinical group of subjects with perinatal depression (PND, 89 subjects) was selected and compared with a control group (C), regarding psychopathological variables and attachment patterns. Results. The ECR showed a prevalence of “Fearful-Avoidant” attachment style in PND group (29.2% versus 1.1%, p < 0.001); additionally, the EPDS average score increases with the increasing of ECR dimensions (Avoidance and Anxiety). Conclusion. The severity of depression increases proportionally to attachment disorganization; therefore, we consider attachment as both an important risk factor as well as a focus for early psychotherapeutic intervention. PMID:26798510
Lieberman, Kate; Le, Huynh-Nhu; Perry, Deborah F
Poor, adolescent, racial/ethnic minority women are at great risk for developing perinatal depression. However, little research has been conducted evaluating interventions for this population. We conducted a systematic review of preventive and treatment interventions for perinatal depression tested with adolescents, with a focus on low income, minority populations. Nine research-based articles (including one that reported on two studies) were reviewed systematically, and quality ratings were assigned based on a validated measure assessing randomization, double-blinding, and reporting of participant withdrawals. Two treatment studies were identified, both of which were successful in reducing depression. Eight prevention studies were located, of which four were more efficacious than control conditions in preventing depression. Studies sampled mostly minority, low socioeconomic status adolescents. No consistent characteristics across efficacious interventions could be identified. This review underscores the need for researchers to further investigate and build an evidence base.
Gaffin, Jonathan M.; Kanchongkittiphon, Watcharoot; Phipatanakul, Wanda
Background The prevalence of asthma has increased dramatically over the past several decades. While hereditary factors are highly important, the rapid rise outstrips the pace of genomic variation. Great emphasis has been placed on potential modifiable early life exposures leading to childhood asthma. Methods We reviewed the recent medical literature for important studies discussing the role of the perinatal and early childhood exposures and the inception of childhood asthma. Results and Discussion Early life exposure to allergens (House dust mite (HDM), furred pets, cockroach, rodent and mold)air pollution (nitrogen dioxide (NO2), ozone (O3), volatile organic compounds (VOCs), and particulate matter (PM)) and viral respiratory tract infections (Respiratory syncytial virus (RSV) and human rhinovirus (hRV)) have been implicated in the development of asthma in high risk children. Conversely, exposure to microbial diversity in the perinatal period may diminish the development of atopy and asthma symptoms. PMID:24952205
Improving breastfeeding rates among Black women is a potential strategy to address disparities in health outcomes that disproportionately impact Black women and children. This quality improvement (QI) initiative aimed to improve perinatal case manager knowledge and self-efficacy to promote breastfeeding among Black, low-income women who use services through Boston Healthy Start Initiative. QI methodology was used to develop and test a two-part strategy for perinatal case managers to promote and support breastfeeding. A positive change was observed in infant feeding knowledge and case manager self-efficacy to promote breastfeeding. Among the 24 mothers participating in this QI initiative, 100% initiated and continued breastfeeding at 1 week postpartum, and 92% were breastfeeding at 2 weeks postpartum.
Congden, Kimberly A.
ABSTRACT Parent education traditionally focuses on childbirth, whereas the perinatal period gets little attention despite parents’ reports of feeling unprepared. Lack of education surrounding newborn behavior leads to decreased maternal confidence and ineffective responsiveness to infant cues for feeding, crying, and sleep. This can cause overfeeding, lowered breastfeeding success, and contributes to parental stress which can impact maternal–infant bonding. Lack of postpartum maternal support adds to fatigue and stress which contributes to poor maternal well-being. This article describes an innovative perinatal program, Baystate’s New Beginnings, modeled after the education from the 2011 California Baby Behavior Campaign and The Secrets of Baby Behavior that combines newborn behavior education and maternal support in the first 3 months postpartum to improve maternal role transition. PMID:27445447
Zhang, Xin; Lv, Cong-Chao; Tian, Jiang; Miao, Ru-Juan; Xi, Wei; Hertz-Picciotto, Irva
We conducted a case–control study using 190 Han children with and without autism to investigate prenatal and perinatal risk factors for autism in China. Cases were recruited through public special education schools and controls from regular public schools in the same region (Tianjin), with frequency matching on sex and birth year. Unadjusted analyses identified seven prenatal and seven perinatal risk factors significantly associated with autism. In the adjusted analysis, nine risk factors showed significant association with autism: maternal second-hand smoke exposure, maternal chronic or acute medical conditions unrelated to pregnancy, maternal unhappy emotional state, gestational complications, edema, abnormal gestational age (<35 or >42 weeks), nuchal cord, gravidity >1, and advanced paternal age at delivery (>30 year-old). PMID:20358271
attachment, during subsequent pregnancies, between mothers who had experienced a previous perinatal loss and those who had not. A Likert Scale was used to...immediate postpartum period which effect attachment to the fetus and later to the infant? Are there key concerns/issues particular to these mothers...the main point behind encouraging bonding immedately during the postpartum period regardless of whether or not the baby lives. With resolution mothers
tested for 28. HIV. If yes, specify sex, age and result * PAST HEALTH HISTORY 1 - yes 2 - no 3 - not applicable 29. Heart Disease 29. 30. Lung...been reported to the Centers for Disease Control and it is now estimated that between 945,000 and 1.4 million persons in the United States are infected...HIV disease progression during pregnancy is uncommon. Perinatal transmission represents the primary route of HIV infection for children. Over 80% of all
Groenendaal, F; Lammers, H; Smit, D; Nikkels, P G J
Hypothesis Nitrotyrosine, a reaction product of peroxynitrite and proteins, could be demonstrated in the postmortem examination of brain tissue of full‐term neonates who had severe perinatal asphyxia. Methods The brain tissue of 22 full‐term neonates who died after severe perinatal asphyxia was examined, including cerebral cortex, basal ganglia, thalamus, hippocampus, brain stem, olives and cerebellum. Median age at death was 52 h. Routine histopathological examination and additional immunohistological staining were carried out with anti‐cysteine protease protein 32 antibodies to detect activated caspase 3, anti‐nitrotyrosine antibodies to detect nitrotyrosine and anti‐CD68 antibodies to detect activated microglia and macrophages, which might be associated with the production of nitric oxide. Staining was scored as none, weak (1–25% positive cells), moderate (26–75% positive cells) or severe (>75% positive cells). Results 14 patients showed global injury, 4 showed injury of the basal ganglia and thalamus, and 4 showed predominantly parasagittal brain injury. One neonate without perinatal asphyxia served as a control. Nitrotyrosine staining of neurones was shown in all neonates with asphyxia, mostly in the thalamus (70%) and inferior olives (68%). Total nitrotyrosine staining tended to be less in the base of the pons and inferior olives of neonates with parasagittal brain injury. Activated caspase 3 was found mostly in the thalamus (60%) and hippocampus (53%). Positive CD68 staining was mainly present in the thalamus (70% positive). Conclusion Nitrotyrosine was found in brain tissue of full‐term neonates, suggesting that nitric oxide toxicity might have a role in hypoxic–ischaemic brain injury at term. This may be relevant for neuroprotective strategies in full‐term neonates with perinatal asphyxia. PMID:16835259
Budin, Wendy C; Gennaro, Susan; OʼConnor, Caitlin; Contratti, Flavia
The purposes of this study were to describe changes in perinatal nurse (n = 70) and physician (n = 88) perceptions of teamwork and safety climate after implementing a 6-month Crew Resource Management training program and compare responses between nurses and physicians. The Teamwork and Safety Climate Survey was administered prior to and 1 year after the intervention. There were significant improvements in nurse and physician perceptions of teamwork and safety climate; however, physicians perceived teamwork more positive than nurses.
Weiner, Renay; Ronsmans, Carine; Dorman, Ed; Jilo, Hilton; Muhoro, Anne; Shulman, Caroline
OBJECTIVES: To identify and quantify risk factors for perinatal mortality in a Kenyan district hospital and to assess the proportion of perinatal deaths attributable to labour complications, maternal undernutrition, malaria, anaemia and human immunodeficiency virus (HIV). METHODS: A cross-sectional study of 910 births was conducted between January 1996 and July 1997 and risk factors for perinatal mortality were analysed. FINDINGS: The perinatal mortality rate was 118 per 1000 births. Complications of labour such as haemorrhage, premature rupture of membranes/premature labour, and obstructed labour/ malpresentation increased the risk of death between 8- and 62-fold, and 53% of all perinatal deaths were attributable to labour complications. Placental malaria and maternal HIV, on the other hand, were not associated with perinatal mortality. CONCLUSIONS: Greater attention needs to be given to the quality of obstetric care provided in the rural district-hospital setting. PMID:14576887
Lord, Catherine; Rieder, Amber; Hall, Geoffrey B C; Soares, Claudio N; Steiner, Meir
Onset/worsening of obsessive-compulsive disorder (OCD) during the perinatal period are frequently seen clinically. No specific tool assessing the unique content, context, severity, and onset of perinatal OCD exists. A self-report scale of perinatal obsessions and compulsions, the Perinatal Obsessive-Compulsive Scale (POCS), was developed and validated. A total of 162 women (67 pregnant, 95 postpartum) participated in this pilot study. They completed the POCS as well as the Yale-Brown Obsessive-Compulsive Scale (Y-BOCS). The POCS has good construct validity, reflected by representative items, high internal consistency, good concurrent validity and discriminative capacity. The most common obsessions were fear of having an unhealthy baby at birth, contamination, the baby being taken away, and infant death. Behavioral compulsions such as repeating rituals, asking for reassurance, checking, and cleaning mirrored these obsessions. The POCS helps clinicians detect perinatal OCD while giving perinatal women an opportunity to openly discuss socially sensitive issues.
Craswell, Alison; Moxham, Lorna; Broadbent, Marc
The collection of perinatal data within Queensland, Australia, has traditionally been achieved via a paper form completed by midwives after each birth. Recently, with an increase in the use of e-health systems in healthcare, perinatal data collection has migrated to an online system. It is suggested that this move from paper to an ehealth platform has resulted in improvement to error rates, completion levels, timeliness of data transfer from healthcare institutions to the perinatal data collection and subsequent publication of data items. Worldwide, perinatal data are collected utilising a variety of methods, but essentially data are used for similar purposes: to monitor outcome patterns within obstetrics and midwifery. This paper discusses current practice in relation to perinatal data collection worldwide and within Australia, with a specific focus on Queensland, highlights relevant issues for midwives, and points to the need for further research into the efficient use of an e-health platform for perinatal data collection.
Nolan, Monica; Fowler, Mary Glenn; Mofenson, Lynne M
Since 1994, trials of zidovudine, zidovudine and lamivudine, and nevirapine have demonstrated that these antiretroviral drugs can substantially reduce the risk of perinatal HIV-1 transmission. With reductions in drug price, identification of simple, effective antiretroviral regimens to prevent perinatal HIV-1 transmission, and an increasing international commitment to support health care infrastructure, antiretrovirals for both perinatal HIV-1 prevention and HIV-1 treatment will likely become more widely available to HIV-1-infected persons in resource-limited countries. In the United States, widespread antiretroviral usage has been associated with increased antiretroviral drug resistance. This raises concern that drug resistance may reduce the effectiveness of perinatal antiretroviral prophylaxis as well as therapeutic intervention strategies. The purpose of this article is to review what is known about resistance and risk of perinatal HIV transmission, assess the interaction between antiretroviral resistance and the prevention of perinatal HIV-1 transmission, and discuss implications for current global prevention and treatment strategies.
Mofenson, Lynne M; Cotton, Mark F
The great success in the prevention and treatment of pediatric HIV in high resource countries, and now in low resource countries, has changed the face of the HIV epidemic in children from one of near certain mortality to that of a chronic disease. However, these successes pose new challenges as perinatally HIV-infected youth survive into adulthood. Increased survival of HIV-infected children is associated with challenges in maintaining adherence to what is likely life-long therapy, and in selecting successive antiretroviral drug regimens, given the limited availability of pediatric formulations, limitations in pharmacokinetic and safety data of drugs in children, and the development of extensive drug resistance in multi-drug-experienced children. Pediatric HIV care must now focus on morbidity related to long-term HIV infection and its treatment. Survival into adulthood of perinatally HIV-infected youth in high resource countries provides important lessons about how the epidemic will change with increasing access to antiretroviral therapy for children in low resource countries. This series of papers will focus on issues related to management of perinatally infected youth and young adults.
Mofenson, Lynne M; Cotton, Mark F
The great success in the prevention and treatment of pediatric HIV in high resource countries, and now in low resource countries, has changed the face of the HIV epidemic in children from one of near certain mortality to that of a chronic disease. However, these successes pose new challenges as perinatally HIV-infected youth survive into adulthood. Increased survival of HIV-infected children is associated with challenges in maintaining adherence to what is likely life-long therapy, and in selecting successive antiretroviral drug regimens, given the limited availability of pediatric formulations, limitations in pharmacokinetic and safety data of drugs in children, and the development of extensive drug resistance in multi-drug-experienced children. Pediatric HIV care must now focus on morbidity related to long-term HIV infection and its treatment. Survival into adulthood of perinatally HIV-infected youth in high resource countries provides important lessons about how the epidemic will change with increasing access to antiretroviral therapy for children in low resource countries. This series of papers will focus on issues related to management of perinatally infected youth and young adults. PMID:23782484
Vannucci, R C
Animal research has provided important information on the pathogenesis of and neuropathologic responses to perinatal cerebral hypoxia-ischemia. In experimental animals, structural brain damage from hypoxia-ischemia has been produced in immature rats, rabbits, guinea pigs, sheep and monkeys (18, 20, 24, 25, 38). Of the several available animal models, the fetal and newborn rhesus monkey and immature rat have been studied most extensively because of their similarities to humans in respect to the physiology of reproduction and their neuroanatomy at or shortly following birth. Given the frequency of occurrence of human perinatal hypoxic-ischemic brain damage and the multiple, often severe neurologic handicaps which ensue in infants and children, it is not surprising that the above described animal models have been developed. These models have provided the basis for investigations to clarify not only physiologic and biochemical mechanisms of tissue injury but also the efficacy of specific management strategies. Hopefully, such animal research will continue to provide important information regarding how best to prevent or minimize the devastating consequences of perinatal cerebral hypoxia-ischemia.
Bhat, Amritha; Byatt, Nancy
Infertility and perinatal loss are common, and associated with lower quality of life, marital discord, complicated grief, major depressive disorder, anxiety disorders, and post-traumatic stress disorder. Young women, who lack social supports, have experienced recurrent pregnancy loss or a history of trauma and / or preexisting psychiatric illness are at a higher risk of experiencing psychiatric illnesses or symptoms after a perinatal loss or during infertility. It is especially important to detect, assess, and treat depression, anxiety, or other psychiatric symptoms because infertility or perinatal loss may be caused or perpetuated by such symptoms. Screening, psychoeducation, provision of resources and referrals, and an opportunity to discuss their loss and plan for future pregnancies can facilitate addressing mental health concerns that arise. Women at risk of or who are currently experiencing psychiatric symptoms should receive a comprehensive treatment plan that includes the following: (1) proactive clinical monitoring, (2) evidence-based approaches to psychotherapy, and (3) discussion of risks, benefits, and alternatives of medication treatment during preconception and pregnancy.
Shin, Incheol; Kim, Hyun J; Lee, Jae E; Gye, Myung C
Emerging evidence suggests that brain aquaporins (AQPs) play important roles in the dynamic regulation of brain water homeostasis and the production of cerebrospinal fluid (CSF) under normal, as well as pathological, conditions. To date, the spatiotemporal expression patterns of AQP1, 4, and 9 have been elucidated in brain tissues. However, the expression of AQP7, an aquaglyceroporin associated with brain development, has not been shown. In the present study, we examined expression of AQP7 during perinatal and adult brain development in the mouse. Throughout brain development, the immunoreactivity of AQP7 was largely found in the choroid plexus (CP). AQP7 immunoreactivity in ependyma (Ep), pia, and blood vessels (BV) was increased during perinatal to postnatal development. Cells in the different layers of cerebral cortex became a little positive for AQP7 immunoreactivity during postnatal development. Optimized semi-quantitative RT-PCR and Western blot analysis revealed that AQP7 mRNA and protein levels increased during perinatal development of brain. To our knowledge, this is the first report on the pattern of AQP7 expression in brain tissues. These results suggest that AQP7 is an important structural element in the choroid plexus and is possibly involved in the production of CSF during brain development in mice.
Background We examined the optimal gestational weight gain (GWG) in the healthy Japanese women with favorable perinatal outcomes of singleton pregnancy. Methods We calculated the average GWG in the women whose height was 150 - 164 cm with favorable perinatal outcomes set for this study. The women were categorized to underweight, normal, overweight and obese based on the pre-pregnancy body mass index categories according to the Institute of Medicine guideline. Results The average GWG in the normal-weight women with the favorable perinatal outcomes was 11.4 ± 3.7 kg. It was not significantly different from that in the underweight and overweight women (12.0 ± 3.4 and 10.0 ± 4.8 kg) by Student’s t-test. The average GWG in the obese women was significantly lower than that in the other three groups (3.2 ± 2.2 kg, P < 0.01). Conclusion Based on the current results, the optimal GWG for the Japanese women without obesity was found to be 10 - 12 kg. PMID:27924177
Jonker, Sonnet S.; Louey, Samantha; Giraud, George D.; Thornburg, Kent L.; Faber, J. Job
Studies in altricial rodents attribute dramatic changes in perinatal cardiomyocyte growth, maturation, and attrition to stimuli associated with birth. Our purpose was to determine whether birth is a critical trigger controlling perinatal cardiomyocyte growth, maturation and attrition in a precocial large mammal, sheep (Ovis aries). Hearts from 0–61 d postnatal lambs were dissected or enzymatically dissociated. Cardiomyocytes were measured by micromorphometry, cell cycle activity assessed by immunohistochemistry, and nuclear number counted after DNA staining. Integration of this new data with published fetal data from our laboratory demonstrate that a newly appreciated >30% decrease in myocyte number occurred in the last 10 d of gestation (P < 0.0005) concomitant with an increase in cleaved poly (ADP-ribose) polymerase 1 (P < 0.05), indicative of apoptosis. Bisegmental linear regressions show that most changes in myocyte growth kinetics occur before birth (median = 15.2 d; P < 0.05). Right ventricular but not left ventricular cell number increases in the neonate, by 68% between birth and 60 d postnatal (P = 0.028). We conclude that in sheep few developmental changes in cardiomyocytes result from birth, excepting the different postnatal degrees of free wall hypertrophy between the ventricles. Furthermore, myocyte number is reduced in both ventricles immediately before term, but proliferation increases myocyte number in the neonatal right ventricle.—Jonker, S. S., Louey, S., Giraud, G. D., Thornburg, K. L., Faber, J. J. Timing of cardiomyocyte growth, maturation, and attrition in perinatal sheep. PMID:26139099
Souto, Pablo A; Marcotegui, Ariel R; Orbea, Lisandro; Skerl, Juan; Perazzo, Juan Carlos
Hepatic encephalopathy (HE) is a neuropsychiatric disorder that commonly complicates the course of patients with liver disease. Despite the fact that the syndrome was probably first recognized hundreds of years ago, the exact pathogenesis still remains unclear. Minimal hepatic encephalopathy (MHE) is the earliest form of HE and is estimated to affect more that 75% of patients with liver cirrhosis. It is characterized by cognitive impairment predominantly attention, reactiveness and integrative function with very subtle clinical manifestations. The development of MHE is associated with worsen in driving skills, daily activities and the increase of overall mortality. Skeletal muscle has the ability to shift from ammonia producer to ammonia detoxifying organ. Due to its large size, becomes the main ammonia detoxifying organ in case of chronic liver failure and muscular glutamine-synthase becomes important due to the failing liver and brain metabolic activity. Gut is the major glutamine consumer and ammonia producer organ in the body. Hepatocellular dysfunction due to liver disease, results in an impaired clearance of ammonium and in its inter-organ trafficking. Intestinal bacteria, can also represent an extra source of ammonia production and in cirrhosis, small intestinal bacterial overgrowth and symbiosis can be observed. In the study of HE, to get close to MHE is to get closer to its big bang; and from here, to travel less transited roads such as skeletal muscle and intestine, is to go even closer. The aim of this editorial is to expose this road for further and deeper work.
Souto, Pablo A; Marcotegui, Ariel R; Orbea, Lisandro; Skerl, Juan; Perazzo, Juan Carlos
Hepatic encephalopathy (HE) is a neuropsychiatric disorder that commonly complicates the course of patients with liver disease. Despite the fact that the syndrome was probably first recognized hundreds of years ago, the exact pathogenesis still remains unclear. Minimal hepatic encephalopathy (MHE) is the earliest form of HE and is estimated to affect more that 75% of patients with liver cirrhosis. It is characterized by cognitive impairment predominantly attention, reactiveness and integrative function with very subtle clinical manifestations. The development of MHE is associated with worsen in driving skills, daily activities and the increase of overall mortality. Skeletal muscle has the ability to shift from ammonia producer to ammonia detoxifying organ. Due to its large size, becomes the main ammonia detoxifying organ in case of chronic liver failure and muscular glutamine-synthase becomes important due to the failing liver and brain metabolic activity. Gut is the major glutamine consumer and ammonia producer organ in the body. Hepatocellular dysfunction due to liver disease, results in an impaired clearance of ammonium and in its inter-organ trafficking. Intestinal bacteria, can also represent an extra source of ammonia production and in cirrhosis, small intestinal bacterial overgrowth and symbiosis can be observed. In the study of HE, to get close to MHE is to get closer to its big bang; and from here, to travel less transited roads such as skeletal muscle and intestine, is to go even closer. The aim of this editorial is to expose this road for further and deeper work. PMID:27895414
Spina, Roberto; Simon, Neil; Markus, Romesh; Muller, David W M; Kathir, Krishna
Contrast-induced encephalopathy (CIE) is an acute and reversible neurological disturbance associated with the intra-arterial administration of iodinated contrast medium during cardiac catheterisation. It may manifest with encephalopathy, motor and sensory disturbances; vision disturbances, including cortical blindness, ophthalmoplegia, aphasia; and seizures. Disruption of the blood-brain barrier and direct neuronal toxicity are believed to be implicated in the pathophysiology of the syndrome. Symptoms appear soon after contrast administration and resolve completely within 24-48 h. Risk factors may include hypertension, diabetes mellitus, renal impairment, the administration of large volumes of iodinated contrast, percutaneous coronary intervention or selective angiography of internal mammary grafts and previous adverse reaction to iodinated contrast. On cerebral imaging, CIE may mimic subarachnoid haemorrhage or cerebral ischaemia, but imaging may be normal. Prognosis is excellent with supportive management alone. CIE may recur, but re-challenge with iodinated contrast without adverse effects has been documented. CIE is a diagnosis of exclusion and is an important clinical entity to consider in the differential diagnosis of stroke following cardiac catheterisation. Physicians should be aware of it and consider it prior to initiating thrombolysis.
Kheir, John N; Lawlor, Michael W; Ahn, Edward S; Lehmann, Leslie; Riviello, James J; Silvera, V Michelle; McManus, Michael; Folkerth, Rebecca D
The pathology of posterior reversible encephalopathy syndrome (PRES) is undefined, since it is rarely fatal and is biopsied in only exceptional circumstances. We describe rapidly progressive PRES following stem cell transplant for acute lymphoblastic leukemia. After development of altered mental status, this 8-year-old girl had T2 prolongation of the white matter in a posterior-dominant distribution, eventually developing cerebellar edema, hemorrhage, hydrocephalus, and herniation. Despite surgical and medical management, she died 36 hours later. At autopsy, the occipital and cerebellar white matter and focal occipital cortical gray matter showed a spectrum of microvascular changes, including dilated perivascular spaces containing proteinaceous exudates and macrophages, as well as fibrinoid necrosis and acute hemorrhage, in a distribution corresponding to the neuroimaging abnormalities and reminiscent of those seen in patients with acute hypertensive encephalopathy. Of note, similar microvascular changes were not seen in the kidney or other systemic sites. Thus, the findings indicate a brain-specific microvascular compromise as the substrate of PRES, at least in the rare instance of cases progressing to fatal outcome.
Tripathi, Suyash; Tripathi, Yamini B
Hepatic encephalopathy is a brain functional disorder, characterized by neuropsychiatric abnormalities with liver failure. High blood ammonia, causing glutamate neurotoxicity is the basic cause, finally leading to low-grade cerebral edema. Its manifestation is more likely in patients of sepsis, oxidative stress, generalized inflammation, gut mal-functioning, amoebiaesis, viral hepatitis, nervous imbalance, etc. Thus, the therapeutic goals primarily include the maintenance of proper blood supply and prevention of hypoxic condition in liver, along with management of factors responsible for high blood ammonia, oxidative stress, inflammation, and high GI- serotonin. The drugs in clinical practice include lactulose, sodium benzoate, flumazenil and rifaximin, supplementation of zinc, branched chain amino acids (BCAA), l-ornithine-l aspartate, antioxidants and iNOS inhibitors. However, herbal formulations would be of great importance as it shows multi-targeted action because it possesses a natural cocktail of secondary metabolites. It can collectively act as an antioxidant, anti-inflammatory, prebiotic, hepatoprotective and neuron-protective agents. We have briefly outlined some of these plants and also recent patents useful in the management of hepatic encephalopathy.