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Sample records for perinatal asphyxial encephalopathy

  1. Blood Biomarkers for Evaluation of Perinatal Encephalopathy

    PubMed Central

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

    2016-01-01

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

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

    ERIC Educational Resources Information Center

    Mitchell, Charles D.

    2006-01-01

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

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

    PubMed Central

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

    2010-01-01

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

  4. Perinatal risk factors for severe injury in neonates treated with whole-body hypothermia for encephalopathy

    PubMed Central

    Wayock, Christopher P.; Meserole, Rachel L.; Saria, Suchi; Jennings, Jacky M.; Huisman, Thierry A. G. M.; Northington, Frances J.; Graham, Ernest M.

    2016-01-01

    Objective Our objective was to identify perinatal risk factors that are available within 1 hour of birth that are associated with severe brain injury after hypothermia treatment for suspected hypoxic-ischemic encephalopathy. Study Design One hundred nine neonates at ≥35 weeks' gestation who were admitted from January 2007 to September 2012 with suspected hypoxic-ischemic encephalopathy were treated with whole-body hypothermia; 98 of them (90%) underwent brain magnetic resonance imaging (MRI) at 7-10 days of life. Eight neonates died before brain imaging. Neonates who had severe brain injury, which was defined as death or abnormal MRI results (cases), were compared with surviving neonates with normal MRI (control subjects). Logistic regression models were used to identify risk factors that were predictive of severe injury. Results Cases and control subjects did not differ with regard to gestational age, birthweight, mode of delivery, or diagnosis of non-reassuring fetal heart rate before delivery. Cases were significantly (P ≤ .05) more likely to have had an abruption, a cord and neonatal arterial gas level that showed metabolic acidosis, lower platelet counts, lower glucose level, longer time to spontaneous respirations, intubation, chest compressions in the delivery room, and seizures. In multivariable logistic regression, lower initial neonatal arterial pH (P = .004), spontaneous respiration at >30 minutes of life (P = .002), and absence of exposure to oxytocin (P = .033) were associated independently with severe injury with 74.3% sensitivity and 74.4% specificity. Conclusion Worsening metabolic acidosis at birth, longer time to spontaneous respirations, and lack of exposure to oxytocin correlated with severe brain injury in neonates who were treated with whole-body hypothermia. These risk factors may help quickly identify neonatal candidates for time-sensitive investigational therapies for brain neuroprotection. PMID:24657795

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

    ERIC Educational Resources Information Center

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

    2010-01-01

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

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

    PubMed Central

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

    2016-01-01

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

  7. Does aetiology of neonatal encephalopathy and hypoxic-ischaemic encephalopathy influence the outcome of treatment?

    PubMed

    Mcintyre, Sarah; Badawi, Nadia; Blair, Eve; Nelson, Karin B

    2015-04-01

    Neonatal encephalopathy, a clinical syndrome affecting term-born and late preterm newborn infants, increases the risk of perinatal death and long-term neurological morbidity, especially cerebral palsy. With the advent of therapeutic hypothermia, a treatment designed for hypoxic or ischaemic injury, associated mortality and morbidity rates have decreased. Unfortunately, only about one in eight neonates (95% confidence interval) who meet eligibility criteria for therapeutic cooling apparently benefit from the treatment. Studies of infants in representative populations indicate that neonatal encephalopathy is a potential result of a variety of antecedents and that asphyxial complications at birth account for only a small percentage of neonatal encephalopathy. In contrast, clinical case series suggest that a large proportion of neonatal encephalopathy is hypoxic or ischaemic, and trials of therapeutic hypothermia are specifically designed to include only infants exposed to hypoxia or ischaemia. This review addresses the differences, definitional and methodological, between infants studied and investigations undertaken, in population studies compared with cooling trials. It raises the question if there may be subgroups of infants with a clinical diagnosis of hypoxic-ischaemic encephalopathy (HIE) in whom the pathobiology of neonatal neurological depression is not fundamentally hypoxic or ischaemic and, therefore, for whom cooling may not be beneficial. In addition, it suggests approaches to future trials of cooling plus adjuvant therapy that may contribute to further improvement of care for these vulnerable neonates.

  8. Does aetiology of neonatal encephalopathy and hypoxic-ischaemic encephalopathy influence the outcome of treatment?

    PubMed

    Mcintyre, Sarah; Badawi, Nadia; Blair, Eve; Nelson, Karin B

    2015-04-01

    Neonatal encephalopathy, a clinical syndrome affecting term-born and late preterm newborn infants, increases the risk of perinatal death and long-term neurological morbidity, especially cerebral palsy. With the advent of therapeutic hypothermia, a treatment designed for hypoxic or ischaemic injury, associated mortality and morbidity rates have decreased. Unfortunately, only about one in eight neonates (95% confidence interval) who meet eligibility criteria for therapeutic cooling apparently benefit from the treatment. Studies of infants in representative populations indicate that neonatal encephalopathy is a potential result of a variety of antecedents and that asphyxial complications at birth account for only a small percentage of neonatal encephalopathy. In contrast, clinical case series suggest that a large proportion of neonatal encephalopathy is hypoxic or ischaemic, and trials of therapeutic hypothermia are specifically designed to include only infants exposed to hypoxia or ischaemia. This review addresses the differences, definitional and methodological, between infants studied and investigations undertaken, in population studies compared with cooling trials. It raises the question if there may be subgroups of infants with a clinical diagnosis of hypoxic-ischaemic encephalopathy (HIE) in whom the pathobiology of neonatal neurological depression is not fundamentally hypoxic or ischaemic and, therefore, for whom cooling may not be beneficial. In addition, it suggests approaches to future trials of cooling plus adjuvant therapy that may contribute to further improvement of care for these vulnerable neonates. PMID:25800486

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

    PubMed

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

    2015-01-01

    Oxidative stress is believed to greatly contribute to the pathogenesis of various diseases, including neurodegeneration. Impairment of mitochondrial energy production and increased mitochondrial oxidative damage are considered early pathological events that lead to neurodegeneration. Manganese superoxide dismutase (Mn-SOD, SOD2) is a mitochondrial antioxidant enzyme that converts toxic superoxide to hydrogen peroxide. To investigate the pathological role of mitochondrial oxidative stress in the central nervous system, we generated brain-specific SOD2-deficient mice (B-Sod2(-/-)) using nestin-Cre-loxp system. B-Sod2(-/-) showed perinatal death, along with severe growth retardation. Interestingly, these mice exhibited spongiform neurodegeneration in motor cortex, hippocampus, and brainstem, accompanied by gliosis. In addition, the mutant mice had markedly decreased mitochondrial complex II activity, but not complex I or IV, in the brain based on enzyme histochemistry. Furthermore, brain lipid peroxidation was significantly increased in the B-Sod2(-/-), without any compensatory alterations of the activities of other antioxidative enzymes, such as catalase or glutathione peroxidase. These results suggest that SOD2 protects the neural system from oxidative stress in the perinatal stage and is essential for infant survival and central neural function in mice. PMID:26301039

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

    PubMed Central

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

    2015-01-01

    Oxidative stress is believed to greatly contribute to the pathogenesis of various diseases, including neurodegeneration. Impairment of mitochondrial energy production and increased mitochondrial oxidative damage are considered early pathological events that lead to neurodegeneration. Manganese superoxide dismutase (Mn-SOD, SOD2) is a mitochondrial antioxidant enzyme that converts toxic superoxide to hydrogen peroxide. To investigate the pathological role of mitochondrial oxidative stress in the central nervous system, we generated brain-specific SOD2-deficient mice (B-Sod2−/−) using nestin-Cre-loxp system. B-Sod2−/− showed perinatal death, along with severe growth retardation. Interestingly, these mice exhibited spongiform neurodegeneration in motor cortex, hippocampus, and brainstem, accompanied by gliosis. In addition, the mutant mice had markedly decreased mitochondrial complex II activity, but not complex I or IV, in the brain based on enzyme histochemistry. Furthermore, brain lipid peroxidation was significantly increased in the B-Sod2−/−, without any compensatory alterations of the activities of other antioxidative enzymes, such as catalase or glutathione peroxidase. These results suggest that SOD2 protects the neural system from oxidative stress in the perinatal stage and is essential for infant survival and central neural function in mice. PMID:26301039

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

    PubMed

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

    2015-01-01

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

  12. Angiogenesis Dysregulation in Term Asphyxiated Newborns Treated with Hypothermia

    PubMed Central

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

    2015-01-01

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

  13. Measurement of the Urinary Lactate/Creatinine Ratio for Early Diagnosis of the Hypoxic–Ischemic Encephalopathy in Newborns

    PubMed Central

    Ghotbi, Nahid; Najibi, Babak

    2010-01-01

    Objective Hypoxic ischemic encephalopathy (HIE) is a major cause of permanent neurological disabilities. Perinatal asphyxia may induce neonatal mortality after birth or neurological impairment among survivors. There are no reliable methods for identifying infants at risk for this disorder. Methods We measured the ratio of lactate/creatinine (L/C) in urine by proton nuclear magnetic resonance spectroscopy within 6 and 24 hours after birth in 50 normal infants and 50 infants with asphyxia who developed hypoxic–ischemic encephalopathy. The study was performed from September 2006 to May 2007. For statistical analysis, the SPSS software was used. Group comparisons were performed with chi-square and t-test(1, 5). Findings L/C ratio was 3.3±2 among asphyxiated neonates in the first six hours after birth which was 11 folds greater than in normal neonates (0.3±0.08, P=0.0001). This ratio decreased to 1.5±0.55 for asphyxiated cases in the first 24 hours after birth, which was 5 folds greater than in control group (P=0.0001). Asphyxiated neonates were subdivided into Group A with mild asphyxia and L/C ratio 2.5±0.5; Group B with moderate asphyxia and L/C ratio 4.2±1.5; and Group C with severe asphyxia and L/C ratio 3.4±3.3. The severity of asphyxia correlated with the greater L/C ratio among our cases and was significant (P=0.0007). The sensitivity and specificity of L/C ratio in cut off point of 0.48, was 96.1% and 100% respectively. Conclusion Measurement of the urinary L/C ratio soon after birth maybe a promising tool to identify asphyxiated neonates and also to predict the severity of asphyxia. PMID:23056679

  14. Vasoparalysis associated with brain damage in asphyxiated term infants

    SciTech Connect

    Pryds, O.; Greisen, G.; Lou, H.; Friis-Hansen, B. )

    1990-07-01

    The relationship of cerebral blood flow to acute changes in arterial carbon dioxide and mean arterial blood pressure (MABP) was determined during the first day of life in 19 severely asphyxiated term infants supported by mechanical ventilation. For comparison, 12 infants without perinatal asphyxia were also investigated. Global cerebral blood flow (CBF infinity) was determined by xenon 133 clearance two or three times within approximately 2 hours. During the cerebral blood flow measurement, the amplitude-integrated electroencephalogram and visual-evoked potential were recorded. Changes in arterial carbon dioxide pressure followed adjustments of the ventilator settings, whereas MABP fluctuated spontaneously. Arterial oxygen pressure and blood glucose concentration were in the normal range. Five of the asphyxiated infants had isoelectric electroencephalograms and died subsequently with severe brain damage. They had a high CBF infinity (mean 30.6 ml/100 gm/min) and abolished carbon dioxide and MABP reactivity. Lower CBF infinity (mean 14.7 ml/100 gm/min) and abolished MABP reactivity were found in another five asphyxiated infants with burst-suppression electroencephalograms in whom computed tomographic or clinical signs of brain lesions developed. The carbon dioxide reactivity was preserved in these infants. In the remaining nine asphyxiated infants without signs of central nervous system abnormality, carbon dioxide and MABP reactivity were preserved, as was also the case in the control group. We conclude that abolished autoregulation is associated with cerebral damage in asphyxiated infants and that the combination of isoelectric electroencephalograms and cerebral hyperperfusion is an early indicator of very severe brain damage.

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

    PubMed

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

    2004-01-01

    We conducted a retrospective analysis of 10 cases of congenital laryngeal stridor. Reports of laryngeal endoscopy and diagnosis define laryngomalacia as laryngeal flaccidity and stridor. Some authors postulate that in addition to immaturity of cartilage, there exist the possibility of laryngeal uncoordination and dyskinesia. They support this idea in cases of late presentation, neurological damage, and atypical cases related with functional state or anesthesia. Laryngeal endoscopies were carried out in 10 cases included in a cohort of subjects from a longitudinal follow-up diagnosed with neurologica damage of perinatal origin. One case was diagnosed with postoperative unilateral paralysis of vocal chord and another identified vascular ring. The eight remaining cases fulfilled laryngomalacia criteria of diagnosis, but because of their characteristics origin is not an anatomic alteration but a functional hypotonia. The need to carry out an integral study to describe co-morbidity is emphasized. PMID:15559228

  16. Serum and Urinary Malondialdehyde (MDA), Uric acid, and Protein as markers of perinatal asphyxia

    PubMed Central

    El Bana, Sawsan Mahmoud; Maher, Sheren Esam; Gaber, Amani Fawzy; Aly, Sanaa Shaker

    2016-01-01

    Introduction Perinatal asphyxia (PA) is among the leading causes of neonatal morbidity and death in neonatal intensive care units (NICUs). The aims of this research were to determine the concentrations of malondialdehyde (MDA), urine MDA, uric acid, and protein in the cord blood of neonates with perinatal asphyxia and to determine their relationship with the severity of perinatal asphyxia. Methods This matched case-control study was conducted from October 2012 to March 2013. All of the cases and controls were selected from the Gynecology & Obstetrics Department and the NICUs, at Qous Central Hospital in Qena, Egypt. We allocated 20 full-term neonates who had perinatal asphyxia to the case group. Also, we selected 20 healthy neonates for the control group. The subjects were matched with respect to age and gender. At birth and 48 hours later, measurements were made of MDA in cord blood and urine, and uric acid, protein, and creatinine also were measured in both groups. The data were analyzed by SPSS, version 17, using the independent-samples t-test, ANOVA, Tukey’s test, and Spearman’s correlation coefficient. Results At birth and 48 hr later, the newborns’ with PA had significantly higher levels of MDA in the cord blood, mean urinary uric acid/creatinine (UUA:Cr), protein/creatinine (UP:Cr), and MDA/creatinine ratio (UMDA:Cr) than the controls; their PA levels were correlated with the degree of hypoxic-ischemic encephalopathy (HIE). The babies who died due to PA had significantly higher levels of cord blood MDA, and they also had higher UUA:Cr, UP:Cr, and UMDA:Cr ratios than the babies who survived. Conclusion The concentration of MDA in cord blood can be used as a diagnostic marker of oxidative stress in asphyxiated neonates. The ratios of the urinary excretion rates of uric acid, protein, and MDA to creatinine increased as the severity of perinatal asphyxia and associated brain damage increased. PMID:27648187

  17. Serum and Urinary Malondialdehyde (MDA), Uric acid, and Protein as markers of perinatal asphyxia

    PubMed Central

    El Bana, Sawsan Mahmoud; Maher, Sheren Esam; Gaber, Amani Fawzy; Aly, Sanaa Shaker

    2016-01-01

    Introduction Perinatal asphyxia (PA) is among the leading causes of neonatal morbidity and death in neonatal intensive care units (NICUs). The aims of this research were to determine the concentrations of malondialdehyde (MDA), urine MDA, uric acid, and protein in the cord blood of neonates with perinatal asphyxia and to determine their relationship with the severity of perinatal asphyxia. Methods This matched case-control study was conducted from October 2012 to March 2013. All of the cases and controls were selected from the Gynecology & Obstetrics Department and the NICUs, at Qous Central Hospital in Qena, Egypt. We allocated 20 full-term neonates who had perinatal asphyxia to the case group. Also, we selected 20 healthy neonates for the control group. The subjects were matched with respect to age and gender. At birth and 48 hours later, measurements were made of MDA in cord blood and urine, and uric acid, protein, and creatinine also were measured in both groups. The data were analyzed by SPSS, version 17, using the independent-samples t-test, ANOVA, Tukey’s test, and Spearman’s correlation coefficient. Results At birth and 48 hr later, the newborns’ with PA had significantly higher levels of MDA in the cord blood, mean urinary uric acid/creatinine (UUA:Cr), protein/creatinine (UP:Cr), and MDA/creatinine ratio (UMDA:Cr) than the controls; their PA levels were correlated with the degree of hypoxic-ischemic encephalopathy (HIE). The babies who died due to PA had significantly higher levels of cord blood MDA, and they also had higher UUA:Cr, UP:Cr, and UMDA:Cr ratios than the babies who survived. Conclusion The concentration of MDA in cord blood can be used as a diagnostic marker of oxidative stress in asphyxiated neonates. The ratios of the urinary excretion rates of uric acid, protein, and MDA to creatinine increased as the severity of perinatal asphyxia and associated brain damage increased.

  18. Auto-erotic asphyxiation: three case reports.

    PubMed

    Cooper, A J

    1996-01-01

    Men who indulge in auto-erotic asphyxial practices are rarely encountered by psychiatrists. More often, they come to light at a coroner's inquest, and a verdict of "accidental death" is usually rendered (e.g., from hanging/suffocation). This paper describes and contrasts three cases of auto-erotic asphyxial practices. The first case, of accidental death by hanging, was an apparently highly successful young man who functioned at a high level and was without "serious psychopathology." In contrast, the other two, both survivors of asphyxial practices, manifested multiple paraphilias and other types of psychopathology. The syndrome may be extremely diverse, and survivors who consult psychiatrists require holistic assessment and treatment tailored to multifarious needs. PMID:8699497

  19. Increased Brain Perfusion Persists over the First Month of Life in Term Asphyxiated Newborns Treated with Hypothermia: Does it Reflect Activated Angiogenesis?

    PubMed

    Shaikh, Henna; Lechpammer, Mirna; Jensen, Frances E; Warfield, Simon K; Hansen, Anne H; Kosaras, Bela; Shevell, Michael; Wintermark, Pia

    2015-06-01

    Many asphyxiated newborns still develop brain injury despite hypothermia therapy. The development of brain injury in these newborns has been related partly to brain perfusion abnormalities. The purposes of this study were to assess brain hyperperfusion over the first month of life in term asphyxiated newborns and to search for some histopathological clues indicating whether this hyperperfusion may be related to activated angiogenesis following asphyxia. In this prospective cohort study, regional cerebral blood flow was measured in term asphyxiated newborns treated with hypothermia around day 10 of life and around 1 month of life using magnetic resonance imaging (MRI) and arterial spin labeling. A total of 32 MRI scans were obtained from 24 term newborns. Asphyxiated newborns treated with hypothermia displayed an increased cerebral blood flow in the injured brain areas around day 10 of life and up to 1 month of life. In addition, we looked at the histopathological clues in a human asphyxiated newborn and in a rat model of neonatal encephalopathy. Vascular endothelial growth factor (VEGF) was expressed in the injured brain of an asphyxiated newborn treated with hypothermia in the first days of life and of rat pups 24-48 h after the hypoxic-ischemic event, and the endothelial cell count increased in the injured cortex of the pups 7 and 11 days after hypoxia-ischemia. Our data showed that the hyperperfusion measured by imaging persisted in the injured areas up to 1 month of life and that angiogenesis was activated in the injured brain of asphyxiated newborns. PMID:25620793

  20. Early biochemical indicators of hypoxic-ischemic encephalopathy after birth asphyxia.

    PubMed

    Nagdyman, N; Kömen, W; Ko, H K; Müller, C; Obladen, M

    2001-04-01

    Hypoxic-ischemic encephalopathy (HIE) after perinatal asphyxia is a condition in which serum concentrations of brain-specific biochemical markers may be elevated. Neuroprotective interventions in asphyxiated newborns require early indicators of brain damage to initiate therapy. We examined brain-specific creatine kinase (CK-BB), protein S-100, and neuron-specific enolase in cord blood and 2, 6, 12, and 24 h after birth in 29 asphyxiated and 20 control infants. At 2 h after birth, median (quartiles) serum CK-BB concentration was 10.0 U/L (6.0-13.0 U/L) in control infants, 16.0 U/L (13.0-23.5 U/L) in infants with no or mild HIE, and 46.5 U/L (21.4-83.0 U/L) in infants with moderate or severe HIE. Serum protein S-100 was 1.6 microg/L (1.4-2.5 microg/L) in control infants, 2.9 microg/L (1.8-4.7 microg/L) in asphyxiated infants with no or mild HIE, and 17.0 microg/L (3.2-34.1 microg/L) in infants with moderate or severe HIE 2 h after birth. No significant difference was detectable in serum neuron-specific enolase between infants with no or mild and moderate or severe HIE 2 and 6 h after birth. A combination of serum protein S-100 (cutoff value, 8.5 microg/L) and CK-BB (cutoff value, 18.8 U/L) 2 h after birth had the highest predictive value (83%) and specificity (95%) of predicting moderate and severe HIE. Cord blood pH (cutoff value, <6.9) and cord blood base deficit (cutoff value, >17 mM) increase the predictive values of protein S-100 and CK-BB. We conclude that elevated serum concentrations of protein S-100 and CK-BB reliably indicate moderate and severe HIE as early as 2 h after birth.

  1. Toxic Encephalopathy

    PubMed Central

    Kim, Jae Woo

    2012-01-01

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

  2. Perinatal depression

    PubMed Central

    Alhusen, Jeanne L.; Alvarez, Carmen

    2016-01-01

    Abstract: Perinatal depression is a common condition with significant adverse maternal, fetal, neonatal, and early childhood outcomes. The perinatal period is an opportune time to screen, diagnose, and treat depression. Improved recognition of perinatal depression, particularly among low-income women, can lead to improved perinatal health outcomes. PMID:26934457

  3. Safety and efficacy of topiramate in neonates with hypoxic ischemic encephalopathy treated with hypothermia (NeoNATI)

    PubMed Central

    2012-01-01

    Background Despite progresses in neonatal care, the mortality and the incidence of neuro-motor disability after perinatal asphyxia have failed to show substantial improvements. In countries with a high level of perinatal care, the incidence of asphyxia responsible for moderate or severe encephalopathy is still 2–3 per 1000 term newborns. Recent trials have demonstrated that moderate hypothermia, started within 6 hours after birth and protracted for 72 hours, can significantly improve survival and reduce neurologic impairment in neonates with hypoxic-ischemic encephalopathy. It is not currently known whether neuroprotective drugs can further improve the beneficial effects of hypothermia. Topiramate has been proven to reduce brain injury in animal models of neonatal hypoxic ischemic encephalopathy. However, the association of mild hypothermia and topiramate treatment has never been studied in human newborns. The objective of this research project is to evaluate, through a multicenter randomized controlled trial, whether the efficacy of moderate hypothermia can be increased by concomitant topiramate treatment. Methods/Design Term newborns (gestational age ≥ 36 weeks and birth weight ≥ 1800 g) with precocious metabolic, clinical and electroencephalographic (EEG) signs of hypoxic-ischemic encephalopathy will be randomized, according to their EEG pattern, to receive topiramate added to standard treatment with moderate hypothermia or standard treatment alone. Topiramate will be administered at 10 mg/kg once a day for the first 3 days of life. Topiramate concentrations will be measured on serial dried blood spots. 64 participants will be recruited in the study. To evaluate the safety of topiramate administration, cardiac and respiratory parameters will be continuously monitored. Blood samplings will be performed to check renal, liver and metabolic balance. To evaluate the efficacy of topiramate, the neurologic outcome of enrolled newborns will be evaluated by serial

  4. Metabolic encephalopathies.

    PubMed

    Angel, Michael J; Young, G Bryan

    2011-11-01

    Kinnier Wilson coined the term metabolic encephalopathy to describe a clinical state of global cerebral dysfunction induced by systemic stress that can vary in clinical presentation from mild executive dysfunction to deep coma with decerebrate posturing; the causes are numerous. Some mechanisms by which cerebral dysfunction occurs in metabolic encephalopathies include focal or global cerebral edema, alterations in transmitter function, the accumulation of uncleared toxic metabolites, postcapillary venule vasogenic edema, and energy failure. This article focuses on common causes of metabolic encephalopathy, and reviews common causes, clinical presentations and, where relevant, management.

  5. Progressive multicystic encephalopathy: is there more than hypoxia-ischemia?

    PubMed

    Garten, Lars; Hueseman, Dieter; Stoltenburg-Didinger, Gisela; Felderhoff-Mueser, Ursula; Weizsaecker, Katharina; Scheer, Ianina; Boltshauser, Eugen; Obladen, Michael

    2007-05-01

    Progressive multicystic encephalopathy following prenatal or perinatal hypoxia-ischemia is a well-described phenomenon in the literature. The authors report on a term infant with a devastating encephalopathy and severe neuronal dysfunction immediately after delivery without a known antecedent of prenatal or perinatal hypoxia or distress. Clinical and paraclinical findings in the patient are compared with those described in the literature. The authors focus on the specific results guiding to the final diagnosis of progressive multicystic encephalopathy and the timing of morphologic changes. As in this case, if the criteria of an acute hypoxic event sufficient to cause neonatal encephalopathy are not met, then factors other than hypoxia-ischemia may be leading to progressive multicystic encephalopathy.

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

    PubMed

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

    2008-04-01

    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.

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

    PubMed

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

    2008-04-01

    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. PMID:17980531

  8. Hepatic encephalopathy.

    PubMed

    Córdoba, Juan; Mínguez, Beatriz

    2008-02-01

    Hepatic encephalopathy is a severe complication of cirrhosis that is related to the effects of ammonia. Analysis of interorgan ammonia trafficking has identified an important role of skeletal muscle in ammonia removal and has highlighted the importance of the nutritional status. Ammonia causes neurotransmitter abnormalities and induces injury to astrocytes that is partially mediated by oxidative stress. These disturbances lead to astrocyte swelling and brain edema, which appear to be involved in the pathogenesis of neurological manifestations. Inflammatory mediators worsen brain disturbances. New methods for assessing hepatic encephalopathy include clinical scales, neuropsychological tests, imaging of portal-systemic circulation, and magnetic resonance of the brain. Reappraisal of current therapy indicates the need for performing placebo-controlled trials and the lack of evidence for administering diets with restricted protein content. Liver transplant should be considered in selected patients with hepatic encephalopathy. Future prospects include new drugs that decrease plasma ammonia, measures to reduce brain edema, and liver-support devices. PMID:18293278

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

    PubMed Central

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

    2014-01-01

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

  10. PRACTICAL ASPECTS OF THERAPEUTIC HYPOTHERMIA IN NEONATES WITH HYPOXIC ISCHEMIC ENCEPHALOPATHY--QUESTIONS AND ANSWERS. PART I. PROVIDING NEWBORN CARE BEFORE AND DURING TRANSFER TO THE REFERENCE CENTER.

    PubMed

    Gulczyńska, Ewa; Gadzinowski, Janusz

    2015-01-01

    The first decade of the 21st century saw the worldwide spread of therapeutic hypothermia as a beneficial therapeutic procedure in neonates with hypoxic-ischemic encephalopathy. New guidelines for the resuscitation of newborns confirm that therapeutic hypothermia should be the standard method of treatment offered to neonates with acute perinatal hypoxia. The quality of care which an asphyxiated newborn receives during and immediately after resuscitation, as well as the mode of preparation for transport, can have a significant impact on improving the outcome, but it can also result in the deterioration of neonates treated with hypothermia. Since to a considerable degree the therapeutic effect depends on the time of beginning the cooling procedure, there is no reason to unnecessarily delay treatment. For this purpose, neonatologists or pediatricians from referring hospitals who do not have the equipment for hypothermia can and even should begin the cooling process while waiting for the arrival of the neonatal transport team. In that short period a number of concerns arise regarding the optimal methods of child care and preparation for transport to the hypothermia center. The authors discuss the possibility of initiating cooling before transportation using simple, so called low-tech cooling methods, the possible risks associated with the incidence of hyperthermia, difficulties in the interpretation of the eligibility criteria, supportive therapy, and the problems connected with the communication process between the medical team and the parents. The aspects that have been analyzed should be helpful for professionals in neonatal wards, outside hypothermia centers.

  11. PRACTICAL ASPECTS OF THERAPEUTIC HYPOTHERMIA IN NEONATES WITH HYPOXIC ISCHEMIC ENCEPHALOPATHY--QUESTIONS AND ANSWERS. PART I. PROVIDING NEWBORN CARE BEFORE AND DURING TRANSFER TO THE REFERENCE CENTER.

    PubMed

    Gulczyńska, Ewa; Gadzinowski, Janusz

    2015-01-01

    The first decade of the 21st century saw the worldwide spread of therapeutic hypothermia as a beneficial therapeutic procedure in neonates with hypoxic-ischemic encephalopathy. New guidelines for the resuscitation of newborns confirm that therapeutic hypothermia should be the standard method of treatment offered to neonates with acute perinatal hypoxia. The quality of care which an asphyxiated newborn receives during and immediately after resuscitation, as well as the mode of preparation for transport, can have a significant impact on improving the outcome, but it can also result in the deterioration of neonates treated with hypothermia. Since to a considerable degree the therapeutic effect depends on the time of beginning the cooling procedure, there is no reason to unnecessarily delay treatment. For this purpose, neonatologists or pediatricians from referring hospitals who do not have the equipment for hypothermia can and even should begin the cooling process while waiting for the arrival of the neonatal transport team. In that short period a number of concerns arise regarding the optimal methods of child care and preparation for transport to the hypothermia center. The authors discuss the possibility of initiating cooling before transportation using simple, so called low-tech cooling methods, the possible risks associated with the incidence of hyperthermia, difficulties in the interpretation of the eligibility criteria, supportive therapy, and the problems connected with the communication process between the medical team and the parents. The aspects that have been analyzed should be helpful for professionals in neonatal wards, outside hypothermia centers. PMID:26958686

  12. Stridor in asphyxiated neonates undergoing therapeutic hypothermia.

    PubMed

    Orme, Judith; Kissack, Christopher; Becher, Julie-Clare

    2014-07-01

    Therapeutic hypothermia is an established standard of care in the treatment of hypoxic-ischemic encephalopathy. Application of therapeutic hypothermia in the clinical setting may reveal a wider spectrum of adverse events than previously reported. We report 5 cases of transient respiratory stridor in 51 infants, occurring at different time points in the cooling process, which appeared to be unrelated to the intubation procedure. Therapeutic hypothermia was associated with transient stridor in this case series. Formal laryngoscopy is required to determine the underlying pathologic etiology.

  13. [Hashimoto encephalopathy].

    PubMed

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

    2013-08-18

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

  14. Perinatal neuroprotection.

    PubMed

    Salmeen, Kirsten E; Jelin, Angie C; Thiet, Mari-Paule

    2014-01-01

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

  15. Dengue encephalopathy.

    PubMed

    Hendarto, S K; Hadinegoro, S R

    1992-06-01

    Dengue encephalopathy or dengue hemorrhagic fever (DHF) with CNS involvement used to be considered a relatively rare condition; but the number of cases reported in human studies has been increasing every year. Diagnosis of dengue encephalopathy is based on clinically diagnosed DHF according to the W.H.O. criteria (1980), with CNS manifestations consisting of abrupt onset of hyperpyrexia, non-transient alteration of consciousness, headache, vomiting--with or without seizures--and normal CSF. Many factors may be considered to be directly or indirectly associated with CNS signs and symptoms in DHF, the main pathology being leakage of plasma into serous spaces and abnormal hemostasis, leading to hypovolemic shock and hemorrhage in many organs of the body. Acute liver failure is considered to be one of the main factors causing brain pathology. One hundred fifty-two cases of dengue encephalopathy admitted during 3 periods at the Cipto Mangunkusumo Hospital in Jakarta were studied retrospectively. The overall incidence was 152 out of 2,441 DHF cases, or 6.2%. The most pronounced symptoms were hyperpyrexia, alteration of consciousness and convulsions. Laboratory examination showed an unusually high increase of serum transaminases, hyponatremia, and hypoxia. Neurologic abnormalities detected were hemiparesis and tetraparesis of the extremities, and second nerve atrophy; such abnormalities were found in 10 out of the 152 cases, or 6.5%.

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

    PubMed

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

    2016-01-01

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

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

    PubMed

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

    2016-01-01

    Hypoxic-ischaemic brain injury involves increased oxidative stress. In asphyxiated newborns iron deposited in the brain catalyses formation of reactive oxygen species. Glutathione (GSH) and vitamin E are key factors protecting cells against such agents. Our previous investigation has demonstrated that newborn rats, showing physiological low body temperature as well as their hyperthermic counterparts injected with deferoxamine (DF) are protected against iron-mediated, delayed neurotoxicity of perinatal asphyxia. Therefore, we decided to study the effects of body temperature and DF on the antioxidant status of the brain in rats exposed neonatally to critical anoxia. Two-day-old newborn rats were exposed to anoxia in 100% nitrogen atmosphere for 10 min. Rectal temperature was kept at 33 °C (physiological to rat neonates), or elevated to the level typical of healthy adult rats (37 °C), or of febrile adult rats (39 °C). Half of the rats exposed to anoxia under extremely hyperthermic conditions (39 °C) were injected with DF. Cerebral concentrations of malondialdehyde (MDA, lipid peroxidation marker) and the levels of GSH and vitamin E were determined post-mortem, (1) immediately after anoxia, (2) 3 days, (3) 7 days, and (4) 2 weeks after anoxia. There were no post-anoxic changes in MDA, GSH and vitamin E concentrations in newborn rats kept at body temperature of 33 °C. In contrast, perinatal anoxia at elevated body temperatures intensified oxidative stress and depleted the antioxidant pool in a temperature-dependent manner. Both the depletion of antioxidants and lipid peroxidation were prevented by post-anoxic DF injection. The data support the idea that hyperthermia may extend perinatal anoxia-induced brain lesions. PMID:26794834

  18. [Perinatal listeriosis].

    PubMed

    Tollan, A; Sundsfjord, A; Lindal, S

    1992-04-30

    Human listeriosis is a rare disease. It may be foodborne. Listeric infection during pregnancy may give a fatal fetal outcome, caused by transplacental passage of organisms from the maternal gastrointestinal tract. We describe a case of perinatal listeriosis which resulted in preterm stillbirth. Perinatal listeriosis should be considered when flue-like symptoms are presented during pregnancy. Early diagnosis and treatment may improve the outcome.

  19. Hashimoto's encephalopathy.

    PubMed

    Schiess, Nicoline; Pardo, Carlos A

    2008-10-01

    Hashimoto's encephalopathy (HE) is a controversial neurological disorder that comprises a heterogenous group of neurological symptoms that manifest in patients with high titers of antithyroid antibodies. Clinical manifestations of HE may include encephalopathic features such as seizures, behavioral and psychiatric manifestations, movement disorders, and coma. Although it has been linked to cases of Hashimoto's thyroiditis or thyroid dysfunction, the most common immunological feature of HE is the presence of high titers of antithyroglobulin or anti-TPO (antimicrosomal) antibodies. At present, it is unclear whether antithyroid antibodies represent an immune epiphenomenon in a subset of patients with encephalopathic processes or they are really associated with pathogenic mechanisms of the disorder. The significance of classifying encephalopathies under the term HE will be determined in the future once the relevance of the role of antithyroid antibodies is demonstrated or dismissed by more detailed experimental and immunopathological studies. The responsiveness of HE to steroids or other therapies such as plasmapheresis supports the hypothesis that this is a disorder that involves immune pathogenic mechanisms. Further controlled studies of the use of steroids, plasmapheresis, or immunosuppressant medications are needed in the future to prove the concept of the pathogenic role of antithyroid antibodies in HE. PMID:18990131

  20. Autoimmune encephalopathies

    PubMed Central

    Leypoldt, Frank; Armangue, Thaís; Dalmau, Josep

    2014-01-01

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

  1. [Hepatic encephalopathy].

    PubMed

    Festi, Davide; Marasco, Giovanni; Ravaioli, Federico; Colecchia, Antonio

    2016-07-01

    Hepatic encephalopathy (HE) is a common complication of liver cirrhosis and it can manifest with a broad spectrum of neuropsychiatric abnormalities of varying severity, acuity and time course with important clinical implications. According to recent guidelines, HE has been classified into different types, depending on the severity of hepatic dysfunction, the presence of porto-systemic shunts and the number of previous episodes or persistent manifestations. From a clinical point of view, HE can be recognized as unimpaired, covert (that deals with minimal and grade 1 according to the grading of mental state), and overt (that is categorized from grade 2 to grade 4). Different and only partially known pathogenic mechanisms have been identified, comprising ammonia, inflammatory cytokines, benzodiazepine-like compounds and manganese deposition. Different therapeutic strategies are available for treating HE, in particular the overt HE, since covert HE needs to be managed case by case. Recognition and treatment of precipitating factors represent fundamental part of the management. The more effective treatments, which can be performed separately or combined, are represented by non-absorbable disaccharides (lactulose and lactitol) and the topic antibiotic rifaximin; other possible therapies, mainly used in patients non responders to previous treatments, are represented by branched chain amino acids and metabolic ammonia scavengers. PMID:27571468

  2. Who should we cool after perinatal asphyxia?

    PubMed

    Thoresen, Marianne

    2015-04-01

    Three ongoing challenges have arisen after the introduction of therapeutic hypothermia (TH) as standard of care for term newborns with moderate or severe perinatal asphyxia: (i) to ensure that the correct group of infants are cooled; (ii) to optimize the delivery of TH and intensive care in relation to the severity of the encephalopathy; (iii) to systematically follow up the long-term efficacy of TH using comparable outcome data between centers and countries. This review addresses the entry criteria for TH, and discusses potential issues regarding patient selection, and management of TH: cooling mild, moderate, and very severe perinatal asphyxia, cooling longer or deeper, and/or starting with a greater delay. This includes cooling of patients outside of standard trial entry criteria, such as after postnatal collapse, premature infants, those with infection, and infants with metabolic, chromosomal or surgical diagnoses in addition to perinatal asphyxia. PMID:25667126

  3. Pathogenesis of Hepatic Encephalopathy

    PubMed Central

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

    2012-01-01

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

  4. Speckle tracking derived strain in infants with severe perinatal asphyxia: a comparative case control study

    PubMed Central

    2013-01-01

    Background Speckle tracking echocardiography is increasingly being used to assess cardiac function in neonates. The objective was to compare speckle tracking strain indices between asphyxiated infants and healthy controls and to ascertain correlations between strain and 2D Doppler derived indices and cardiac troponin (biochemical marker of myocardial injury). Methods Clinical and echocardiographic data from severely asphyxiated infants undergoing therapeutic hypothermia was evaluated retrospectively. This was compared with prospective data from healthy infants. Correlations between variables were assessed using Pearson’s coefficient of correlation. Results Twenty four infants with severe perinatal asphyxia were admitted during the study period of which 3 were not cooled and were excluded. The gestational age and birth weights of cases and controls were comparable. The mean left ventricular global longitudinal strain (GLS) from apical 4 chamber view was noted to be significantly impaired in the asphyxiated infants (– 11.01% ± 2.48 vs – 21.45% ± 2.74, p <0.001). Cardiac output was significantly lower in the asphyxiated infants (97 ± 26 vs 230 ± 60 ml/kg/min). In asphyxiated infants, GLS correlated positively with cardiac output (r2 = 0.86, p< 0.001) and negatively with serum troponin levels (r2 = 0.64, p< 0.001). GLS was less impaired in infants on inotropes compared to those not on inotropic support, -12.55% (1.9) vs -10.2% (1.3), p= 0.018. Infants who died had a lower global strain value compared to survivors, – 9.7% (1.6) vs – 12.8% (2.6), p = 0.02. Conclusions 2D Speckle derived strain was impaired in asphyxiated infants. Significant correlations between GLS and cardiac output and troponin were noted. PMID:24229323

  5. Difference in molecular pathology of natriuretic peptides in the myocardium between acute asphyxial and cardiac deaths.

    PubMed

    Chen, Jian-Hua; Michiue, Tomomi; Ishikawa, Takaki; Maeda, Hitoshi

    2012-07-01

    In investigating death due to mechanical asphyxiation and drowning, a cardiac attack is important for discriminating between possible causes of death and as a contributory factor in death processes; however, general pathologies involving visceral congestion are often similar. The present study compared terminal cardiac dysfunction in these fatalities using the molecular pathology of atrial and brain natriuretic peptides (ANP and BNP) in the myocardium as markers of cardiac strain. Both mechanical asphyxiation (n=27) and drowning (n=23) showed significantly lower ANP and BNP mRNA expressions in bilateral ventricular walls than sudden cardiac deaths (n=36). In addition, right atrial wall BNP mRNA expression was lower in asphyxiation; however, immunostaining did not demonstrate any difference among these fatalities. Differences among the subtypes of asphyxiation or between fresh- and saltwater drowning were insignificant. These observations suggest a difference between primary heart failure in sudden cardiac death and terminal cardiac dysfunction secondary to fatal asphyxiation or drowning.

  6. The Scottish perinatal neuropathology study: clinicopathological correlation in early neonatal deaths

    PubMed Central

    Becher, J; Bell, J; Keeling, J; McIntosh, N; Wyatt, B

    2004-01-01

    Background: A proportion of neonatal deaths from asphyxia have been shown to be associated with pre-existing brain injury. Objectives: (a) To compare the epidemiology of infants displaying signs of birth asphyxia with those not showing signs; (b) to examine the neuropathology and determine if possible the timing of brain insult comparing asphyxiated with non-asphyxiated infants; (c) to compare the clinical features of those born with birth asphyxia with and without pre-labour damage. Methods: Over a two year period, all 22 Scottish delivery units collected clinical details on early neonatal deaths. Requests for post mortem included separate requests for detailed neuropathological examination of the brain. Infants were classified into two groups: birth asphyxia and non-birth asphyxia. Clinicopathological correlation was used to attempt to define the time of brain insult. Results: Detailed clinical data were available on 137 of 174 early neonatal deaths that met the inclusion criteria. Seventy of 88 parents who had agreed to post mortem examination consented to a detailed examination of additional samples from the brain; in 53 of these cases the infant was born in an asphyxiated condition. All asphyxiated and encephalopathic infants, 38% of mature and 52% of preterm infants with features of birth asphyxia but without encephalopathy, and only one of 12 infants without any signs of birth asphyxia showed damage consistent with onset before the start of labour. Conclusions: In a large proportion of neonatal deaths, brain injury predates the onset of labour. This is more common in infants born in an asphyxiated condition. PMID:15321957

  7. Bovine Spongiform Encephalopathy

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  8. Epileptic Encephalopathies: An Overview

    PubMed Central

    Khan, Sonia; Al Baradie, Raidah

    2012-01-01

    Epileptic encephalopathies are an epileptic condition characterized by epileptiform abnormalities associated with progressive cerebral dysfunction. In the classification of the International League Against Epilepsy eight age-related epileptic encephalopathy syndromes are recognized. These syndromes include early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. Other epileptic syndromes such as migrating partial seizures in infancy and severe epilepsy with multiple independent spike foci may be reasonably added. In this paper, we provide an overview of epileptic encephalopathies including clinical neurophysiological features, cognitive deterioration, and management options especially that these conditions are generally refractory to standard antiepileptic drugs. PMID:23213494

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

    PubMed Central

    Suruda, A; Agnew, J

    1989-01-01

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

  10. Perinatal Asphyxia from the Obstetric Standpoint: Diagnosis and Interventions.

    PubMed

    Herrera, Christina A; Silver, Robert M

    2016-09-01

    Perinatal asphyxia is a general term referring to neonatal encephalopathy related to events during birth. Asphyxia refers to a deprivation of oxygen for a duration sufficient to cause neurologic injury. Most cases of perinatal asphyxia are not necessarily caused by intrapartum events but rather associated with underlying chronic maternal or fetal conditions. Of intrapartum causes, obstetric emergencies are the most common and are not always preventable. Screening high-risk pregnancies with ultrasound, Doppler velocimetry, and antenatal testing can aid in identifying fetuses at risk. Interventions such as intrauterine resuscitation or operative delivery may decrease the risk of severe hypoxia from intrauterine insults and improve long-term neurologic outcomes. PMID:27524445

  11. Discharge planning and follow-up care: the asphyxiated infant.

    PubMed

    Parker, L

    1991-01-01

    Discharge planning and follow-up care of the asphyxiated infant is a complex process. Models of discharge planning, team member responsibilities, and teaching responsibilities are components of hospital discharge plans. Special care needs of these infants may include vision, hearing, immunizations, seizures, medications, and feeding. Families and health care professionals need to be familiar with programs providing financial resources for care of the infant such as private insurance, prepaid health care, Medicaid, Medical Needy program, Children with Special Health Care Needs (CSHCN), federal legislation mandating education and services for high-risk infants (PL 99-142 and PL 99-457) and intervention programs. Families returning to Newborn Follow-up programs become acquainted with a variety of professionals and types of neonatal and infant assessments. Providing teaching materials and information regarding special health problems, services and outcome, as it becomes known, is the responsibility of the extended health care team of nurses, physicians, home health services, psychologists, and therapists.

  12. Hepatic encephalopathy: a review.

    PubMed

    Lizardi-Cervera, Javier; Almeda, Paloma; Guevara, Luis; Uribe, Misael

    2003-01-01

    Hepatic encephalopathy (HE) is a complication that presents in as many as 28% of patients with cirrhosis, and reported up to ten years after the diagnosis of cirrhosis. Commonly, it is observed in patients with severe hepatic failure and is characterized by neuropsychiatric manifestations that can range in severity from a mild alteration in mental state to a coma; additionally, some neuromuscular symptoms can be observed. This complication of either acute or chronic hepatic disease is the result of a diminished hepatic reservoir and inability to detoxify some toxins that originate in the bowel. Today, the role of astrocytes, specifically the Alzheimer type II cells, is known to be very important in the pathogenesis of the hepatic encephalopathy, and will be reviewed later. In conclusion, the objectives of this review are: To understand the pathogenesis of hepatic encephalopathy, To recognize the precipitating factors, as well as preventive measures for the development of the hepatic encephalopathy, To describe the new classification of hepatic encephalopathy and its clinical implications, To recognize the clinical manifestations and stages of the disease, To understand the main diagnostic tests used to detect the hepatic encephalopathy, To describe the main therapeutic treatments of hepatic encephalopathy. PMID:15115963

  13. Portal systemic encephalopathy.

    PubMed

    Schenker, S; Bay, M K

    1997-05-01

    The goal of this article is to update the status of Portal systemic encephalopathy (PSE) in the light of new data. First, PSE is the context of other types of hepatic encephalopathy. Subsequently, current views of the pathogenesis of the disorder are discussed, followed by an analysis of therapeutic options. Diagnosis will not be considered, as no major new developments have recently been documented in this area.

  14. Perinatal rights.

    PubMed

    Munir, A E

    1984-01-01

    The history of perinatal rights is traced to determine how far the law has settled with reasonable certainty and principles can be drawn from decided cases, where the law remains uncertain. It is unlikely that there will be legislation in the near future to bring the law up to date in these matters. The right to prevent conception is accepted these day by practically all shades of opinion. Opinions on methods may differ, but the dividing line between what is contraception and what amounts to abortion is sometimes difficult to determine. The object of the offense of abortion is to protect human life. Briefly, Section 58 of the British Offences Against the Person Act 1861 makes it an offense for a pregnant woman to try unlawfully to procure her own miscarriage and for any person to try to procure unlawfully the miscarriage of a woman, whether she is pregnant or not. The precise time from which the developing ovum is protected has not been legislatively or judicially determined. In 1962 a report commissioned by the British Council of Churches suggested that for legal purposes conception should be taken to commence with implantation, i.e., about 2 weeks after fertilization. It is possible to argue that human life begins at fertilization but that is not a very convincing arugument these days. A better view seens to be that so long as the postcoital pill is taken before the fertilized egg is implanted in the womb it is contraception rather than abortion. The matter will not be totally free from question until Parliament of the courts determine the issue. The Attorney General's view that this form of postcoital treatment does not constitute a criminal offenses within either Section 58 or 59 of the Offences Against the Person Act 1860 goes a long way towards clarifying the position. Opinions begin to divide again when considering the next step after conception. Regarding abortion, the doctor should ensure that be keeps within the Abortion Act 1967 by acting with a

  15. The encephalopathy of sepsis.

    PubMed

    Jackson, A C; Gilbert, J J; Young, G B; Bolton, C F

    1985-11-01

    Twelve fatal cases of encephalopathy associated with sepsis were examined in a ten-year retrospective study. The sources of infection and organisms isolated were variable. Six of the patients had focal neurologic signs; five had seizures. The level of consciousness varied from drowsiness to deep coma, and electroencephalograms revealed diffuse or multifocal abnormalities. Computed tomographic head scans and cerebrospinal fluid examinations were usually unremarkable. Eight patients had disseminated microabscesses in the brain at autopsy. Four patients had proliferation of astrocytes and microglia in the cerebral cortex, a feature associated with metabolic encephalopathies. Additional findings included cerebral infarcts, brain purpura, multiple small white matter hemorrhages, and central pontine myelinolysis. Although sepsis may cause encephalopathy by producing disturbances in cerebral synaptic transmission and cerebral energy production through a toxic mechanism, bacterial invasion of the brain with the formation of disseminated microabscesses is also an important cause.

  16. [Hashimoto's encephalopathy and autoantibodies].

    PubMed

    Yoneda, Makoto

    2013-04-01

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

  17. Immunosuppressive measles encephalopathy.

    PubMed

    Pedersen, F K; Schiøtz, P O; Valerius, N H; Hertz, H

    1978-01-01

    A case of measles infection without a rash, which was followed by a severe encephalopathy after two months, is described in a 2 1/2 year old boy. At the age of 8 months he had been irradiated for an inoperable intrathoracic neuroblastoma, and at the time of exposure to measles he was being treated with cyclophosphamide and vincristine. This case closely resembles other cases recently described and termed immunosuppressive measles encephalopathy. The syndrome is believed to represent the effect of measles virus in patients with deficient cellular immunity induced by antineoplastic treatment. The importance of protecting children on immunosuppressive treatment for contracting measles is stressed.

  18. Management of Covert Hepatic Encephalopathy

    PubMed Central

    Waghray, Abhijeet; Waghray, Nisheet; Mullen, Kevin

    2015-01-01

    Hepatic encephalopathy is a reversible progressive neuropsychiatric disorder that encompasses a wide clinical spectrum. Covert hepatic encephalopathy is defined as patients with minimal hepatic encephalopathy and Grade I encephalopathy by West-Haven Criteria. Terminology such as “sub-clinical”, “latent”, and “minimal” appear to trivialize the disease and have been replaced by the term covert. The lack of clinical signs means that covert hepatic encephalopathy is rarely recognized or treated outside of clinical trials with options for therapy based on patients with episodic hepatic encephalopathy. This review discusses the current available options for therapy in covert hepatic encephalopathy and focuses on non-absorbable disacharides (lactulose or lactitol), antibiotics (rifaximin), probiotics/synbiotics and l-ornithine-l-aspartate. PMID:26041963

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

    PubMed Central

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

    2016-01-01

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

  20. Long-Term Cognitive Outcomes of Birth Asphyxia and the Contribution of Identified Perinatal Asphyxia to Cerebral Palsy.

    PubMed

    Pappas, Athina; Korzeniewski, Steven J

    2016-09-01

    Neonatal encephalopathy among survivors of presumed perinatal asphyxia is recognized as an important cause of cerebral palsy (CP) and neuromotor impairment. Recent studies suggest that moderate to severe neonatal encephalopathy contributes to a wide range of neurodevelopmental and cognitive impairments among survivors with and without CP. Nearly 1 of every 4 to 5 neonates treated with hypothermia has or develops CP. Neonatal encephalopathy is diagnosed in only approximately 10% of all cases. This article reviews the long-term cognitive outcomes of children with presumed birth asphyxia and describes what is known about its contribution to CP. PMID:27524454

  1. Detection of acute kidney injury in premature asphyxiated neonates by serum neutrophil gelatinase-associated lipocalin (sNGAL) – sensitivity and specificity of a potential new biomarker

    PubMed Central

    Pejović, Biljana; Erić-Marinković, Jelena; Pejović, Marija; Kotur-Stevuljević, Jelena; Peco-Antić, Amira

    2015-01-01

    Introduction Acute kidney injury (AKI) is common in neonatal intensive care units (NICU). In recent years, every effort is made for early detection of AKI. Our hypothesis was that serum neutrophil gelatinase-associated lipocalin (sNGAL) may be a reliable screening test for early diagnosis of AKI in premature neonates after perinatal asphyxia. Therefore, our aim was to assess the diagnostic accuracy of sNGAL for AKI in premature asphyxiated neonates. Materials and methods AKI was defined in the third day of life (DOL 3) as a serum creatinine (sCr) increase ≥ 26.5 μmol/L from baseline (the lowest previous sCr). According to the increase of sCr, AKI patients were divided in AKIN1 (sCr increase up to 1.9 baseline) and AKIN2 (sCr increase from 2.0 to 2.9 baseline). sNGAL levels were measured on DOL 1, 3 and 7. Results AKI was diagnosed in 73 (0.676) of 108 enrolled premature asphyxiated neonates. Sixty one patients (0.836) were classified in AKIN1 and 12 patients (0.164) in AKIN2. sNGAL reached the maximal concentrations on DOL 1 within 4 hours after admission to NICU, being higher in AKI compared with no-AKI group (160.8 ± 113.1 vs. 87.1 ± 81.6; P < 0.001) as well as in AKIN2 compared with AKIN1 group (222.8 ± 112.9 vs. 147.8 ± 109.9; P < 0.001). The best areas under the receiver operating characteristic curves (AUC) for prediction of AKI were 0.72 [95% (0.62-0.80) P < 0.001] on DOL1 at 2h and 0.72 [95% (0.63-0.80) P < 0.001] at 4th hour after admission respectively. The corresponding sNGAL cutoff concentrations were 84.87 ng/mL (sensitivity 69.0% and specificity 71.9%) and 89.43 ng/mL (sensitivity 65.7% and specificity 74.3%). Conclusions In premature asphyxiated neonates sNGAL measured within the first 4 hours of DOL 1 is predictive of the occurrence and severity of AKI. Therefore, plasma levels of NGAL may be used for early diagnosis of AKI in these patients. PMID:26525750

  2. [Bovine spongiform encephalopathy].

    PubMed

    Suárez Fernández, G

    2001-01-01

    An histórical and conceptual review is made about Bovine Spongiform Encephalopathy or mad cows disease and an epidemiological analysis as a present and future health problem. This analysis of BSE should not be negative, considering the truths that we know today. PMID:11783042

  3. KCNQ2 encephalopathy

    PubMed Central

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

    2016-01-01

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

  4. KCNQ2 encephalopathy

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2012-01-01

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

  6. Perinatal depression: A clinical update.

    PubMed

    Alhusen, Jeanne L; Alvarez, Carmen

    2016-05-19

    Perinatal depression is a common condition with significant adverse maternal, fetal, neonatal, and early childhood outcomes. The perinatal period is an opportune time to screen, diagnose, and treat depression. Improved recognition of perinatal depression, particularly among low-income women, can lead to improved perinatal health outcomes. PMID:26934457

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

    ERIC Educational Resources Information Center

    Samuels, R.; Chadwick, D. D.

    2006-01-01

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

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

    PubMed Central

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

    2013-01-01

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

  9. Pharmacological Neuroprotection after Perinatal Hypoxic-Ischemic Brain Injury

    PubMed Central

    Fan, Xiyong; Kavelaars, Annemieke; Heijnen, Cobi J; Groenendaal, Floris; van Bel, Frank

    2010-01-01

    Perinatal hypoxia-ischemia (HI) is an important cause of neonatal brain injury. Recent progress in the search for neuroprotective compounds has provided us with several promising drugs to reduce perinatal HI-induced brain injury. In the early stage (first 6 hours after birth) therapies are concentrated on prevention of the production of reactive oxygen species or free radicals (xanthine-oxidase-, nitric oxide synthase-, and prostaglandin inhibition), anti-inflammatory effects (erythropoietin, melatonin, Xenon) and anti-apoptotic interventions (nuclear factor kappa B- and c-jun N-terminal kinase inhibition); in a later stage stimulation of neurotrophic properties in the neonatal brain (erythropoietin, growth factors) can be targeted to promote neuronal and oligodendrocyte regeneration. Combination of pharmacological means of treatment with moderate hypothermia, which is accepted now as a meaningful therapy, is probably the next step in clinical treatment to fight post-asphyxial brain damage. Further studies should be directed at a more rational use of therapies by determining the optimal time and dose to inhibit the different potentially destructive molecular pathways or to enhance endogenous repair while at the same time avoiding adverse effects of the drugs used. PMID:21629441

  10. Pharmacoeconomics of hepatic encephalopathy.

    PubMed

    Neff, Guy

    2010-05-01

    Understanding and appreciating the science of pharmacoeconomics have become even more important for health care providers and insurers during the recent economic downturn. Evaluating the true costs of any disease is complex; both direct costs, such as costs of drug therapy and the provision of care, and indirect costs, such as lost earnings and reduced quality of life, must be taken into account. With chronic liver disease, the most recent data indicate that direct costs were more than $2 billion whereas indirect costs were more than $450 million. Hepatic encephalopathy, a common complication of chronic liver disease, contributes to this economic burden. Although patients' length of stay during hospitalization for hepatic encephalopathy decreased from almost 9 days to 6 days (and has remained stable over the past few years) from 1993 to 2007, hospitalization costs rose from $13,000 to $30,000/hospital stay. In addition, 22% of patients were discharged directly to nursing homes or rehabilitation centers, which increases total costs. When assessing therapy for hepatic encephalopathy, it is important to consider the total costs of the disease, not just treatment costs. Although more expensive on a daily basis than lactulose, rifaximin has been shown to reduce hospitalization rates, has a better adverse-effect profile, and increases patient compliance. One study found that rifaximin produced a cost savings/patient/year of more than $3000 over lactulose therapy. PMID:20412038

  11. Toxic encephalopathy induced by capecitabine.

    PubMed

    Niemann, B; Rochlitz, C; Herrmann, R; Pless, M

    2004-01-01

    Toxic encephalopathy is a rarely described side effect of 5-fluorouracil which usually presents with cerebellar, neuropsychiatric, and focal neurological symptoms. Magnetic resonance imaging findings are described as patchy white matter alterations. We report the 1st case of capecitabine-induced toxic encephalopathy with epilepsy-like symptoms and diffuse white matter alterations on magnetic resonance imaging.

  12. Outcome After Therapeutic Hypothermia in Term Neonates With Encephalopathy and a Syndromic Diagnosis.

    PubMed

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

    2015-10-01

    The large randomized, controlled trials of therapeutic hypothermia for hypoxic-ischemic encephalopathy excluded neonates with congenital disorders. The objective of this study was to report our experience using hypothermia in neonates with signs of hypoxic-ischemic encephalopathy 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, 8 (5%) had a syndromic disorder and were cooled per guidelines for nonsyndromic neonates. Perinatal characteristics of infants with and without syndromic disorder were not significantly different. Overall outcome was poor: 38% had evidence of acute hypoxic-ischemic injury, 3 subjects died, and 2 survivors had low developmental quotient (ie, 25). The risk versus benefit of therapeutic hypothermia for hypoxic-ischemic encephalopathy among neonates with congenital brain malformations or syndromic diagnoses is uncertain.

  13. Outcome After Therapeutic Hypothermia in Term Neonates With Encephalopathy and a Syndromic Diagnosis.

    PubMed

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

    2015-10-01

    The large randomized, controlled trials of therapeutic hypothermia for hypoxic-ischemic encephalopathy excluded neonates with congenital disorders. The objective of this study was to report our experience using hypothermia in neonates with signs of hypoxic-ischemic encephalopathy 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, 8 (5%) had a syndromic disorder and were cooled per guidelines for nonsyndromic neonates. Perinatal characteristics of infants with and without syndromic disorder were not significantly different. Overall outcome was poor: 38% had evidence of acute hypoxic-ischemic injury, 3 subjects died, and 2 survivors had low developmental quotient (ie, 25). The risk versus benefit of therapeutic hypothermia for hypoxic-ischemic encephalopathy among neonates with congenital brain malformations or syndromic diagnoses is uncertain. PMID:25762585

  14. Diets in Encephalopathy.

    PubMed

    Abdelsayed, George G

    2015-08-01

    As many as 80% of patients with end-stage liver disease and hepatic encephalopathy have significant protein-calorie malnutrition. Because of the severe hypercatabolic state of cirrhosis, the provision of liberal amounts of carbohydrate (at least 35 to 40 kcal/kg per day), and between 1.2 and 1.6 g/kg of protein is necessary. Protein restriction is not recommended. Branched-chain amino acid supplementation and vegetable protein are associated with improved outcomes. Dietary supplementation with vitamins, minerals (with the notable exception of zinc) and probiotics should be decided on a case-by-case basis. PMID:26195204

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

    PubMed

    Campbell, Margaret L

    2007-12-01

    The purpose of this exploratory study was to identify behaviors that may signify respiratory distress across cognitive states in response to an asphyxial threat. Patients undergoing a ventilator weaning trial were assessed and observed at baseline and during weaning with a capnograph/oximeter and video camera. Cognitive state was categorized at baseline, and an emotion report was elicited after the trial. Pulmonary stress and fear behaviors were similar across cognitive states. Hypercarbia predicted activation of fear behaviors. Gender differences characterized emotion reporting. An asphyxial threat may induce an innate array of behaviors that cannot be volitionally controlled and that may have the same appearance across cognitive states. Recognizing respiratory distress behaviors may improve nursing care of patients who are cognitively impaired. PMID:18022811

  16. Hypertension and hypertensive encephalopathy.

    PubMed

    Price, Raymond S; Kasner, Scott E

    2014-01-01

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

  17. [Value of the intravenous route for the use of phenobarbital in asphyxiated full term newborn infants].

    PubMed

    Gold, F; Bourin, M; Granry, J C; Amine, A; Breteau, M; Laugier, J

    1979-06-01

    Phenobarbital (20 mg/kg) was given intravenously to asphyxiated full term neonates who were less than 48 hours old. Further doses were given for 4 days (5 mgs/kg/d). Plasma levels were within the therapeutic range from 5 to 36 hours after the first injection but the maintenance dose always resulted in overdose by 5th day. The exact maintenance dose needs to be determined.

  18. Effect of vasopressin on hippocampal injury in a rodent model of asphyxial cardiopulmonary arrest

    PubMed Central

    ZHANG, NAN; ZANG, XIU-XIAN; DONG, NING; LIU, FANG; WANG, SHAO-KUN; YAN, HE; XU, DA-HAI; LIU, XIAO-LIANG; PANG, LI

    2016-01-01

    The effect of vasopressin on the neuronal injury following the restoration of spontaneous circulation (ROSC) in cardiac arrest (CA) is not yet fully understood. The present study was conducted in order to investigate the effect of vasopressin alone, or in combination with epinephrine, on the ROSC and hippocampal injury in a rat model of asphyxial CA. Asphyxial CA was induced in 144 rats by clamping the tracheal tube, and animals were allocated equally into the following three groups: Treatment with vasopressin (0.8 U/kg); epinephrine (0.2 mg/kg); or vasopressin (0.8 U/kg) plus epinephrine (0.2 mg/kg). An additional 48 rats underwent a sham surgical procedure without asphyxial CA and cardiopulmonary resuscitation. Hippocampal tissue was harvested at 1, 3, 6 and 12 h post-ROSC, and the levels of p38 mitogen-activated protein kinase (MAPK) and nuclear factor-κB (NF-κB) p65 were determined using immunohistochemistry. In comparison with rats treated with epinephrine alone, higher ROSC success rates were observed in rats treated with vasopressin, or vasopressin plus epinephrine. In addition, treatment with vasopressin attenuated hippocampal injury and reduced hippocampal p38 MAPK and NF-κB expression more efficiently compared with epinephrine alone. In conclusion, treatment with vasopressin exhibits a protective effect in patients experiencing CA, and this may be attributed to the inhibition of p38 MAPK and NF-κB expression. PMID:27073454

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

    NASA Astrophysics Data System (ADS)

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

    2016-05-01

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

  20. Hepatic encephalopathy: historical remarks.

    PubMed

    Amodio, Piero

    2015-03-01

    The history of hepatic encephalopathy (HE) is briefly reviewed since the beginning of western medicine by Hippocrates. For about 2000 years the main evidence was the mere association between jaundice, fever and delirium. A clear link between delirium and cirrhosis was proven in the 17th century by Morgagni. In subsequent times the focus was manly the descriptions of symptoms and the only pathophysiological improvement was the evidence that jaundice, per se, does not alter brain function. Only at the end of the 19th century Hann et al proved the role of portal-systemic shunt and pf nitrogenous derivates in the pathophysiology of the syndrome. A terrific development of knowledge occurred in the last 60 years, after the works of Sherlock in London. Nowadays some consensus about HE was reached, so that new developments will likely occur. PMID:26041956

  1. [Bromide encephalopathies (author's transl)].

    PubMed

    Loubrieu, G; Bourin, M; Bizière, K; Breteau, M; Autret, A

    1979-07-01

    Bromide encephalopathies are frequently reported in Northern America and Great Britain. There is no characteristic clinical pattern, the neurological symptoms are multiple, this is readily explained by the diffusion of bromide ions to all regions in the central nervous system. An accurate history (bromide intake, followed by the slow onset of digestive and neuropsychiatric symptoms) as well as an apparent hyperchloremia are of the greatest aid in suggesting the diagnosis. The incidence of this type of intoxication is greater in women over 50. The association of a salt free diet to bromide therapy favors the onset of clinical symptoms because of the competition between bromide and chloride at the choroid plexus and at the renal tubule.

  2. [Prevention of hepatic encephalopathy].

    PubMed

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

    2014-06-01

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

  3. Neurodevelopmental Follow Up After Therapeutic Hypothermia for Perinatal Asphyxia

    PubMed Central

    Zubcevic, Smail; Heljic, Suada; Catibusic, Feriha; Uzicanin, Sajra; Sadikovic, Mirna; Krdzalic, Belma

    2015-01-01

    Introduction: Neuroprotective benefit of therapeutic hypothermia in term newborns with hypoxic-ischemic encephalopathy (HIE) was assessed by analyzing survival and neurodevelopmental outcome of neonates subjected to this procedure. Material and methods: Newborns with gestational age > 36 weeks and < 6 hours of age with moderate to severe asphyxial encephalopathy underwent cooling protocol at a temperature of 33.5 °C for 72 hours and rewarming period of 6 hours. Outcome measures assessed were death and neurodevelopmental characteristics, which were compared at the different age using ASQ-3. Twenty-five children were assessed at age 3-6, 12-18 and 24-36 months. Median gestational age was 40 weeks, birth weight 3470 g, Apgar score 2/4 and pH on admission to the hospital 7.02. Four (16%) children died. Results: At the first assessment developmental categories of communication were normal in 78.9%, problem solving in 63.2%, personal-social in 68.4%, gross motor in 68.4%, and fine motor in 42.1% with a high need of retesting in this area. Second assessment was done in 17 patients: developmental categories of communication normal in 58.8%, problem solving in 70.6%, personal-social in 64.7%, gross motor in 64.7%, and fine motor in 35.3%. Third evaluation was done in 14 patients: developmental categories of communication were normal in 64.3%, problem solving in 71.4%, personal-social in 57.1%, gross motor in 64.3%, and fine motor in 42.9%. Conclusion: There was no correlation between baseline parameters and outcome. Results of the study are showing that therapeutic hypothermia in term newborns can provide better survival and less neurologic sequels in HIE patients. PMID:26843725

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

    PubMed

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

    2016-02-01

    Therapeutic hypothermia is well known for neuroprotection in asphyxiated neonates with hypoxic ischemic encephalopathy. The authors aimed to study the feasibility and safety of therapeutic hypothermia and short term outcome in neonates with hypoxic ischemic encephalopathy (HIE). Total 31 neonates with moderate to severe HIE were enrolled in the study. Continuous temperature recording was noted in 31 neonates; 17 neonates were studied prospectively while 14 neonates were studied retrospectively. Rectal temperature was monitored in 31 neonates and maintained between 33 and 34 °C by switching off the warmer and using ice packs. Reusable ice packs were used which were inexpensive. Therapeutic hypothermia was maintained for 72 h and babies were then rewarmed 0.5 °C every hour. Therapeutic hypothermia was feasible and inexpensive. There was no major complication during the study. MRI was done in 17 neonates; 52 % were found to have normal MRI at the end of first week. Among the study neonates (n = 31) 64.5 % were neurologically normal at the time of discharge. To conclude, therapeutic hypothermia is feasible in a low resource setting and is a safe way of neuroprotection. Short term outcome was also favourable in these neonates. PMID:26141549

  5. The role of inflammation in perinatal brain injury

    PubMed Central

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

    2015-01-01

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

  6. The role of inflammation in perinatal brain injury.

    PubMed

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

    2015-04-01

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

  7. The transmissible spongiform encephalopathies of livestock

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  8. Neonatal resuscitation adhering to oxygen saturation guidelines in asphyxiated lambs with meconium aspiration

    PubMed Central

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

    2016-01-01

    BACKGROUND The Neonatal Resuscitation Program (NRP) recommends upper and lower limits of preductal saturations (SpO2) extrapolated from studies in infants resuscitated in room air. These limits have not been validated in asphyxia and lung disease. METHODS Seven control term lambs delivered by cesarean section were ventilated with 21% O2. Thirty lambs with asphyxia with meconium aspiration were randomly assigned to resuscitation with 21% O2 (n = 6), 100% O2 (n = 6), or initiation with 21% O2 followed by variable FIO2 to maintain NRP target SpO2 ranges (n = 18). Hemodynamic and ventilation parameters were recorded for 15 min. RESULTS Control lambs maintained preductal SpO2 near the lower limit of NRP target range. Asphyxiated lambs had low SpO2 (38 ± 2%), low arterial pH (6.99 ± 0.01), and high PaCO2 (96 ± 7 mm Hg) at birth. Resuscitation with 21% O2 resulted in SpO2 values below the target range with low pulmonary blood flow (Qp) compared to variable FIO2 group. The increase in PaO2 and Qp with variable FIO2 resuscitation was similar to control lambs. CONCLUSION Maintaining SpO2 as recommended by NRP by actively adjusting inspired O2 leads to effective oxygenation and higher Qp in asphyxiated lambs with lung disease. Our findings support the current NRP SpO2 guidelines for O2 supplementation during resuscitation of an asphyxiated neonate. PMID:26672734

  9. Sepsis-Associated Encephalopathy

    PubMed Central

    Cotena, Simona; Piazza, Ornella

    2012-01-01

    Summary 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. PMID:23905041

  10. Chronic traumatic encephalopathy.

    PubMed

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

    2013-01-01

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

  11. [Mitochondrial neurogastrointestinal encephalopathy disease].

    PubMed

    Benureau, A; Meyer, P; Maillet, O; Leboucq, N; Legras, S; Jeziorski, E; Fournier-Favre, S; Jeandel, C; Gaignard, P; Slama, A; Rivier, F; Roubertie, A; Carneiro, M

    2014-12-01

    Mitochondrial neurogastrointestinal encephalopathy disease (MNGIE) is a rare autosomal-recessive syndrome, resulting from mutations in the TYMP gene, located at 22q13. The mutation induces a thymidine phosphorylase (TP) deficit, which leads to a nucleotide pool imbalance and to instability of the mitochondrial DNA. The clinical picture regroups gastrointestinal dysmotility, cachexia, ptosis, ophthalmoplegia, peripheral neuropathy, and asymptomatic leukoencephalopathy. The prognosis is unfavorable. We present the case of a 14-year-old Caucasian female whose symptoms started in early childhood. The diagnosis was suspected after magnetic resonance imaging (MRI), performed given the atypical features of mental anorexia, which revealed white matter abnormalities. She presented chronic vomiting, postprandial abdominal pain, and problems gaining weight accompanied by cachexia. This diagnosis led to establishing proper care, in particular an enteral and parenteral nutrition program. There is no known specific effective treatment, but numerous studies are in progress. In this article, after reviewing the existing studies, we discuss the main diagnostic and therapeutic aspects of the disease. We argue for the necessity of performing a cerebral MRI given the atypical features of a patient with suspected mental anorexia (or when the clinical pattern of a patient with mental anorexia seems atypical), so that MNGIE can be ruled out. PMID:25282463

  12. Sepsis-associated encephalopathy.

    PubMed

    Gofton, Teneille E; Young, G Bryan

    2012-10-01

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

  13. Sepsis Associated Encephalopathy.

    PubMed

    Chaudhry, Neera; Duggal, Ashish Kumar

    2014-01-01

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

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

    PubMed

    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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

  17. [Therapeutic hypothermia for neonatal hypoxic-ischemic encephalopathy].

    PubMed

    Gulczyńska, Ewa; Gadzinowski, Janusz

    2012-03-01

    Hypoxia-ischemia in the perinatal period is a serious condition affecting infants, which can result in death and cerebral palsy and associated disabilities. There has been significant research progress in hypoxic-ischemic encephalopathy over the last 2 decades. Many new molecular mechanisms of asphyxia have been identified. Despite all these advances, therapeutic interventions in HIE remain to be limited. Recently it has been revealed that mild therapeutic hypothermia is the only modality shown to improve neurologic outcome. The authors present a summary of pathogenesis of HIE, animal studies of cooling for hypoxic and ischemic models, and first publications on human therapeutic hypothermia trials. The diagnosis of encephalopathy in full-term neonates and enrollment criteria for hypothermia are also discussed. The current data from randomized control trials of hypothermia as neuroprotection for full and near-term infants are presented along with the results of meta-analyses of these trials. Finally the status of ongoing neonatal hypothermia trials as well as status of therapeutic hypothermia in Poland is summarized.

  18. Hashimoto's Encephalopathy Presenting with Chorea.

    PubMed

    Sharan, Abhijeet; Sengupta, Sarbani; Mukhopadhyay, Sarmishtha; Ghosh, Bhaskar

    2015-09-01

    Hashimoto's encephalopathy (HE) is a steroid-responsive relapsing neuropsychiatric disorder associated with high titers of antithyroid antibody with or without thyroid dysfunction. We report a case of HE in a 78 year old female who developed sudden onset behavioral abnormalities associated with choreiform movement of extremities. All causes of vascular, infective, metabolic, autoimmune, paraneoplastic and toxic encephalopathy were excluded. Anti-thyroid peroxidase (anti-TPO) antibody was found to be raised with very high titre. A diagnosis of HE was made. Prompt treatment with high dose steroid led to dramatic improvement of symptoms including choreiform movement. PMID:27608878

  19. Neuropsychological and educational problems at school age associated with neonatal encephalopathy

    PubMed Central

    Marlow, N; Rose, A; Rands, C; Draper, E

    2005-01-01

    Background: Adverse cognitive and educational outcomes are often ascribed to perinatal hypoxia without good evidence. Objective: To investigate neurocognitive and behavioural outcomes after neonatal encephalopathy. Methods: Sixty five children with neonatal encephalopathy, identified using the Trent Neonatal Survey database for 1992–1994, were followed up at the age of 7 years. They were examined at school, with a classmate for those in mainstream school, by a paediatrician and a psychologist. Neonatal encephalopathy was graded as moderate or severe using published definitions. Findings: Fifteen children had major disability, all with cerebral palsy; eight were in special school with severe cognitive impairment (IQ<55). Disability was present in 6% of the moderate and 42% of the severe encephalopathy group. Of the 50 children without motor disability, cognitive scores were lowest in the severe group (mean IQ difference from peers –11.3 points (95% confidence interval (CI) –19.0 to –3.6) and with similar scores for the moderate group compared with classmates (mean difference –1.7 points (95% CI –7.3 to +3.9). Neuropsychological testing showed similar findings in all domains. In particular, memory and attention/executive functions were impaired in the severe group. Despite relatively small differences in performance of the moderate group, special educational needs were identified more often in both encephalopathy groups, associated with lower achievement on national curriculum attainment targets. Interpretation: After neonatal encephalopathy, subtle cognitive impairments are found in the absence of neuromotor impairment. Subtle impairments are found more commonly after a more severe clinical course. Studies of brain protection strategies require long term follow up to study effects on cognitive outcome. PMID:16113154

  20. Preclinical Models of Encephalopathy of Prematurity.

    PubMed

    Jantzie, Lauren L; Robinson, Shenandoah

    2015-01-01

    Encephalopathy of prematurity (EoP) encompasses the central nervous system (CNS) abnormalities associated with injury from preterm birth. Although rapid progress is being made, limited understanding exists of how cellular and molecular CNS injury from early birth manifests as the myriad of neurological deficits in children who are born preterm. More importantly, this lack of direct insight into the pathogenesis of these deficits hinders both our ability to diagnose those infants who are at risk in real time and could potentially benefit from treatment and our ability to develop more effective interventions. Current barriers to clarifying the pathophysiology, developmental trajectory, injury timing, and evolution include preclinical animal models that only partially recapitulate the molecular, cellular, histological, and functional abnormalities observed in the mature CNS following EoP. Inflammation from hypoxic-ischemic and/or infectious injury induced in utero in lower mammals, or actual prenatal delivery of more phylogenetically advanced mammals, are likely to be the most clinically relevant EOP models, facilitating translation to benefit infants. Injury timing, type, severity, and pathophysiology need to be optimized to address the specific hypothesis being tested. Functional assays of the mature animal following perinatal injury to mimic EoP should ideally test for the array of neurological deficits commonly observed in preterm infants, including gait, seizure threshold and cognitive and behavioral abnormalities. Here, we review the merits of various preclinical models, identify gaps in knowledge that warrant further study and consider challenges that animal researchers may face in embarking on these studies. While no one model system is perfect, insights relevant to the clinical problem can be gained with interpretation of experimental results within the context of inherent limitations of the chosen model system. Collectively, optimal use of multiple models

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

    PubMed

    Dehaene, Isabelle; Roelens, Kristien; Page, Geert

    2014-09-29

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

  2. Postdatism -- a perinatal problem?

    PubMed

    Chhabra, S; Sood, S

    1990-01-01

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

  3. Maternal and perinatal mortality.

    PubMed

    Krishna Menon, M K

    1972-01-01

    A brief analysis of data from the records of the Government Hospital for Women and Children in Madras for a 36-year period (1929-1964) is presented. India with a population of over 550 million has only 1 doctor for each 6000 population. For the 80% of the population which is rural, the doctor ratio is only 88/1 million. There is also a shortage of paramedical personnel. During the earlier years of this study period, abortions, puerperal infections; hemorrhage, and toxemia accounted for nearly 75% of all meternal deaths, while in later years deaths from these causes were 40%. Among associated factors in maternal mortality, anemia was the most frequent, it still accounts for 20% and is a contributory factor in another 20%. The mortality from postpartum hemorrhage was 9.3% but has now decreased to 2.8%. Eclampsia is a preventable disease and a marked reduction in maternal and perinatal mortality from this cause has been achieved. Maternal deaths from puerperal infections have dropped from 25% of all maternal deaths to 7%. Uterine rupture has been reduced from 75% to 9.3% due to modern facilities. Operative deliveries still have an incidence of 2.1% and a mortality rate of 1.4% of all deliveries. These rates would be further reduced by more efficient antenatal and intranatal care. Reported perinatal mortality of infants has been reduced from 182/1000 births to an average of 78/1000 in all areas, but is 60.6/1000 in the city of Madras. Socioeconomic standards play an important role in perinatal mortality, 70% of such deaths occurring in the lowest economic groups. Improvement has been noted in the past 25 years but in rural areas little progress has been made. Prematurity and low birth weights are still larger factors in India than in other countries, with acute infectious diseases, anemia, and general malnutrition among mothers the frequent causes. Problems requiring further efforts to reduce maternal and infant mortality are correct vital statistics, improved

  4. Perinatal Grief in Latino Parents

    PubMed Central

    Whitaker, Claudia; Kavanaugh, Karen; Klima, Carrie

    2013-01-01

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

  5. Psychological treatments for perinatal depression.

    PubMed

    Stuart, Scott; Koleva, Hristina

    2014-01-01

    Perinatal depression is prevalent and greatly affects the mother and infant. Fortunately, empirically validated psychological treatments are available for postpartum depression and depression during pregnancy. Primary among these are interpersonal psychotherapy and cognitive-behavioural therapy, which have been shown to be effective for perinatal women across the spectrum from mild to severe depression. At present, interpersonal psychotherapy is better validated than antidepressant medication for perinatal depression, and should be considered as a first-line treatment option, especially for pregnant and breast-feeding women who are depressed. More studies are needed to evaluate further the relative efficacy of psychotherapy and medication, and more thoroughly test other psychological treatments. PMID:24269903

  6. Pharmacotherapy for hepatic encephalopathy.

    PubMed

    Phongsamran, Paula V; Kim, Jiwon W; Cupo Abbott, Jennifer; Rosenblatt, Angela

    2010-06-18

    Hepatic encephalopathy (HE) is a challenging clinical complication of liver dysfunction with a wide spectrum of neuropsychiatric abnormalities that range from mild disturbances in cognitive function and consciousness to coma and death. The pathogenesis of HE in cirrhosis is complex and multifactorial, but a key role is thought to be played by circulating gut-derived toxins of the nitrogenous compounds, most notably ammonia. Therapeutic treatment options for HE are currently limited and have appreciable risks and benefits associated with their use. Management of HE primarily involves avoidance of precipitating factors, limitation of dietary protein intake, and administration of various ammonia-lowering therapies such as non-absorbable disaccharides and select antimicrobial agents. Non-absorbable disaccharides, such as lactulose, have traditionally been regarded as first-line pharmacotherapy for patients with HE. However, multiple adverse events have been associated with their use. In addition, recent literature has questioned the true efficacy of the disaccharides for this indication. Neomycin, metronidazole and vancomycin may be used as alternative treatments for patients intolerant or unresponsive to non-absorbable disaccharides. Antimicrobials reduce bacterial production of ammonia and other bacteria-derived toxins through suppression of intestinal flora. Neomycin has been reported to be as effective as lactulose, and similar efficacy has been reported with vancomycin and metronidazole for the management of HE. However, the adverse effects frequently associated with these antimicrobials limit their use as first-line pharmacological agents. Neomycin is the most commonly used antimicrobial for HE and, although poorly absorbed, systemic exposure to the drug in sufficient amounts causes hearing loss and renal toxicity. Long-term neomycin therapy requires annual auditory testing and continuous monitoring of renal function. Long-term use of metronidazole has been

  7. Autoerotic asphyxial deaths: analysis of nineteen fatalities in Alberta, 1978 to 1989.

    PubMed

    Tough, S C; Butt, J C; Sanders, G L

    1994-04-01

    This paper presents an unusual form of sexual (masturbatory) activity and brings this unusual cause of death to wider medical attention and understanding. All 19 cases of autoerotic asphyxial death that occurred between 1978 and 1989 in the province of Alberta, Canada were reviewed. The fatal victim of autoerotic asphyxia is typically a single male aged 15 to 29 years. Autoerotic sexual activity is typically performed in isolation; often there is evidence of repetitive practice. The accidental death usually results when the "safety" mechanism designed to alleviate neck compression fails. Often the first sign of the activity (usually a surprise to family and friends) is death itself. Physicians who are alert to the practice may suggest counselling when patients present with sexual concerns, unusual marks around the neck or evidence of abrasions to limbs suggesting bondage or other masochistic practices. PMID:8033021

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

    PubMed

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

    2012-07-01

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

  9. Randomized trial of volume infusion during resuscitation of asphyxiated neonatal piglets.

    PubMed

    Wyckoff, Myra; Garcia, Damian; Margraf, Linda; Perlman, Jeffrey; Laptook, Abbot

    2007-04-01

    Despite its use, there is little evidence to support volume infusion (VI) during neonatal cardiopulmonary resuscitation (CPR). This study compares 5% albumin (ALB), normal saline (NS), and no VI (SHAM) on development of pulmonary edema and restoration of mean arterial pressure (MAP) during resuscitation of asphyxiated piglets. Mechanically ventilated swine (n=37, age: 8 +/- 4 d, weight: 2.2 +/- 0.7 kg) were progressively asphyxiated until pH <7.0, Paco2 >100 mm Hg, heart rate (HR) <100 bpm, and MAP <20 mm Hg. After 5 min of ventilatory resuscitation, piglets were randomized blindly to ALB, NS, or SHAM infusion. Animals were recovered for 2 h before euthanasia and lung tissue sampled for wet-to-dry weight ratio (W/D) as a marker of pulmonary edema. SHAM MAP was similar to VI during resuscitation. At 2 h post-resuscitation, MAP of SHAM (48 +/- 13 mm Hg) and ALB (43 +/- 19 mm Hg) was higher than NS (29 +/- 10 mm Hg; p=0.003 and 0.023, respectively). After resuscitation, SHAM piglets had less pulmonary edema (W/D: 5.84 +/- 0.12 versus 5.98 +/- 0.19; p=0.03) and better dynamic compliance (Cd) compared with ALB or NS (Cd: 1.43 +/- 0.69 versus 0.97 +/- 0.37 mL/cm H2O, p=0.018). VI during resuscitation did not improve MAP, and acute recovery of MAP was poorer with NS compared with ALB. VI was associated with increased pulmonary edema. In the absence of hypovolemia, VI during neonatal resuscitation is not beneficial.

  10. Asphyxiation Incidents by Hydrogen Sulfide at Manure Storage Facilities of Swine Livestock Farms in Korea.

    PubMed

    Park, Jihoon; Kang, Taesun; Jin, Suhyun; Heo, Yong; Kim, Kyungran; Lee, Kyungsuk; Tsai, Perngjy; Yoon, Chungsik

    2016-01-01

    Livestock workers are involved in a variety of tasks, such as caring for animals, maintaining the breeding facilities, cleaning, and manure handling, and are exposed to health and safety risks. Hydrogen sulfide is considered the most toxic by-product of the manure handling process at livestock facilities. Except for several reports in developed countries, the statistics and cause of asphyxiation incidents in farms have not been collected and reported systematically, although the number of these incidents is expected to increase in developing and underdeveloped countries. In this study, the authors compiled the cases of work-related asphyxiation incidents at livestock manure storage facilities and analyzed the main causes. In this survey, a total of 17 incidents were identified through newspapers or online searches and public reports. Thirty workers died and eight were injured due to work-related tasks and rescue attempts from 1998 to 2013 in Korea. Of the 30 fatalities, 18 occurred during manure handling/maintenance tasks and 12 during rescue attempts. All incidents except for one case occurred during the warm season from the late spring (April) to early autumn (September) when manure is likely to decompose rapidly. It is important to train employees involved in the operation of the facilities (i.e., owners, managers, employees) regarding the appropriate prevention strategies for confined space management, such as hazard identification before entry, periodical facility inspection, restriction of unnecessary access, proper ventilation, and health and safety. Sharing information or case reports on previous incidents could also help prevent similar cases from occurring and reduce the number of fatalities and injuries. PMID:26765950

  11. Countrywide analysis of perinatal outcome.

    PubMed

    Stembera, Z; Kravka, A; Mandys, F

    1988-01-01

    The computer laboratory of the Research Institute for the Care of Mother and Child in Prague performs annually a countrywide analysis of perinatal outcome in order to obtain a background for the preparation of the optimal strategy for improving perinatal care in CSR in the future. The total as well as weight specific perinatal mortality rate further sub-divided into early neonatal death rate and late fetal death rate and differentiated according to the birthweight, was correlated with the incidence of different factors influencing the perinatal mortality rate both countrywide and for each of the eight provinces of CSR. This way a correlation was found between some of the mentioned perinatal outcomes and e.g. instrumental equipment of obstetrical departments and neonatal intensive care units, frequency of caesarean sections, or transport of LBW newborns in incubators or "in utero" etc. The results of this analysis have proved that there still remain in some provinces opportunity for further decrease in perinatal mortality due to the incomplete observance of the two intervention strategies "Risk approach" and "New technology" which were introduced in the whole country during the last 10 years. PMID:3221298

  12. Preterm Hypoxic–Ischemic Encephalopathy

    PubMed Central

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

    2016-01-01

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

  13. [Wernicke encephalopathy accompanying linitis plastica].

    PubMed

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

    2014-01-01

    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. PMID:24379094

  14. Hepatic encephalopathy: diagnosis and treatment.

    PubMed

    Salgado, Melissa; Cortes, Yonaira

    2013-06-01

    Hepatic encephalopathy (HE) is a neurologic syndrome resulting from the synergistic action of multiple pathologic factors, which are discussed in a companion article. Early recognition of the clinical signs can improve treatment outcome, as well as reduce the incidence of risk factors. Multimodal treatment of HE is usually indicated. Studies on the pathogenesis and treatment of HE in people may shed new light on further treatment modalities in small animal patients. PMID:23677823

  15. Perinatal lethal osteogenesis imperfecta.

    PubMed Central

    Cole, W G; Dalgleish, R

    1995-01-01

    Perinatal lethal osteogenesis imperfecta is the result of heterozygous mutations of the COL1A1 and COL1A2 genes that encode the alpha 1(I) and alpha 2(I) chains of type I collagen, respectively. Point mutations resulting in the substitution of Gly residues in Gly-X-Y amino acid triplets of the triple helical domain of the alpha 1(I) or alpha 2(I) chains are the most frequent mutations. They interrupt the repetitive Gly-X-Y structure that is mandatory for the formation of a stable triple helix. Most babies have their own private de novo mutation. However, the recurrence rate is about 7% owing to germline mosaicism in one parent. The mutations act in a dominant negative manner as the mutant pro alpha chains are incorporated into type I procollagen molecules that also contain normal pro alpha chains. The abnormal molecules are poorly secreted, more susceptible to degradation, and impair the formation of the extracellular matrix. The collagen fibres are abnormally organised and mineralisation is impaired. The severity of the clinical phenotype appears to be related to the type of mutation, its location in the alpha chain, the surrounding amino acid sequences, and the level of expression of the mutant allele. Images PMID:7643358

  16. Preclinical Models of Encephalopathy of Prematurity

    PubMed Central

    Jantzie, Lauren L.; Robinson, Shenandoah

    2015-01-01

    Encephalopathy of prematurity (EoP) encompasses the central nervous system (CNS) abnormalities associated with injury from preterm birth. Although rapid progress is being made, limited understanding exists of how the cellular and molecular CNS injury from early birth manifests as the myriad of neurological deficits in children who are born preterm. More importantly, this lack of direct insight into the pathogenesis of these deficits hinders both our ability to diagnose those infants who are at risk in real-time and could potentially benefit from treatment, and our ability to develop more effective interventions. Current barriers to clarifying the pathophysiology, developmental trajectory, injury timing and evolution include preclinical animal models that only partially recapitulate the molecular, cellular, histological and functional abnormalities observed in the mature CNS following EoP. Inflammation from hypoxic-ischemic and/or infectious injury induced in utero in lower mammals, or actual prenatal delivery of more phylogenetically-advanced mammals, are likely to be the most clinically relevant EOP models, facilitating translation to benefit infants. Injury timing, type, severity and pathophysiology need to be optimized to address the specific hypothesis being tested. Functional assays of the mature animal following perinatal injury to mimic EoP should ideally test for the array of neurological deficits commonly observed in preterm infants including gait, seizure threshold, cognitive and behavioral abnormalities. Here, we review the merits of various preclinical models, identify gaps in knowledge that warrant further study and consider challenges that animal researchers may face in embarking on these studies. While no one model system is perfect, insights relevant to the clinical problem can be gained with interpretation of experimental results within the context of inherent limitations of the chosen model system. Collectively, optimal use of multiple models will

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

    PubMed

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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed

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

    2007-05-01

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

  20. [Perinatal sources of stem cells].

    PubMed

    Piskorska-Jasiulewicz, Magdalena Maria; Witkowska-Zimny, Małgorzata

    2015-03-08

    Recently, stem cell biology has become an interesting topic. Several varieties of human stem cells have been isolated and identified in vivo and in vitro. Successful application of hematopoietic stem cells in hematology has led to the search for other sources of stem cells and expanding the scale of their application. Perinatal stem cells are a versatile cell population, and they are interesting for both scientific and practical objectives. Stem cells from perinatal tissue may be particularly useful in the clinic for autologous transplantation for fetuses and newborns, and after banking in later stages of life, as well as for in utero transplantation in the case of genetic disorders. In this review paper we focus on the extraction and therapeutic potential of stem cells derived from perinatal tissues such as the placenta, the amnion, amniotic fluid, umbilical cord blood and Wharton's jelly.

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

    PubMed Central

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

    2016-01-01

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

  2. Cervical lung herniation complicating a case of acute asphyxial asthma in a child.

    PubMed

    Martchek, Melissa A; Padilla, Benjamin E; Zonfrillo, Mark R; Friedlaender, Eron Y

    2015-04-01

    The abrupt onset of respiratory failure secondary to asthma, known as acute asphyxial asthma (AAA) in adults, is uncommonly reported in children. Here, we report a case of a child with the acute onset of respiratory failure consistent with AAA complicated by the finding of a neck mass during resuscitation. This 11-year-old boy with a history of asthma initially presented in respiratory failure with altered mental status after the complaint of difficulty in breathing minutes before collapsing at home. Initially, his respiratory failure was thought to be secondary to status asthmaticus, and treatment was initiated accordingly. However, a neck mass noted during the resuscitation was cause for concern, and other etiologies for his respiratory failure were considered, including an airway obstructing neck mass. After pediatric surgery and anesthesia consultation for intubation and possible tracheostomy placement, general anesthesia was induced in the operating room with an inhaled anesthetic, with prompt resolution of the bronchspasm and decompression of the neck mass. Review of the imaging and clinical course ultimately yielded a diagnosis of cervical lung herniation as the etiology of his neck mass. We report this case of AAA and cervical lung herniation and a review of the literature of these 2 uncommon phenomena in children. PMID:25831031

  3. Co-occurrence of Joubert syndrome and Jeune asphyxiating thoracic dystrophy.

    PubMed

    Lehman, A M; Eydoux, P; Doherty, D; Glass, I A; Chitayat, D; Chung, B Y H; Langlois, S; Yong, S L; Lowry, R B; Hildebrandt, F; Trnka, P

    2010-06-01

    Ciliary disorders share typical features, such as polydactyly, renal and biliary cystic dysplasia, and retinitis pigmentosa, which often overlap across diagnostic entities. We report on two siblings of consanguineous parents and two unrelated children, both of unrelated parents, with co-occurrence of Joubert syndrome and Jeune asphyxiating thoracic dystrophy, an association that adds to the observation of common final patterns of malformations in ciliary disorders. Using homozygosity mapping in the siblings, we were able to exclude all known genes/loci for both syndromes except for INVS, AHI1, and three genes from the previously described Jeune locus at 15q13. No pathogenic variants were found in these genes by direct sequencing. In the third child reported, sequencing of RPGRIP1L, ARL13B, AHI1, TMEM67, OFD1, CC2D2A, and deletion analysis of NPHP1 showed no mutations. Although this study failed to identify a mutation in the patients tested, the co-occurrence of Joubert and Jeune syndromes is likely to represent a distinct entity caused by mutations in a yet to be discovered gene. The mechanisms by which certain organ systems are affected more than others in the spectrum of ciliary diseases remain largely unknown. PMID:20503315

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

    PubMed

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

    2013-11-01

    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.

  5. Mutations in the Gene Encoding IFT Dynein Complex Component WDR34 Cause Jeune Asphyxiating Thoracic Dystrophy

    PubMed Central

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

    2013-01-01

    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. PMID:24183451

  6. PeriStats: Perinatal Statistics

    MedlinePlus

    ... is developed by the March of Dimes Perinatal Data Center and provides access to maternal and infant health ... on PeriStats sometimes different from my health department's data? What should I do if pop-up blocker ... We acknowledge the Centers for Disease Control and Prevention for its support ...

  7. Evolution of Apparent Diffusion Coefficient and Fractional Anisotropy in the Cerebrum of Asphyxiated Newborns Treated with Hypothermia over the First Month of Life.

    PubMed

    Kwan, Saskia; Boudes, Elodie; Benseler, Anouk; Gilbert, Guillaume; Saint-Martin, Christine; Shevell, Michael; Wintermark, Pia

    2015-01-01

    The objective of this study was to assess the evolution of diffusion-weighted imaging (DWI) and diffusion-tensor imaging (DTI) over the first month of life in asphyxiated newborns treated with hypothermia and to compare it with that of healthy newborns. Asphyxiated newborns treated with hypothermia were enrolled prospectively; and the presence and extent of brain injury were scored on each MRI. Apparent diffusion coefficient (ADC) and fractional anisotropy (FA) values were measured in the basal ganglia, in the white matter and in the cortical grey matter. Sixty-one asphyxiated newborns treated with hypothermia had a total of 126 ADC and FA maps. Asphyxiated newborns developing brain injury eventually had significantly decreased ADC values on days 2-3 of life and decreased FA values around day 10 and 1 month of life compared with those not developing brain injury. Despite hypothermia treatment, asphyxiated newborns may develop brain injury that still can be detected with advanced neuroimaging techniques such as DWI and DTI as early as days 2-3 of life. A study of ADC and FA values over time may aid in the understanding of how brain injury develops in these newborns despite hypothermia treatment.

  8. Erythropoietin: a novel therapy for hypoxic-ischaemic encephalopathy?

    PubMed

    Wu, Yvonne W; Gonzalez, Fernando F

    2015-04-01

    Perinatal hypoxic-ischaemic encephalopathy (HIE) occurs in 1 to 3 per 1000 term births. HIE is not preventable in most cases, and therapies are limited. Hypothermia improves outcomes and is the current standard of care. Yet, clinical trials suggest that 44-53% of infants who receive hypothermia will die or suffer moderate to severe neurological disability. In this article, we review the preclinical and clinical evidence for erythropoietin (EPO) as a potential novel neuroprotective agent for the treatment of HIE. EPO is a novel neuroprotective agent, with remarkable neuroprotective and neuroregenerative effects in animals. Rodent and primate models of neonatal brain injury support the safety and efficacy of multiple EPO doses for improving histological and functional outcomes after hypoxia-ischaemia. Small clinical trials of EPO in neonates with HIE have also provided evidence supporting safety and preliminary efficacy in humans. There is currently insufficient evidence to support the use of high-dose EPO in newborns with HIE. However, several on-going trials will provide much needed data regarding the safety and efficacy of this potential new therapy when given in conjunction with hypothermia for HIE. Novel neuroprotective therapies are needed to further reduce the rate and severity of neurodevelopmental disabilities resulting from HIE. High-dose EPO is a promising therapy that can be administered in conjunction with hypothermia. However, additional data are needed to determine the safety and efficacy of this adjuvant therapy for HIE.

  9. Erythropoietin: a novel therapy for hypoxic-ischaemic encephalopathy?

    PubMed

    Wu, Yvonne W; Gonzalez, Fernando F

    2015-04-01

    Perinatal hypoxic-ischaemic encephalopathy (HIE) occurs in 1 to 3 per 1000 term births. HIE is not preventable in most cases, and therapies are limited. Hypothermia improves outcomes and is the current standard of care. Yet, clinical trials suggest that 44-53% of infants who receive hypothermia will die or suffer moderate to severe neurological disability. In this article, we review the preclinical and clinical evidence for erythropoietin (EPO) as a potential novel neuroprotective agent for the treatment of HIE. EPO is a novel neuroprotective agent, with remarkable neuroprotective and neuroregenerative effects in animals. Rodent and primate models of neonatal brain injury support the safety and efficacy of multiple EPO doses for improving histological and functional outcomes after hypoxia-ischaemia. Small clinical trials of EPO in neonates with HIE have also provided evidence supporting safety and preliminary efficacy in humans. There is currently insufficient evidence to support the use of high-dose EPO in newborns with HIE. However, several on-going trials will provide much needed data regarding the safety and efficacy of this potential new therapy when given in conjunction with hypothermia for HIE. Novel neuroprotective therapies are needed to further reduce the rate and severity of neurodevelopmental disabilities resulting from HIE. High-dose EPO is a promising therapy that can be administered in conjunction with hypothermia. However, additional data are needed to determine the safety and efficacy of this adjuvant therapy for HIE. PMID:25800490

  10. Encephalopathy

    MedlinePlus

    ... pressure in the skull, prolonged exposure to toxic elements (including solvents, drugs, radiation, paints, industrial chemicals, and certain metals), chronic progressive trauma, poor nutrition, ...

  11. Treatment of Overt Hepatic Encephalopathy.

    PubMed

    Sussman, Norman L

    2015-08-01

    Hepatic encephalopathy (HE) is defined by an altered mental status in the setting of portosystemic shunting, with or without cirrhosis. The basis of HE is probably multi-factorial, but increased ammonia delivery to the brain is thought to play a pivotal role. Medical therapies have typically focused on reducing blood ammonia concentrations. These measures are moderately effective, but further improvements will require identification of new therapeutic targets. Two medications, lactulose and rifaximin, are currently approved for the treatment of HE in the USA - new compounds are available off-label, and are in clinical trials. The presence of HE is associated with a higher risk of death in cirrhotic patients. Liver transplantation typically cures HE, but HE does not increase the MELD score, and therefore does not contribute to the likelihood of liver transplantation. PMID:26195208

  12. Suicide and Chronic Traumatic Encephalopathy.

    PubMed

    Iverson, Grant L

    2016-01-01

    For nearly 80 years, suicidality was not considered to be a core clinical feature of chronic traumatic encephalopathy (CTE). In recent years, suicide has been widely cited as being associated with CTE, and now depression has been proposed to be one of three core diagnostic features alongside cognitive impairment and anger control problems. This evolution of the clinical features has been reinforced by thousands of media stories reporting a connection between mental health problems in former athletes and military veterans, repetitive neurotrauma, and CTE. At present, the science underlying the causal assumption between repetitive neurotrauma, depression, suicide, and the neuropathology believed to be unique to CTE is inconclusive. Epidemiological evidence indicates that former National Football League players, for example, are at lower, not greater, risk for suicide than men in the general population. This article aims to discuss the critical issues and literature relating to these possible relationships. PMID:26449269

  13. Bovine Spongiform Encephalopathy: Atypical Pros and Cons

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

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

    PubMed Central

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

    2016-01-01

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

  15. Bovine spongiform encephalopathy: "mad cow disease".

    PubMed

    1996-07-01

    Bovine spongiform encephalopathy (BSE), also known as "mad cow disease," is a fatal brain disease of cattle first recognized in the United Kingdom. In humans, the most common transmissible spongiform encephalopathy is Creutzfeldt-Jacob Disease (CJD). Although no cases of CJD have been directly linked to beef consumption, an advisory committee has reported that 10 recent cases of a CJD variant may be associated with BSE. This announcement has alarmed consumers well beyond the borders of the United Kingdom.

  16. Ethical issues in perinatal genetics.

    PubMed

    Chervenak, Frank A; McCullough, Laurence B

    2011-04-01

    Ethics is an essential dimension of perinatal genetics. This article introduces perinatologists to the ethical principles of beneficence and respect for autonomy and uses these ethical principles to articulate the ethical concept of the fetus as a patient. Together these constitute an ethical framework that we apply to risk assessment, in response to which women may be divided into four groups: prenatal genetic counseling, and the responsible management of pregnancies complicated by genetic anomalies of the fetus.

  17. Ethical issues in perinatal genetics.

    PubMed

    Chervenak, Frank A; McCullough, Laurence B

    2011-04-01

    Ethics is an essential dimension of perinatal genetics. This article introduces perinatologists to the ethical principles of beneficence and respect for autonomy and uses these ethical principles to articulate the ethical concept of the fetus as a patient. Together these constitute an ethical framework that we apply to risk assessment, in response to which women may be divided into four groups: prenatal genetic counseling, and the responsible management of pregnancies complicated by genetic anomalies of the fetus. PMID:21051301

  18. Adverse Perinatal Outcome in Subsequent Pregnancy after Stillbirth by Placental Vascular Disorders

    PubMed Central

    Monari, Francesca; Pedrielli, Giulia; Vergani, Patrizia; Pozzi, Elisa; Mecacci, Federico; Serena, Caterina; Neri, Isabella; Facchinetti, Fabio

    2016-01-01

    Objective To evaluate outcome in the pregnancy following a stillbirth (SB) by a placental vascular disorders. Study Design A prospective, observational, multicenter study was conducted in woman with a history of stillbirth (> 22 weeks) between 2005 and June 2013, in 3 Italian University Hospitals. Causes of SB were previously identified after extensive investigations. Pregnant women were enrolled within the first trimester. The main outcome was “adverse neonatal outcome”, including perinatal death, fetal growth restriction, early preterm birth <33+6 weeks, hypoxic-ischemic encephalopathy, intracranial hemorrhage or respiratory distress. Results Out of 364 index pregnancies, 320 women (87.9%) had a subsequent pregnancy during the study period. Forty-seven had an early pregnancy loss. Out of 273 babies, 67 (24.5%) had an adverse perinatal outcome, including 1 SB and 1 early neonatal death (3.7/1000). Women who had a SB related to placental vascular disorders (39.6%), were at higher risk of an adverse neonatal outcome compared with women whose SB was unexplained or resulted from other causes (Adj. OR = 2.1, 95%CI: 1.2–3.8). Moreover, also obesity independently predicts an adverse perinatal outcome (Adj OR = 2.1, 95%CI: 1.1–4.3). Conclusion When previous SB is related to placental vascular disorders there is a high risk for adverse neonatal outcomes in the subsequent pregnancy. Maternal obesity is an additional risk factor. PMID:27228078

  19. Ischemic perinatal stroke: challenge and opportunities.

    PubMed

    Raju, Tonse N K

    2008-08-01

    The second highest risk group for developing a cerebral stroke is the perinatal period, generally defined as 20 weeks of gestation through 28th postnatal day of age. In this commentary, a brief overview of ischemic perinatal strokes is presented. Ischemic perinatal stroke (IPS) occurs at a rate of 1 : 2300 to 1 : 5000 births, accounting for 30% of children with hemiplegic cerebral palsy (CP). Thus, IPS is the most common known cause for CP [1-3]. Although they occur frequently, much remains to be studied about perinatal strokes in general and the ischemic variety in particular. PMID:18705894

  20. [Pathogenesis of acute encephalitis and acute encephalopathy].

    PubMed

    Shiomi, Masashi

    2011-03-01

    Many aspects of the pathogenesis of acute encephalitis and acute encephalopathy have been clarified in this decade, although many unknown mechanisms remain to be elucidated. According to progress of MRI and neuroimmunological analysis and the observation of clinical findings, many new syndromes were found, which enhanced our understanding of acute encephalitis and acute encephalopathy. The pathogenesis of encephalitis is divided into infection and immune mediated mechanisms. The antibodies to neuronal surface antigens(NSA) such as NMDA receptors, leucin-rich glioma inactivated 1 (LGI1) and aquaporin 4 were demonstrated in specific encephalitis, limbic encephalitis and neuromyelitis optica. Anti-NSA antibody encephalitis should be treated by immunotherapy such as corticosteroid and plasmapheresis. Acute encephalitis with refractory repetitive partial seizures (AERRPS) is a devastating postinfectious disease in children and adults, although the pathogenesis of AERRPS is poorly understood. Influenza associated encephalopathy(IAE) is characterized by it's high incidence in Japanese children between 1 year and 5 years of age, its onset in the first or the second day of illness and its high mortality (15-30%) and morbidity (25-40%). We proposed the classification of IAE with poor prognosis from the neuroradiological findings. Four types of encephalopathy seem to be differentiated from each other, acute necrotizing encephalopathy (ANE) type, hemorrhagic shock and encephalopathy syndrome (HSES) type, acute brain swelling (ABS) type, febrile convulsive status epilepticus (FCSE) type. The notable radiological features are thalamic lesions in ANE, diffuse cerebral cortical cytotoxic edema in HSES, reversible cerebral swelling in ABS which sometimes reaches lethal brain herniation, and in FCSE type, dendritic high signal in subcortical white matter by DWI ("bright tree appearance") appears simultaneously with the later onset of repetitive focal seizure. These four types are

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

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

    2006-01-01

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

  3. Chronic traumatic encephalopathy and athletes.

    PubMed

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

    2015-10-27

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

  4. Encephalopathy caused by lanthanum carbonate

    PubMed Central

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

    2011-01-01

    Lanthanum carbonate is a nonaluminum, noncalcium phosphate-binding agent, which is widely used in patients with end-stage chronic kidney disease. Until now, no significant side-effects have been described for the clinical use of lanthanum carbonate, and there are no available clinical data regarding its tissue stores. Here we report the case of a 59-year-old patient who was admitted with confusional syndrome. The patient received 3750 mg of lanthanum carbonate daily. Examinations were carried out, and the etiology of the encephalopathy of the patient could not be singled out. The lanthanum carbonate levels in serum and cerebrospinal fluid were high, and the syndrome eased after the drug was removed. The results of our study confirm that, in our case, the lanthanum carbonate did cross the blood-brain barrier (BBB). Although lanthanum carbonate seems a safe drug with minimal absorption, this work reveals the problem derived from the increase of serum levels of lanthanum carbonate, and the possibility that it may cross the BBB. Further research is required on the possible pathologies that increase serum levels of lanthanum carbonate, as well as the risks and side-effects derived from its absorption. PMID:25984155

  5. Joseph Haydn's encephalopathy: new aspects.

    PubMed

    Blahak, Christian; Bäzner, Hansjörg; Hennerici, Michael G

    2015-01-01

    With increasing age, Joseph Haydn complained of progressive forgetfulness preventing him from composing for about the last 8 years of his life. He spent his days more and more inactive and immobilized, suffering from a disabling gait disturbance. Still, most biographers consider diffuse atherosclerosis and congestive heart failure to be reasons for Haydn's medical condition and physical decline during the last years of his life. A more sophisticated and detailed inspection of documents and sources, however, leads to the diagnosis of subcortical vascular encephalopathy (SVE), caused by progressive cerebral small vessel disease. Important features of the disease are mood changes, urinary symptoms, and in particular a characteristic gait disturbance, while dementia is only mild and occurs later in the course. Haydn was severely disabled by the symptoms of SVE for several years and often reported difficulties in the completion of his last oratorio "Die Jahreszeiten" (The Seasons). Subsequently, the disease prevented him from composing another large oratorio, "Das jüngste Gericht" (The last judgement), which had been already drafted. Finally, the progress of SVE stopped his long career as a composer and conductor at the age of 73 years. PMID:25684297

  6. Clinical Neurophysiology of Hepatic Encephalopathy

    PubMed Central

    Amodio, Piero; Montagnese, Sara

    2015-01-01

    Background/Objectives Hepatic encephalopathy (HE) has relevant impact on the quality of life of patients and their caregivers and causes relevant costs because of hospitalizations and work days lost. Its quantification is important to perform adequate clinical trials on this relevant complication of cirrhosis and portal-systemic shunting. Clinical neurophysiology, which detects functional alterations of the nervous system, has been applied to the study of HE for over 60 years. This review aims at summarizing and clarifying the role of neurophysiologic techniques in the study of HE. Methods A narrative review was performed aiming at interpreting the cited papers and the techniques on the basis of their physiological and pathophysiological meaning. Results The potential role of EEG, quantified EEG, evoked potentials—both exogenous, endogenous and motor—have been clarified to the reader that may be unfamiliar with neurophysiology. Conclusions The EEG, reflecting the oscillatory changes of neural network is the preferable tool to detect and monitor HE, with the exception of its most severe stage, when EEG flattens. SSEP and MEP have indication to detect and monitor transmission alterations that are likely related to myelin changes and microedema. PMID:26041960

  7. Gut microbiota and hepatic encephalopathy.

    PubMed

    Dhiman, Radha K

    2013-06-01

    There is a strong relationship between liver and gut; while the portal venous system receives blood from the gut, and its contents may affect liver functions, liver in turn, affects intestinal functions through bile secretion. There is robust evidence that the pathogenesis of hepatic encephalopathy (HE) is linked to alterations in gut microbiota and their by-products such as ammonia, indoles, oxindoles, endotoxins, etc. In the setting of intestinal barrier and immune dysfunction, these by-products are involved in the pathogenesis of complications of liver cirrhosis including HE and systemic inflammation plays an important role. Prebiotics, probiotics and synbiotics may exhibit efficacy in the treatment of HE by modulating the gut flora. They improve derangement in flora by decreasing the counts of pathogenic bacteria and thus improving the endotoxemia, HE and the liver disease. Current evidence suggest that the trials evaluating the role of probiotics in the treatment of HE are of not high quality and all trials had high risk of bias and high risk of random errors. Therefore, the use of probiotics for patients with HE cannot be currently recommended. Further RCTs are required. This review summarizes the main literature findings about the relationships between gut flora and HE, both in terms of the pathogenesis and the treatment of HE.

  8. Joseph Haydn's encephalopathy: new aspects.

    PubMed

    Blahak, Christian; Bäzner, Hansjörg; Hennerici, Michael G

    2015-01-01

    With increasing age, Joseph Haydn complained of progressive forgetfulness preventing him from composing for about the last 8 years of his life. He spent his days more and more inactive and immobilized, suffering from a disabling gait disturbance. Still, most biographers consider diffuse atherosclerosis and congestive heart failure to be reasons for Haydn's medical condition and physical decline during the last years of his life. A more sophisticated and detailed inspection of documents and sources, however, leads to the diagnosis of subcortical vascular encephalopathy (SVE), caused by progressive cerebral small vessel disease. Important features of the disease are mood changes, urinary symptoms, and in particular a characteristic gait disturbance, while dementia is only mild and occurs later in the course. Haydn was severely disabled by the symptoms of SVE for several years and often reported difficulties in the completion of his last oratorio "Die Jahreszeiten" (The Seasons). Subsequently, the disease prevented him from composing another large oratorio, "Das jüngste Gericht" (The last judgement), which had been already drafted. Finally, the progress of SVE stopped his long career as a composer and conductor at the age of 73 years.

  9. Chronic Traumatic Encephalopathy: A Review

    PubMed Central

    Saulle, Michael; Greenwald, Brian D.

    2012-01-01

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

  10. While waiting: early recognition and initial management of neonatal hypoxic-ischemic encephalopathy.

    PubMed

    Sussman, Craig B; Weiss, Michael D

    2013-12-01

    Hypoxic-ischemic encephalopathy (HIE) occurring during the perinatal period is one of the primary causes of severe, long-term neurological deficits in children. Initial systemic supportive therapy remains a critical aspect of HIE management. In addition to support therapy, the widespread use of hypothermia has demonstrated a reduction in death and neurodevelopmental disability in infants with moderate to severe HIE. Neonates with HIE born outside of tertiary care centers must be rapidly identified as hypothermia candidates and have emergent transport arranged. While waiting for the transport team to arrive, these neonates often require intensive stabilization, including meticulous temperature management. This article examines the need for HIE outreach teaching programs, assists in the identification of a neonate for hypothermia therapy, and supplies evidence-based recommendations for the initial stabilization and care of neonates delivered at nontertiary care facilities. The guidelines and materials supplied represent the outreach model used by our regional hypothermia center and disseminated to the surrounding referral hospitals.

  11. Impaired cerebral autoregulation and brain injury in newborns with hypoxic-ischemic encephalopathy treated with hypothermia.

    PubMed

    Massaro, An N; Govindan, R B; Vezina, Gilbert; Chang, Taeun; Andescavage, Nickie N; Wang, Yunfei; Al-Shargabi, Tareq; Metzler, Marina; Harris, Kari; du Plessis, Adre J

    2015-08-01

    Impaired cerebral autoregulation may contribute to secondary injury in newborns with hypoxic-ischemic encephalopathy (HIE). Continuous, noninvasive assessment of cerebral pressure autoregulation can be achieved with bedside near-infrared spectroscopy (NIRS) and systemic mean arterial blood pressure (MAP) monitoring. This study aimed to evaluate whether impaired cerebral autoregulation measured by NIRS-MAP monitoring during therapeutic hypothermia and rewarming relates to outcome in 36 newborns with HIE. Spectral coherence analysis between NIRS and MAP was used to quantify changes in the duration [pressure passivity index (PPI)] and magnitude (gain) of cerebral autoregulatory impairment. Higher PPI in both cerebral hemispheres and gain in the right hemisphere were associated with neonatal adverse outcomes [death or detectable brain injury by magnetic resonance imaging (MRI), P < 0.001]. NIRS-MAP monitoring of cerebral autoregulation can provide an ongoing physiological biomarker that may help direct care in perinatal brain injury.

  12. Impaired cerebral autoregulation and brain injury in newborns with hypoxic-ischemic encephalopathy treated with hypothermia.

    PubMed

    Massaro, An N; Govindan, R B; Vezina, Gilbert; Chang, Taeun; Andescavage, Nickie N; Wang, Yunfei; Al-Shargabi, Tareq; Metzler, Marina; Harris, Kari; du Plessis, Adre J

    2015-08-01

    Impaired cerebral autoregulation may contribute to secondary injury in newborns with hypoxic-ischemic encephalopathy (HIE). Continuous, noninvasive assessment of cerebral pressure autoregulation can be achieved with bedside near-infrared spectroscopy (NIRS) and systemic mean arterial blood pressure (MAP) monitoring. This study aimed to evaluate whether impaired cerebral autoregulation measured by NIRS-MAP monitoring during therapeutic hypothermia and rewarming relates to outcome in 36 newborns with HIE. Spectral coherence analysis between NIRS and MAP was used to quantify changes in the duration [pressure passivity index (PPI)] and magnitude (gain) of cerebral autoregulatory impairment. Higher PPI in both cerebral hemispheres and gain in the right hemisphere were associated with neonatal adverse outcomes [death or detectable brain injury by magnetic resonance imaging (MRI), P < 0.001]. NIRS-MAP monitoring of cerebral autoregulation can provide an ongoing physiological biomarker that may help direct care in perinatal brain injury. PMID:26063779

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

    PubMed Central

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

    2015-01-01

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

  14. SPTAN1 encephalopathy: distinct phenotypes and genotypes.

    PubMed

    Tohyama, Jun; Nakashima, Mitsuko; Nabatame, Shin; Gaik-Siew, Ch'ng; Miyata, Rie; Rener-Primec, Zvonka; Kato, Mitsuhiro; Matsumoto, Naomichi; Saitsu, Hirotomo

    2015-04-01

    Recent progress in genetic analysis reveals that a significant proportion of cryptogenic epileptic encephalopathies are single-gene disorders. Mutations in numerous genes for early-onset epileptic encephalopathies have been rapidly identified, including in SPTAN1, which encodes α-II spectrin. The aim of this review is to delineate SPTAN1 encephalopathy as a distinct clinical syndrome. To date, a total of seven epileptic patients with four different in-frame SPTAN1 mutations have been identified. The major clinical features of SPTAN1 mutations include epileptic encephalopathy with hypsarrhythmia, no visual attention, acquired microcephaly, spastic quadriplegia and severe intellectual disability. Brainstem and cerebellar atrophy and cerebral hypomyelination, as observed by magnetic resonance imaging, are specific hallmarks of this condition. A milder variant is characterized by generalized epilepsy with pontocerebellar atrophy. Only in-frame SPTAN1 mutations in the last two spectrin repeats in the C-terminal region lead to dominant negative effects and these specific phenotypes. The last two spectrin repeats are required for α/β spectrin heterodimer associations and the mutations can alter heterodimer formation between the two spectrins. From these data we suggest that SPTAN1 encephalopathy is a distinct clinical syndrome owing to specific SPTAN1 mutations. It is important that this syndrome is recognized by pediatric neurologists to enable proper diagnostic work-up for patients. PMID:25631096

  15. Hypothermia for hypoxic–ischemic encephalopathy

    PubMed Central

    Cotten, C Michael; Shankaran, Seetha

    2010-01-01

    Moderate to severe hypoxic–ischemic injury in newborn infants, manifested as encephalopathy immediately or within hours after birth, is associated with a high risk of either death or a lifetime with disability. In recent multicenter clinical trials, hypothermia initiated within the first 6 postnatal hours has emerged as a therapy that reduces the risk of death or impairment among infants with hypoxic–ischemic encephalopathy. Prior to hypothermia, no therapies directly targeting neonatal encephalopathy secondary to hypoxic–ischemic injury had convincing evidence of efficacy. Hypothermia therapy is now becoming increasingly available at tertiary centers. Despite the deserved enthusiasm for hypothermia, obstetric and neonatology caregivers, as well as society at large, must be reminded that in the clinical trials more than 40% of cooled infants died or survived with impairment. Although hypothermia is an evidence-based therapy, additional discoveries are needed to further improve outcome after HIE. In this article, we briefly present the epidemiology of neonatal encephalopathy due to hypoxic–ischemic injury, describe the rationale for the use of hypothermia therapy for hypoxic–ischemic encephalopathy, and present results of the clinical trials that have demonstrated the efficacy of hypothermia. We also present findings noted during and after these trials that will guide care and direct research for this devastating problem. PMID:20625441

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

    PubMed

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

    2015-11-01

    Oxidative stress (OS) occurs at birth in all newborns as a consequence of the hyperoxic challenge due to the transition from the hypoxic intrauterine environment to extrauterine life. Free radical (FRs) sources such as inflammation, hyperoxia, hypoxia, ischaemia-reperfusion, neutrophil and macrophage activation, glutamate and free iron release, all increases the OS during the perinatal period. Newborns, and particularly preterm infants, have reduced antioxidant defences and are not able to counteract the harmful effects of FRs. Energy metabolism is central to life because cells cannot exist without an adequate supply of ATP. Due to its growth, the mammalian brain can be considered as a steady-state system in which ATP production matches ATP utilisation. The developing brain is particularly sensitive to any disturbances in energy generation, and even a short-term interruption can lead to long-lasting and irreversible damage. Whenever energy failure develops, brain damage can occur. Accumulating evidence indicates that OS is implicated in the pathogenesis of many neurological diseases, such as intraventricular haemorrhage, hypoxic-ischaemic encephalopathy and epilepsy. PMID:23968388

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

    PubMed

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

    2015-11-01

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

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

    PubMed Central

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

    2015-01-01

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

  19. Perinatal depression: implications for child mental health.

    PubMed

    Muzik, Maria; Borovska, Stefana

    2010-12-01

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

  20. Guidelines for Perinatal Care. Second Edition.

    ERIC Educational Resources Information Center

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

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

  1. Minimal Brain Dysfunction: Associations with Perinatal Complications.

    ERIC Educational Resources Information Center

    Nichols, Paul L.

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

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

    ERIC Educational Resources Information Center

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

    2011-01-01

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

  3. Clinical review of genetic epileptic encephalopathies

    PubMed Central

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

    2012-01-01

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

  4. Hashimoto Encephalopathy or Neurosarcoidosis? A Case Report

    PubMed Central

    Sapkota, Biggya L.; Pitiyanuvath, Nataria

    2015-01-01

    Hashimoto encephalopathy (HE) is a rare autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased antithyroid antibody levels. Neurosarcoidosis is also a rare entity that occurs in less than 5% of patients with systemic sarcoidosis. Neurosarcoidosis usually presents with cranial neuropathies, myelopathy, or new-onset seizure. We report a case of a 49-year-old caucasian woman, previously healthy, who initially presented for a workup of a new-onset seizure. She had a gradually progressive course with neurocognitive decline and recurrent partial seizures refractory to conventional antiepileptic drugs. Her seizures responded well to a course of intravenous immunoglobulin. She was subsequently diagnosed with HE and pulmonary sarcoidosis based on serological and pathological studies. She improved neurologically once the seizures were controlled. Hashimoto encephalopathy is a rare condition that is potentially treatable and presents with various neuropsychiatric manifestations. It is a diagnosis of exclusion that requires a strong clinical suspicion and is often underrecognized. PMID:25829987

  5. Why the confusion in Hashimoto's encephalopathy?

    PubMed

    Jayasekera, Bodiabaduge A P; McShane, Michael Anthony; Roy, Prem; Anand, Geetha

    2011-01-01

    A 13-year-old girl presented with an afebrile seizure followed by prolonged confusion and visual hallucinations. Initial investigations in the form of blood tests, cerebrospinal fluid analysis and head imaging by CT, were normal. She represented with two further episodes within a period of 3 weeks. Further investigations considering infective, metabolic and some autoimmune causes of encephalopathy were negative. An MRI head scan was normal. Thyroid function testing disclosed primary hypothyroidism and elevated antithyroid antibodies. She responded well to glucocorticoid therapy for presumed Hashimoto's encephalopathy (HE). HE describes patients with various neurological manifestations with elevated titres of antithyroid antibodies. There are no clear criteria for diagnosis, with many cases labelled as HE. Responses to corticosteroid therapy are favourable. In patients with unexplained encephalopathy, HE should be considered given the favourable response to glucocorticoid therapy. PMID:22691944

  6. Wernicke's Encephalopathy Complicating Hyperemesis during Pregnancy.

    PubMed

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

    2016-01-01

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

  7. Sepsis-associated encephalopathy: not just delirium

    PubMed Central

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

    2011-01-01

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

  8. Pediatric Epileptic Encephalopathies: Pathophysiology and Animal Models.

    PubMed

    Shao, Li-Rong; Stafstrom, Carl E

    2016-05-01

    Epileptic encephalopathies are syndromes in which seizures or interictal epileptiform activity contribute to or exacerbate brain function, beyond that caused by the underlying pathology. These severe epilepsies begin early in life, are associated with poor lifelong outcome, and are resistant to most treatments. Therefore, they represent an immense challenge for families and the medical care system. Furthermore, the pathogenic mechanisms underlying the epileptic encephalopathies are poorly understood, hampering attempts to devise novel treatments. This article reviews animal models of the three classic epileptic encephalopathies-West syndrome (infantile spasms), Lennox-Gastaut syndrome, and continuous spike waves during sleep or Landau-Kleffner syndrome-with discussion of how animal models are revealing underlying pathophysiological mechanisms that might be amenable to targeted therapy. PMID:27544466

  9. Perinatal loss, trauma, and dream reports.

    PubMed

    Kroth, Jerry; Garcia, Marylynne; Hallgren, Michelle; LeGrue, Emilyann; Ross, Maureen; Scalise, Juliana

    2004-06-01

    This study investigated correlations among dream characteristics and measures of trauma and perinatal bereavement as reported by women who have experienced perinatal loss. 37 women who had experienced perinatal loss were randomly selected from a perinatal support group and administered the Impact of Event Scale, the Perinatal Grief Scale, and the KJP Dream Inventory. Scores on the Impact of Events Scale (IES) correlated with Emotional Pain (.41), Despair (.37), Dreams of Death (.31), Dreams of Water (-.29), and Dreams of Being Famous (-.36). Subjects who reported higher Social Support and Emotional Expressiveness throughout their trauma showed lower scores on IES Total scores (-.52), Despair (-.62), and reported dreaming more in color (.41). Results are discussed in terms of the hypothesized role dreams may play in the grief-recovery process. PMID:15217043

  10. Perinatal Safety: From Concept to Nursing Practice

    PubMed Central

    Kennedy, Holly Powell

    2010-01-01

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

  11. A literature review on integrated perinatal care

    PubMed Central

    Rodríguez, Charo; des Rivières-Pigeon, Catherine

    2007-01-01

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

  12. Concussion in Chronic Traumatic Encephalopathy

    PubMed Central

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

    2015-01-01

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

  13. Concussion in Chronic Traumatic Encephalopathy.

    PubMed

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

    2015-10-01

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

  14. Transmissible encephalopathies: speculations and realities.

    PubMed

    Manuelidis, Laura

    2003-01-01

    Virtually all transmissible encephalopathies (TSEs), such as scrapie, CJD, and BSE, are caused by a type of infectious particle that remains enigmatic. The language of prion theory supersedes the reality of what is, and what is not known. This review questions the predictive value, consistency and accuracy of this now dominant assumption. Many people believe the normal cellular prion protein (PrP) self-converts into an infectious amyloid protein or prion. Although the amyloidogenic capacity of proteins is well established, the concept of an infectious protein without nucleic acid was "revolutionary." Diverse experiments have repeatedly shown, however, that this protein alone, in any form, is incapable of reproducing transmissible infection. In contrast, the infectious agent copurifies with many other molecules, including nucleic acids, while it separates from the majority of PrP. The infectious particle has a homogeneous viral size of ~25 nm, and infectivity is markedly reduced by conditions that disrupt viral core components but do not disrupt multimers of PrP amyloid. Additionally, the infectious agent replicates to high levels before any PrP abnormalities can be detected. Hence, we initially proposed that PrP changes are part of the host's pathologic response to high levels of infectious agent, but not the agent itself. Newer data clarifying a role for myeloid cells in the spread of infection, the unique character of two different agent strains propagated in a single animal, and the demonstration of long nucleic acids in a variety of simplified high titer preparations continue to raise serious questions for the prion hypothesis. Moreover, the epidemic spread of TSEs, and the activation of host innate immune mechanisms by infection, further indicate these agents are recognizably foreign, and probably viral.

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

    Technology Transfer Automated Retrieval System (TEKTRAN)

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

  16. Dengue fever with encephalopathy in Australia.

    PubMed

    Row, D; Weinstein, P; Murray-Smith, S

    1996-03-01

    During an epidemic of dengue type 2 virus in the rural community of Charters Towers, North Queensland, Australia, in 1993, 210 cases presented to the local hospital with signs and symptoms of classic dengue fever. Two cases were noteworthy because of neurologic complications, which included drowsiness, short term memory loss, agitation, and seizure. The cases are presented in detail because they are the first cases of dengue-associated encephalopathy to be documented in Australia. An increasing number of cases of encephalopathy associated with classic dengue fever is being reported world wide, but the etiology of this clinical syndrome remains unknown.

  17. Current issues in perinatal epidemiology.

    PubMed Central

    Berendes, H. W.

    1987-01-01

    The main national data sources for perinatal epidemiology are birth and death certificates, yet routinely linked birth and death certificate data are still not available in the U.S. Completeness and quality of the reporting of perinatal events should be considered in examining trends over time and between jurisdictions. The U.S. has experienced a marked decline in its infant mortality rate, but only a very modest decline in the rate of low birth weight. Research must focus more on studies of pre-term labor, rather than low birth weight, which include children who are underground or who are born too early and who, therefore, may represent different etiologies. Sensitive hormonal tests may provide more precise estimates of the rate of very early fetal loss. Management of labor and delivery and of the high-risk newborn have undergone marked changes during the last 15 years, and yet clinical trials have not played a major role in the evaluation of these changes. The difference in reproductive outcomes between whites and blacks, especially in the rate of low birth weight, have persisted and are not understood. Data bases are becoming available for intergenerational studies to determine whether nature or nurture accounts for this difference. PMID:3660860

  18. A Piglet Model of Neonatal Hypoxic-Ischemic Encephalopathy.

    PubMed

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

    2015-05-16

    Birth asphyxia, which causes hypoxic-ischemic encephalopathy (HIE), accounts for 0.66 million deaths worldwide each year, about a quarter of the world's 2.9 million neonatal deaths. Animal models of HIE have contributed to the understanding of the pathophysiology in HIE, and have highlighted the dynamic process that occur in brain injury due to perinatal asphyxia. Thus, animal studies have suggested a time-window for post-insult treatment strategies. Hypothermia has been tested as a treatment for HIE in pdiglet models and subsequently proven effective in clinical trials. Variations of the model have been applied in the study of adjunctive neuroprotective methods and piglet studies of xenon and melatonin have led to clinical phase I and II trials(1,2). The piglet HIE model is further used for neonatal resuscitation- and hemodynamic studies as well as in investigations of cerebral hypoxia on a cellular level. However, it is a technically challenging model and variations in the protocol may result in either too mild or too severe brain injury. In this article, we demonstrate the technical procedures necessary for establishing a stable piglet model of neonatal HIE. First, the newborn piglet (< 24 hr old, median weight 1500 g) is anesthetized, intubated, and monitored in a setup comparable to that found in a neonatal intensive care unit. Global hypoxia-ischemia is induced by lowering the inspiratory oxygen fraction to achieve global hypoxia, ischemia through hypotension and a flat trace amplitude integrated EEG (aEEG) indicative of cerebral hypoxia. Survival is promoted by adjusting oxygenation according to the aEEG response and blood pressure. Brain injury is quantified by histopathology and magnetic resonance imaging after 72 hr.

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

    PubMed

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

    2013-10-01

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

  20. [Progressive multifocal encephalopathy in a LED patient].

    PubMed

    Tikkakoski, Tapani; Ingo, Sinikka; Julin, Lillemor; Kanckos, Sven

    2015-01-01

    Progressive multifocal encephalopathy (PML) is a rare demyelinating disease of the central nervous system, caused by the reactivation of the JC virus in the body during immunosuppression. The use of monoclonal antibodies predisposes to PML, and the epidemiology of the disease has changed. We describe the first PML published from Finland and associated with rituximab treatment in a LED patient.

  1. Phenytoin-induced encephalopathy in a child

    PubMed Central

    Mehndiratta, Sumit

    2016-01-01

    Phenytoin is a commonly used antiepileptic medication in the pediatric age group, but it has a narrow therapeutic range. Various adverse effects have been reported commonly. We report a relatively rare case of encephalopathy in a child from overdose of injectable phenytoin due to ignorance of the previous treatment. Scrutiny of medical records and history is of utmost importance while administering such medications.

  2. PRIONS AND THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

    EPA Science Inventory

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

  3. The transmissible spongiform encephalopathies of livestock.

    PubMed

    Greenlee, Justin J; Greenlee, M Heather West

    2015-01-01

    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, transmissible mink encephalopathy (TME) in mink, and Kuru and Creutzfeldt-Jakob disease (CJD) in humans. These diseases are associated with the accumulation of a protease-resistant, disease-associated isoform of the prion protein (called PrP(Sc)) in the central nervous system and other tissues, depending on the host species. Typically, TSEs are acquired through exposure to infectious material, but inherited and spontaneous TSEs also occur. All TSEs share pathologic features and infectious mechanisms but have distinct differences in transmission and epidemiology due to host factors and strain differences encoded within the structure of the misfolded prion protein. The possibility that BSE can be transmitted to humans as the cause of variant Creutzfeldt-Jakob disease has brought attention to this family of diseases. This review is focused on the TSEs of livestock: bovine spongiform encephalopathy in cattle and scrapie in sheep and goats.

  4. The transmissible spongiform encephalopathies of livestock.

    PubMed

    Greenlee, Justin J; Greenlee, M Heather West

    2015-01-01

    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, transmissible mink encephalopathy (TME) in mink, and Kuru and Creutzfeldt-Jakob disease (CJD) in humans. These diseases are associated with the accumulation of a protease-resistant, disease-associated isoform of the prion protein (called PrP(Sc)) in the central nervous system and other tissues, depending on the host species. Typically, TSEs are acquired through exposure to infectious material, but inherited and spontaneous TSEs also occur. All TSEs share pathologic features and infectious mechanisms but have distinct differences in transmission and epidemiology due to host factors and strain differences encoded within the structure of the misfolded prion protein. The possibility that BSE can be transmitted to humans as the cause of variant Creutzfeldt-Jakob disease has brought attention to this family of diseases. This review is focused on the TSEs of livestock: bovine spongiform encephalopathy in cattle and scrapie in sheep and goats. PMID:25991695

  5. Psychometric tests for diagnosing minimal hepatic encephalopathy.

    PubMed

    Weissenborn, Karin

    2013-06-01

    While it is consensus that minimal hepatic encephalopathy (mHE) has significant impact on a patient's daily living, and thus should be diagnosed and treated, there is no consensus about the optimal diagnostic tools. At present the most frequently used psychometric methods for diagnosing minimal hepatic encephalopathy are the Inhibitory Control Test and the Psychometric Hepatic Encephalopathy Score PHES. Another frequently used method is Critical Flicker Frequency. The PHES and the Repeatable Battery for the Assessment of Neuropsychological Status have been recommended for diagnosing mHE by a working party commissioned by the International Society for Hepatic Encephalopathy and Nitrogen Metabolism. Recently the Continuous Reaction Time Test, which has been used in the 1980ies, has gained new interest. Today, no data are available that allow to decide which of these methods is the most appropriate. In fact, even basic information such as dependence on age, sex and education or influence of diseases that frequently accompany liver cirrhosis upon test results is missing for most of them. Future studies must address these questions to improve diagnosis of mHE. PMID:22993201

  6. Perinatal Complications and Aging Indicators by Midlife

    PubMed Central

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

    2014-01-01

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

  7. Hepatic encephalopathy: An update of pathophysiologic mechanisms.

    PubMed

    Hazell, A S; Butterworth, R F

    1999-11-01

    Hepatic encephalopathy (HE) is a neuropsychiatric disorder that occurs in both acute and chronic liver failure. Although the precise pathophysiologic mechanisms responsible for HE are not completely understood, a deficit in neurotransmission rather than a primary deficit in cerebral energy metabolism appears to be involved. The neural cell most vulnerable to liver failure is the astrocyte. In acute liver failure, the astrocyte undergoes swelling resulting in increased intracranial pressure; in chronic liver failure, the astrocyte undergoes characteristic changes known as Alzheimer type II astrocytosis. In portal-systemic encephalopathy resulting from chronic liver failure, astrocytes manifest altered expression of several key proteins and enzymes including monoamine oxidase B, glutamine synthetase, and the so-called peripheral-type benzodiazepine receptors. In addition, expression of some neuronal proteins such as monoamine oxidase A and neuronal nitric oxide synthase are modified. In acute liver failure, expression of the astrocytic glutamate transporter GLT-1 is reduced, leading to increased extracellular concentrations of glutamate. Many of these changes have been attributed to a toxic effect of ammonia and/or manganese, two substances that are normally removed by the hepatobiliary route and that in liver failure accumulate in the brain. Manganese deposition in the globus pallidus in chronic liver failure results in signal hyperintensity on T1-weighted Magnetic Resonance Imaging and may be responsible for the extrapyramidal symptoms characteristic of portal-systemic encephalopathy. Other neurotransmitter systems implicated in the pathogenesis of hepatic encephalopathy include the serotonin system, where a synaptic deficit has been suggested, as well as the catecholaminergic and opioid systems. Further elucidation of the precise nature of these alterations could result in the design of novel pharmacotherapies for the prevention and treatment of hepatic

  8. Food Safety: Bovine Spongiform Encephalopathy (Mad Cow Disease).

    PubMed

    Acheson, David W. K.

    2002-01-01

    Bovine spongiform encephalopathy is just one of a group of diseases known as transmissible spongiform encephalopathies. Only recently has it become recognized that transmissible spongiform encephalopathies are likely due to proteins known as prions. Although it has been recognized that transmissible spongiform encephalopathies may readily spread within species, the recent observations that bovine spongiform encephalopathy in cattle may have originated from another transmissible spongiform encephalopathy in sheep, known as scrapie, is cause for concern. Further, bovine spongiform encephalopathy has now been strongly linked with a universally fatal human neurologic disease known as new variant Creutzfeldt-Jakob disease. Currently the only approach to preventing bovine spongiform encephalopathy, and subsequent new variant Creutzfeldt-Jakob disease in humans, from ingestion of bovine spongiform encephalopathy-infected material is to avoid consumption of contaminated food. Little can be done to treat food that will destroy prions and leave a palatable product. At this stage we are continuing to learn about transmissible spongiform encephalopathies and their implications on human health. This is an ever-changing situation and has an unpredictable element in terms of the extent of the current outbreaks in England and other parts of Europe. PMID:11984426

  9. Genetic and perinatal effects of abused substances

    SciTech Connect

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

    1987-01-01

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

  10. Recurrent perinatal loss: a case study.

    PubMed

    Kavanaugh, K; Robertson, P A

    1999-01-01

    To date, investigators have not demonstrated a clear relationship between a parent's history of prior perinatal losses and intensity of grief response following a subsequent perinatal loss. Examining this relationship for low-income, African-American parents is important because they are a vulnerable population due to the high incidence of perinatal mortality in Blacks and their other life stressors that can impact on grief response and caring needs. The purpose of this case study was to examine the impact of recurrent perinatal loss on a low-income African-American parent. The research design for this study was case report, using interview data collected from a mother who had recently experienced her fourth perinatal loss, which occurred at twenty-five weeks of gestation. Transcripts from two open-ended interviews were analyzed. The theoretical framework used to guide analysis of this case study was Lazarus and Folkman's stress and coping theory. Results demonstrated that the prior perinatal losses did not appear as critical components of the way the mother responded to her most recent loss. Instead, perception of the care she received from healthcare providers and how that care related to her experiences with her one living child who was born at the same gestational age was an important determinant in how she responded to her loss. The results of this case study demonstrate the importance assessing a person's perception of their experience and those factors which contribute to the way they respond.

  11. 76 FR 38667 - Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-07-01

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

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

    PubMed

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

    2012-03-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2016-01-01

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

  15. [Maternal mortality and perinatal mortality].

    PubMed

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

    1982-01-01

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

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

    PubMed

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

    2016-05-01

    Hashimoto's encephalopathy (HE) is a rare not well understood, progressive and relapsing multiform disease, characterized by seizures, movement disorders, subacute cognitive dysfunction, psychiatric symptoms and responsiveness to steroid therapy. The disorder is generally associated with thyroid diseases and the most common feature is the presence of anti-thyroperoxidase antibodies (TPOAb). Patients are usually euthyroid or mildly hypothyroid at presentation. All age groups can be affected. The pathophysiology is still unclear, especially the link between elevated serum TPOAb and the encephalopathy. Most reported cases occurred in women and girls. Unspecific symptoms, non-pathognomonic laboratory neurophysiology and neuroimaging features make its diagnosis a real challenge for clinicians. The case of a 16 year old boy, with a clinical picture of HE associated with hypothyroidism, demonstrating an excellent response to high dose steroids is presented together with a systematic review of the literature. PMID:26849953

  17. Hepatic encephalopathy after treatment with temozolomide.

    PubMed

    Goldbecker, Annemarie; Tryc, Anita Blanka; Raab, Peter; Worthmann, Hans; Herrmann, Julian; Weissenborn, Karin

    2011-05-01

    Temozolomide in combination with radiation has been in use for more than 5 years for the therapy of glioblastoma. Known adverse effects concerning the gastrointestinal system are elevation of liver enzymes. We present the case of a patient treated with temozolomide who developed severe cholestatic liver damage and consecutive hepatic encephalopathy. Neurological symptoms were mistaken as being caused by focal brain damage for more than 9 months. After the correct diagnosis had been made and the treatment had been started, the patient's condition ameliorated. In conclusion, neurological deficits in patients with known brain lesion should not be attributed automatically to the pre-existing damage even if it is progressive but should be examined carefully, also including toxic and metabolic encephalopathies into the differential diagnosis. Furthermore, new side effects of drugs have to be considered. At least this case might lead to a closer monitoring of liver enzymes during temozolomide therapy.

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

    PubMed

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

    2014-10-25

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

  19. Head stereotypies in STXBP1 encephalopathy.

    PubMed

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

    2013-08-01

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

  20. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion.

    PubMed

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-09-01

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

  1. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion

    PubMed Central

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-01-01

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

  2. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion.

    PubMed

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-09-01

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

  3. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion

    PubMed Central

    Hughes, Adrienne; Brown, Alisha; Valento, Matthew

    2016-01-01

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

  4. Transmissible Spongiform Encephalopathy and Meat Safety

    NASA Astrophysics Data System (ADS)

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

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

  5. The Role of the Neurointensive Care Nursery for Neonatal Encephalopathy.

    PubMed

    Glass, Hannah C; Rowitch, David H

    2016-09-01

    Neonatal encephalopathy due to intrapartum events is estimated at 1 to 2 per 1000 live births in high-income countries. Outcomes have improved over the past decade due to implementation of therapeutic hypothermia, the only clinically available neuroprotective strategy for hypoxic-ischemic encephalopathy. Neonatal encephalopathy is the most common condition treated within a neonatal neurocritical care unit. Neonates with encephalopathy benefit from a neurocritical care approach due to prevention of secondary brain injury through attention to basic physiology, earlier recognition and treatment of neurologic complications, consistent management using guidelines and protocols, and use of optimized teams at dedicated referral centers. PMID:27524453

  6. Neural crest-specific loss of Prkar1a causes perinatal lethality resulting from defects in intramembranous ossification.

    PubMed

    Jones, Georgette N; Pringle, Daphne R; Yin, Zhirong; Carlton, Michelle M; Powell, Kimerly A; Weinstein, Michael B; Toribio, Ramiro E; La Perle, Krista M D; Kirschner, Lawrence S

    2010-08-01

    The cranial neural crest (CNC) undergoes complex molecular and morphological changes during embryogenesis in order to form the vertebrate skull, and nearly three quarters of all birth defects result from defects in craniofacial development. The molecular events leading to CNC differentiation have been extensively studied; however, the role of the cAMP-dependent protein kinase [protein kinase A (PKA)] during craniofacial development has only been described in palate formation. Here, we provide evidence that strict PKA regulation in postmigratory CNC cells is essential during craniofacial bone development. Selective inactivation of Prkar1a, a regulatory subunit of the PKA holoenzyme, in the CNC results in perinatal lethality caused by dysmorphic craniofacial development and subsequent asphyxiation. Additionally, aberrant differentiation of CNC mesenchymal cells results in anomalous intramembranous ossification characterized by formation of cartilaginous islands in some areas and osteolysis of bony trabeculae with fibrous connective tissue stabilization in others. Genetic interaction studies revealed that genetic reduction of the PKA catalytic subunit C(alpha) was able to rescue the phenotype, whereas reduction in Cbeta had no effect. Overall, these observations provide evidence of the essential role of proper regulation of PKA during the ossification of the bones of the skull. This knowledge may have implications for the understanding and treatment of craniofacial birth defects. PMID:20534695

  7. De novo mutations in epileptic encephalopathies.

    PubMed

    Allen, Andrew S; Berkovic, Samuel F; Cossette, Patrick; Delanty, Norman; Dlugos, Dennis; Eichler, Evan E; Epstein, Michael P; Glauser, Tracy; Goldstein, David B; Han, Yujun; Heinzen, Erin L; Hitomi, Yuki; Howell, Katherine B; Johnson, Michael R; Kuzniecky, Ruben; Lowenstein, Daniel H; Lu, Yi-Fan; Madou, Maura R Z; Marson, Anthony G; Mefford, Heather C; Esmaeeli Nieh, Sahar; O'Brien, Terence J; Ottman, Ruth; Petrovski, Slavé; Poduri, Annapurna; Ruzzo, Elizabeth K; Scheffer, Ingrid E; Sherr, Elliott H; Yuskaitis, Christopher J; Abou-Khalil, Bassel; Alldredge, Brian K; Bautista, Jocelyn F; Berkovic, Samuel F; Boro, Alex; Cascino, Gregory D; Consalvo, Damian; Crumrine, Patricia; Devinsky, Orrin; Dlugos, Dennis; Epstein, Michael P; Fiol, Miguel; Fountain, Nathan B; French, Jacqueline; Friedman, Daniel; Geller, Eric B; Glauser, Tracy; Glynn, Simon; Haut, Sheryl R; Hayward, Jean; Helmers, Sandra L; Joshi, Sucheta; Kanner, Andres; Kirsch, Heidi E; Knowlton, Robert C; Kossoff, Eric H; Kuperman, Rachel; Kuzniecky, Ruben; Lowenstein, Daniel H; McGuire, Shannon M; Motika, Paul V; Novotny, Edward J; Ottman, Ruth; Paolicchi, Juliann M; Parent, Jack M; Park, Kristen; Poduri, Annapurna; Scheffer, Ingrid E; Shellhaas, Renée A; Sherr, Elliott H; Shih, Jerry J; Singh, Rani; Sirven, Joseph; Smith, Michael C; Sullivan, Joseph; Lin Thio, Liu; Venkat, Anu; Vining, Eileen P G; Von Allmen, Gretchen K; Weisenberg, Judith L; Widdess-Walsh, Peter; Winawer, Melodie R

    2013-09-12

    Epileptic encephalopathies are a devastating group of severe childhood epilepsy disorders for which the cause is often unknown. Here we report a screen for de novo mutations in patients with two classical epileptic encephalopathies: infantile spasms (n = 149) and Lennox-Gastaut syndrome (n = 115). We sequenced the exomes of 264 probands, and their parents, and confirmed 329 de novo mutations. A likelihood analysis showed a significant excess of de novo mutations in the ∼4,000 genes that are the most intolerant to functional genetic variation in the human population (P = 2.9 × 10(-3)). Among these are GABRB3, with de novo mutations in four patients, and ALG13, with the same de novo mutation in two patients; both genes show clear statistical evidence of association with epileptic encephalopathy. Given the relevant site-specific mutation rates, the probabilities of these outcomes occurring by chance are P = 4.1 × 10(-10) and P = 7.8 × 10(-12), respectively. Other genes with de novo mutations in this cohort include CACNA1A, CHD2, FLNA, GABRA1, GRIN1, GRIN2B, HNRNPU, IQSEC2, MTOR and NEDD4L. Finally, we show that the de novo mutations observed are enriched in specific gene sets including genes regulated by the fragile X protein (P < 10(-8)), as has been reported previously for autism spectrum disorders.

  8. Extending the KCNQ2 encephalopathy spectrum

    PubMed Central

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

    2013-01-01

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

  9. Using saccades to diagnose covert hepatic encephalopathy.

    PubMed

    Cunniffe, Nicholas; Munby, Henry; Chan, Shona; Saatci, Defne; Edison, Eric; Carpenter, R H S; Massey, Dunecan

    2015-06-01

    Covert Hepatic Encephalopathy (CHE), previously known as Minimal Hepatic Encephalopathy, is a subtle cognitive defect found in 30-70 % of cirrhosis patients. It has been linked to poor quality of life, impaired fitness to drive, and increased mortality: treatment is possible. Despite its clinical significance, diagnosis relies on psychometric tests that have proved unsuitable for use in a clinical setting. We investigated whether measurement of saccadic latency distributions might be a viable alternative. We collected data on 35 cirrhosis patients at Addenbrooke's Hospital, Cambridge, with no evidence of clinically overt encephalopathy, and 36 age-matched healthy controls. Performance on standard psychometric tests was evaluated to determine those patients with CHE as defined by the World Congress of Gastroenterology. We then compared visually-evoked saccades between those with CHE and those without, as well as reviewing blood test results and correlating saccadic latencies with biochemical parameters and prognostic scores. Cirrhosis patients have significantly longer median saccadic latencies than healthy controls. Those with CHE had significantly prolonged saccadic latencies when compared with those without CHE. Analysis of a cirrhosis patient's saccades can diagnose CHE with a sensitivity of 75 % and a specificity of 75 %. We concluded that analysis of a cirrhosis patient's saccadic latency distributions is a fast and objective measure that can be used as a diagnostic tool for CHE. This improved early diagnosis could direct avoidance of high-risk activities such as driving, and better inform treatment strategies. PMID:25586511

  10. Perinatal arrhythmias -- diagnosis and treatment.

    PubMed

    Moura, Cláudia; Vieira, António; Guimarães, Hercília; Areias, José Carlos

    2002-01-01

    We did a retrospective analysis of the clinical files of 26 neonates with arrhythmia born during the period between January 1994 and February 1999. Fourteen (53.8%) of the neonates were male and 16 (61.5%) had prenatal diagnosis. Four (15.3%) had associated congenital heart disease. Twenty-one (80.7%) had abnormal rhythm and five (19.2%) had cardiac conduction disorder. Digoxin was the therapy of first choice to restore normal rhythm, used in 66.6% of the patients, followed by adenosine (16.6%) and electrical cardioversion (16.6%). An epicardial pacemaker was used in two of the three neonates with complete atrioventricular (AV) block. One neonate died due to cerebral hemorrhage. All the neonates were discharged in a clinically stable condition and 16 (88.8%) of them were medicated with digoxin. In a follow-up period that varied from 0 to 71 months (mean of 30.8 months), two patients had an episode of supraventricular tachycardia (SVT) after treatment withdrawal. Perinatal arrhythmias, although uncommon, can be life-threatening, and hence we consider our experience with these situations worth presenting.

  11. Hurricane Katrina and perinatal health.

    PubMed

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

    2009-12-01

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

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

    PubMed

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

    2015-05-01

    We report 3 cases of suprachoroidal hemorrhage (SCH) found to be triggered by increased intrathoracic pressure and detected using postmortem computed tomography (PMCT). Case 1 was a man aged in his 50s who was found dead at a landslide site. The autopsy showed clogging of the upper respiratory tract with soil debris from the landslide. The cause of death was determined to be asphyxia. PMCT showed SCH in both eyes, which was believed to be caused by chest compression or choking on the soil debris from the landslide. Case 2 was a woman aged in her 60s who was found dead in the sea. The autopsy revealed injuries primarily to her chest. We concluded that the cause of death was drowning. PMCT showed SCH in her right eye that was believed to be caused by chest compression. Case 3 was a woman aged in her 80s who was buried in a snowdrift and potentially died from hypothermia. PMCT showed SCH in both eyes, which was considered to be from an increase in intrathoracic pressure that might have been caused by the burial in the snow. Histological findings showed serous retinal detachment associated with retinal pigment epithelium damage due to SCH, which indicated that she was alive for several hours after the onset of SCH. The increase in intrathoracic pressure caused by dyspnea or chest compression was considered responsible for the onset of SCH in all of the present cases. PMCT might assist with the differential diagnosis of traumatic asphyxiation by SCH. PMID:25533924

  13. DYNC2H1 mutations cause asphyxiating thoracic dystrophy and short rib-polydactyly syndrome, type III.

    PubMed

    Dagoneau, Nathalie; Goulet, Marie; Geneviève, David; Sznajer, Yves; Martinovic, Jelena; Smithson, Sarah; Huber, Céline; Baujat, Geneviève; Flori, Elisabeth; Tecco, Laura; Cavalcanti, Denise; Delezoide, Anne-Lise; Serre, Valérie; Le Merrer, Martine; Munnich, Arnold; Cormier-Daire, Valérie

    2009-05-01

    Jeune asphyxiating thoracic dystrophy (ATD) is an autosomal-recessive chondrodysplasia characterized by short ribs and a narrow thorax, short long bones, inconstant polydactyly, and trident acetabular roof. ATD is closely related to the short rib polydactyly syndrome (SRP) type III, which is a more severe condition characterized by early prenatal expression and lethality and variable malformations. We first excluded IFT80 in a series of 26 fetuses and children belonging to 14 families diagnosed with either ATD or SRP type III. Studying a consanguineous family from Morocco, we mapped an ATD gene to chromosome 11q14.3-q23.1 in a 20.4 Mb region and identified homozygous mutations in the cytoplasmic dynein 2 heavy chain 1 (DYNC2H1) gene in the affected children. Compound heterozygosity for DYNC2H1 mutations was also identified in four additional families. Among the five families, 3/5 were diagnosed with ATD and 2/5 included pregnancies terminated for SRP type III. DYNC2H1 is a component of a cytoplasmic dynein complex and is directly involved in the generation and maintenance of cilia. From this study, we conclude that ATD and SRP type III are variants of a single disorder belonging to the ciliopathy group.

  14. Mutations in CSPP1 Cause Primary Cilia Abnormalities and Joubert Syndrome with or without Jeune Asphyxiating Thoracic Dystrophy

    PubMed Central

    Tuz, Karina; Bachmann-Gagescu, Ruxandra; O’Day, Diana R.; Hua, Kiet; Isabella, Christine R.; Phelps, Ian G.; Stolarski, Allan E.; O’Roak, Brian J.; Dempsey, Jennifer C.; Lourenco, Charles; Alswaid, Abdulrahman; Bönnemann, Carsten G.; Medne, Livija; Nampoothiri, Sheela; Stark, Zornitza; Leventer, Richard J.; Topçu, Meral; Cansu, Ali; Jagadeesh, Sujatha; Done, Stephen; Ishak, Gisele E.; Glass, Ian A.; Shendure, Jay; Neuhauss, Stephan C.F.; Haldeman-Englert, Chad R.; Doherty, Dan; Ferland, Russell J.

    2014-01-01

    Joubert syndrome (JBTS) is a recessive ciliopathy in which a subset of affected individuals also have the skeletal dysplasia Jeune asphyxiating thoracic dystrophy (JATD). Here, we have identified biallelic truncating CSPP1 (centrosome and spindle pole associated protein 1) mutations in 19 JBTS-affected individuals, four of whom also have features of JATD. CSPP1 mutations explain ∼5% of JBTS in our cohort, and despite truncating mutations in all affected individuals, the range of phenotypic severity is broad. Morpholino knockdown of cspp1 in zebrafish caused phenotypes reported in other zebrafish models of JBTS (curved body shape, pronephric cysts, and cerebellar abnormalities) and reduced ciliary localization of Arl13b, further supporting loss of CSPP1 function as a cause of JBTS. Fibroblasts from affected individuals with CSPP1 mutations showed reduced numbers of primary cilia and/or short primary cilia, as well as reduced axonemal localization of ciliary proteins ARL13B and adenylyl cyclase III. In summary, CSPP1 mutations are a major cause of the Joubert-Jeune phenotype in humans; however, the mechanism by which these mutations lead to both JBTS and JATD remains unknown. PMID:24360808

  15. End-tidal CO₂ detection of an audible heart rate during neonatal cardiopulmonary resuscitation after asystole in asphyxiated piglets.

    PubMed

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

    2011-05-01

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

  16. Substance use in the perinatal period

    PubMed Central

    Forray, Ariadna; Foster, Dawn

    2015-01-01

    Perinatal substance use remains a major public health problem and is associated with a number of deleterious maternal and fetal effects. Polysubstance use in pregnancy is common, and can potentiate adverse maternal and fetal outcomes. Tobacco is the most commonly used substance in pregnancy, followed by alcohol and illicit substances. The treatments for perinatal substance use are limited and consist mostly of behavioral and psychosocial interventions. Of these contingency management has shown the most efficacy. More recently, novel interventions such as progesterone for postpartum cocaine use have shown promise. The purpose of this review is to examine the recent literature on the use of tobacco, alcohol, cannabis, stimulants, and opioids in the perinatal period, their effects on maternal and fetal health and current treatments. PMID:26386836

  17. Substance Use in the Perinatal Period.

    PubMed

    Forray, Ariadna; Foster, Dawn

    2015-11-01

    Perinatal substance use remains a major public health problem and is associated with a number of deleterious maternal and fetal effects. Polysubstance use in pregnancy is common and can potentiate adverse maternal and fetal outcomes. Tobacco is the most commonly used substance in pregnancy, followed by alcohol and illicit substances. The treatments for perinatal substance use are limited and consist mostly of behavioral and psychosocial interventions. Of these, contingency management has shown the most efficacy. More recently, novel interventions such as progesterone for postpartum cocaine use have shown promise. The purpose of this review is to examine the recent literature on the use of tobacco, alcohol, cannabis, stimulants, and opioids in the perinatal period, their effects on maternal and fetal health, and current treatments. PMID:26386836

  18. Comparison of Transmissible Mink Encephalopathy Isolates in Raccoons

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  19. Evaluation of the zoonotic potential of transmissible mink encephalopathy

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  20. Typical and atypical cases of bovine spongiform encephalopathy

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  1. The Spectrum of Disease in Chronic Traumatic Encephalopathy

    ERIC Educational Resources Information Center

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

    2013-01-01

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

  2. Perinatal care: the Kuranko context of choice.

    PubMed

    Ross, J S

    1991-01-01

    The ethnomedical aspects of childbirth and factors that influence Kuranko women in the northern Sierra Leone center of Kabala (a multiethnic town of some 15,000 inhabitants) in making decisions regarding perinatal care are the focus of this article. I found that Kuranko women develop perinatal care strategies from services available in the formal and informal health-care sectors. The discussion is situated within a context that values primary health care and health promotion and advocates the use of these frameworks in ways that are informed by Kuranko social organization and concepts of development.

  3. Hemophagocytic lymphohistiocytosis: A rare cause of recurrent encephalopathy

    PubMed Central

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

    2016-01-01

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

  4. Hemophagocytic lymphohistiocytosis: A rare cause of recurrent encephalopathy.

    PubMed

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

    2016-08-01

    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

  5. Hemophagocytic lymphohistiocytosis: A rare cause of recurrent encephalopathy.

    PubMed

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

    2016-08-01

    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.

  6. Hemophagocytic lymphohistiocytosis: A rare cause of recurrent encephalopathy

    PubMed Central

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

    2016-01-01

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

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

    PubMed

    Shbarou, Rolla; Mikati, Mohamad A

    2016-05-01

    Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various encephalopathies that are reviewed in this article include the following: early infantile epileptic encephalopathy or Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, severe myoclonic epilepsy in infancy (Dravet syndrome), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epileptic encephalopathy with continuous spike-and-wave during sleep. Their clinical features, prognosis as well as underlying genetic etiologies are presented and updated. PMID:27544470

  8. Chronic Traumatic Encephalopathy and Movement Disorders: Update.

    PubMed

    Tarazi, Apameh; Tator, Charles H; Tartaglia, Maria Carmela

    2016-05-01

    Association of repetitive brain trauma with progressive neurological deterioration has been described since the 1920s. Punch drunk syndrome and dementia pugilistica (DP) were introduced first to explain symptoms in boxers, and more recently, chronic traumatic encephalopathy (CTE) has been used to describe a neurodegenerative disease in athletes and military personal with a history of multiple concussions. Although there are many similarities between DP and CTE, a number of key differences are apparent especially when comparing movement impairments. The aim of this review is to compare clinical and pathological aspects of DP and CTE with a focus on disorders of movement. PMID:27021775

  9. Repetitive Head Impacts and Chronic Traumatic Encephalopathy.

    PubMed

    McKee, Ann C; Alosco, Michael L; Huber, Bertrand R

    2016-10-01

    Chronic traumatic encephalopathy (CTE) is a distinctive neurodegenerative disease that occurs as a result of repetitive head impacts. CTE can only be diagnosed by postmortem neuropathologic examination of brain tissue. CTE is a unique disorder with a pathognomonic lesion that can be reliably distinguished from other neurodegenerative diseases. CTE is associated with violent behaviors, explosivity, loss of control, depression, suicide, memory loss and cognitive changes. There is increasing evidence that CTE affects amateur athletes as well as professional athletes and military veterans. CTE has become a major public health concern.

  10. [Star fruit (Averrhoa carambola) toxic encephalopathy].

    PubMed

    Signaté, A; Olindo, S; Chausson, N; Cassinoto, C; Edimo Nana, M; Saint Vil, M; Cabre, P; Smadja, D

    2009-03-01

    Ingestion of star fruit (Averrhoa carambola) can induce severe intoxication in subjects with chronic renal failure. Oxalate plays a key role in the neurotoxicity of star fruit. We report the cases of two patients with unknown chronic renal insufficiency who developed severe encephalopathy after ingestion of star fruit. The two patients developed intractable hiccups, vomiting, impaired consciousness and status epilepticus. Diffusion-weighted MR imaging showed cortical and thalamic hyperintense lesions related to epileptic status. They improved after being submitted to continuous hemofiltration which constitutes the most effective treatment during the acute phase.

  11. Repetitive Head Impacts and Chronic Traumatic Encephalopathy.

    PubMed

    McKee, Ann C; Alosco, Michael L; Huber, Bertrand R

    2016-10-01

    Chronic traumatic encephalopathy (CTE) is a distinctive neurodegenerative disease that occurs as a result of repetitive head impacts. CTE can only be diagnosed by postmortem neuropathologic examination of brain tissue. CTE is a unique disorder with a pathognomonic lesion that can be reliably distinguished from other neurodegenerative diseases. CTE is associated with violent behaviors, explosivity, loss of control, depression, suicide, memory loss and cognitive changes. There is increasing evidence that CTE affects amateur athletes as well as professional athletes and military veterans. CTE has become a major public health concern. PMID:27637402

  12. Correlation of EEG, CT, and MRI Brain with Neurological Outcome at 12 Months in Term Newborns with Hypoxic Ischemic Encephalopathy

    PubMed Central

    Jose, Annu; Matthai, John; Paul, Sarah

    2013-01-01

    Objective: To correlate electroencephalogram (EEG), computed tomography (CT), and magnetic resonance imaging (MRI) brain with neurological outcome at 12 months in term neonates with hypoxic ischemic encephalopathy. Design: Prospective observational study. Setting: Neonatal intensive care unit (NICU) in a tertiary care teaching hospital. Materials and Methods: The study was conducted between June 2010 and November 2011. Consecutive term neonates with perinatal asphyxia and hypoxic ischemic encephalopathy were the subjects. All babies were managed as per standard protocol. EEG was done as soon as the baby was stable and CT brain within 7 days. MRI was done at 3 months. Neurodevelpmental assessment was done at 12 months. Results: Of the 31 babies, four died and one was lost to follow-up. Neurodevelopmental at 12 months of age was normal in 15 babies. EEG was normal in six babies and all of them had a normal neurodevelopment. Thirteen of the 14 babies with burst suppression pattern were abnormal (P<0.001). CT brain was normal in 14 and all of them had normal neurodevelopment (P<0.001), while 11 of the 12 with cerebral edema had abnormal outcome (P<0.001). Of the 16 babies with normal MRI, 14 were normal, while all six babies with abnormal signals in the cortex and thalamus had abnormal outcome (P=0.002). Conclusions: A normal EEG and CT brain in a term newborn with hypoxic ischemic encephalopathy (HIE) is associated with good neurological outcome. Burst suppression pattern in EEG, bleeds, or hypodensities in the CT and involvement of basal ganglia/thalamus in the MRI are predictors of abnormal outcome. PMID:24251256

  13. Global and cultural perinatal nursing research: improving clinical practice.

    PubMed

    Callister, Lynn Clark

    2011-01-01

    High-quality perinatal nursing care should be based on the best evidence including research findings, clinical expertise, and the preferences of women and their families. Principles of perinatal research initiatives are defined, with suggested research priorities designed to close current gaps in the micro and macro environments of perinatal nursing throughout the world. Nearly a decade ago, the following question was asked, "Where is the 'E' (evidence) in maternal child health?" Improving the quality and safety of perinatal nursing care for culturally diverse women globally is the primary goal of nurse researchers leading the future of perinatal healthcare.

  14. Perinatal home care: one entrepreneur's experience.

    PubMed

    Eaton, D G

    1994-10-01

    Nurses have responded to the entrepreneurial movement by entering into various nontraditional roles and starting their own businesses. This article describes the author's experience in establishing a perinatal home-care business. The characteristics of women and nurse entrepreneurs are discussed, as are the components of a business plan and how to manage a business.

  15. DRINKING WATER ARSENIC AND PERINATAL OUTCOMES

    EPA Science Inventory

    Drinking Water Arsenic and Perinatal Outcomes
    DT Lobdell, Z Ning, RK Kwok, JL Mumford, ZY Liu, P Mendola

    Many studies have documented an association between drinking water arsenic (DWA) and cancer, vascular diseases, and dermatological outcomes, but few have investigate...

  16. Perinatal mortality--an analysis of causes and strategies.

    PubMed

    Gupta, Neeru

    2011-04-01

    Perinatal mortality is the most sensitive index while imparting healthcare to mother during pregnancy and delivery and also to the baby in perinatal period. Perinatal mortality is higher in rural areas than in urban areas. Worldover perinatal or infant mortality rate is on decline. Developed countries are ahead of developing nations in giving good antenatal, intrapartal as well as neonatal care. Factors responsible for perinatal mortality in Indian context lie in sociodemographic, maternal and foetal aspects. Regional differences also are there in India while assessing perinatal mortality and delivery practices. The lacunae are to be identified while recommending strategies to be taken to lower the perinatal mortality. A community based data system should be developed so that the information should flow from down to above, from village to subcentre to primary health centre and further from district to state. Some newborns need special care. Since newborns need early recognition of danger signs and prompt treatment measures. PMID:22187796

  17. Is there an association between female circumcision and perinatal death?

    PubMed Central

    Essen, Birgitta; Bodker, Birgit; Sjoberg, N-O; Gudmundsson, Saemundur; Ostergren, P-O; Langhoff-Roos, Jens

    2002-01-01

    OBJECTIVE: In Sweden, a country with high standards of obstetric care, the high rate of perinatal mortality among children of immigrant women from the Horn of Africa raises the question of whether there is an association between female circumcision and perinatal death. METHOD: To investigate this, we examined a cohort of 63 perinatal deaths of infants born in Sweden over the period 1990-96 to circumcised women. FINDINGS: We found no evidence that female circumcision was related to perinatal death. Obstructed or prolonged labour, caused by scar tissue from circumcision, was not found to have any impact on the number of perinatal deaths. CONCLUSION: The results do not support previous conclusions that genital circumcision is related to perinatal death, regardless of other circumstances, and suggest that other, suboptimal factors contribute to perinatal death among circumcised migrant women. PMID:12219153

  18. Management options for minimal hepatic encephalopathy.

    PubMed

    Bajaj, Jasmohan S

    2008-12-01

    Minimal hepatic encephalopathy (MHE) is a neurocognitive dysfunction that is present in the majority of patients with cirrhosis. MHE has a characteristic cognitive profile that cannot be diagnosed clinically. This cognitive dysfunction is independent of sleep dysfunction or problems with overall intelligence. MHE has a significant impact on quality of life, the ability to function in daily life and progression to overt hepatic encephalopathy. Driving ability can be impaired in MHE and this may be a significant factor behind motor vehicle accidents. A crucial aspect of the clinical care of MHE patients is their driving history, which is often ignored during routine care and can add a vital dimension to the overall disease assessment. Driving history should be an integral part of the care of patients with MHE. The preserved communication skills and lack of specific signs and insight make MHE difficult to diagnose. The predominant strategies for MHE diagnosis are psychometric or neurophysiological testing. These are usually limited by financial, normative or time constraints. Studies into inhibitory control, cognitive drug research and critical flicker frequency tests are encouraging. These tests do not require a psychologist for administration and interpretation. Lactulose and probiotics have been studied for their potential use as therapies for MHE, but these are not standard-of-care practices at this time. Therapy can improve the quality of life in MHE patients but the natural history, specific diagnostic strategies and treatment options are still being investigated. PMID:19090738

  19. Clinical presentation of chronic traumatic encephalopathy

    PubMed Central

    Daneshvar, Daniel H.; Baugh, Christine M.; Seichepine, Daniel R.; Montenigro, Philip H.; Riley, David O.; Fritts, Nathan G.; Stamm, Julie M.; Robbins, Clifford A.; McHale, Lisa; Simkin, Irene; Stein, Thor D.; Alvarez, Victor E.; Goldstein, Lee E.; Budson, Andrew E.; Kowall, Neil W.; Nowinski, Christopher J.; Cantu, Robert C.; McKee, Ann C.

    2013-01-01

    Objective: The goal of this study was to examine the clinical presentation of chronic traumatic encephalopathy (CTE) in neuropathologically confirmed cases. Methods: Thirty-six adult male subjects were selected from all cases of neuropathologically confirmed CTE at the Boston University Center for the Study of Traumatic Encephalopathy brain bank. Subjects were all athletes, had no comorbid neurodegenerative or motor neuron disease, and had next-of-kin informants to provide retrospective reports of the subjects' histories and clinical presentations. These interviews were conducted blind to the subjects' neuropathologic findings. Results: A triad of cognitive, behavioral, and mood impairments was common overall, with cognitive deficits reported for almost all subjects. Three subjects were asymptomatic at the time of death. Consistent with earlier case reports of boxers, 2 relatively distinct clinical presentations emerged, with one group whose initial features developed at a younger age and involved behavioral and/or mood disturbance (n = 22), and another group whose initial presentation developed at an older age and involved cognitive impairment (n = 11). Conclusions: This suggests there are 2 major clinical presentations of CTE, one a behavior/mood variant and the other a cognitive variant. PMID:23966253

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

    PubMed Central

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

    2015-01-01

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

  1. Strategies to reduce perinatal and neonatal mortality.

    PubMed

    Singh, M; Paul, V K

    1988-06-01

    The perinatal mortality rate in India averages 66.3/1000 live births. 60% of all infant deaths occur during the 1st month, making the neonatal mortality rate 76/1000 in rural areas and 39/1000 in urban areas. These rates have remained static since 1974. Over 90% of all deliveries occur at home and are conducted by untrained birth attendants. The major causes of perinatal deaths are immaturity/low birth weight, birth asphyxia/trauma, neonatal infections, and congenital malformations. Neonatal tetanus alone is responsible for 230,000-280,000 deaths a year. Hypoxia, low birth weight, and tetanus are preventable, if primary perinatal care is provided and high-risk pregnancies are recognized and referred to facilities where fetal monitoring and neonatal care are available. It is proposed to train all of the country's 5 million traditional birth attendants by 1990 to deliver primary perinatal care. By 1990 also there will be 1 village health guide for every 1000 people. All traditional birth attendants must know how to give mouth-to-mouth resuscitation, and the infrastructure for an adequate referral system must be established. In order to reduce the incidence of low birth weight, the Integrated Child Development Service Scheme proposes that all pregnant women receive a dietary supplement of 500 calories and 25 gm protein, and that pregnant women be given a 2-hour midday rest period. The control of malaria and intestinal infections with chloroquine and antibiotics would do much to reduce low birth weight. Simple technologies for measuring birth weight indicators, such as chest circumference or mid-arm circumference, require only a tape measure. Finally, technics of mass communication must be utilized to spread knowledge of perinatal and neonatal care. PMID:3069742

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

    SciTech Connect

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

    1995-01-02

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

  3. Dengue viral infections as a cause of encephalopathy.

    PubMed

    Malavige, G N; Ranatunga, P K; Jayaratne, S D; Wijesiriwardana, B; Seneviratne, S L; Karunatilaka, D H

    2007-04-01

    The aim of this study was to determine the clinical characteristics and poor prognostic factors associated with high mortality in dengue encephalopathy. Fifteen patients with confirmed dengue infections, who developed encephalopathy, were recruited from two tertiary care hospitals in Colombo, Sri Lanka. Among the factors that contributed to encephalopathy were: Acute liver failure (73%), electrolyte imbalances (80%) and shock (40%). Five (33.3%) patients developed seizures. Disseminated intravascular coagulation was seen in five (33.3%). Secondary bacterial infections were observed in 8 (53.3%) of our patients. The overall mortality rate was 47%.

  4. Hepatic Encephalopathy-the Old and the New.

    PubMed

    Kandiah, Prem A; Kumar, Gagan

    2016-07-01

    Hepatic encephalopathy occurs ubiquitously in all causes of advanced liver failure, however, its implications on mortality diverge and vary depending upon acuity and severity of liver failure. This associated mortality has decreased in subsets of liver failure over the last 20 years. Aside from liver transplantation, this improvement is not attributable to a single intervention but likely to a combination of practical advances in critical care management. Misconceptions surrounding many facets of hepatic encephalopathy exists due to heterogeneity in presentation, pathophysiology and outcome. This review is intended to highlight the important concepts, rationales and strategies for managing hepatic encephalopathy.

  5. Severe early onset ethylmalonic encephalopathy with West syndrome.

    PubMed

    Papetti, Laura; Garone, Giacomo; Schettini, Livia; Giordano, Carla; Nicita, Francesco; Papoff, Paola; Zeviani, Massimo; Leuzzi, Vincenzo; Spalice, Alberto

    2015-12-01

    Ethylmalonic encephalopathy (EE) is a rare autosomal recessive disorder characterized by early onset encephalopathy, chronic diarrhoea, petechiae, orthostatic acrocyanosis and defective cytochrome c oxidase (COX) in muscle and brain. High levels of lactic, ethylmalonic and methylsuccinic acids are detected in body fluids. EE is caused by mutations in ETHE1 gene, a mitochondrial sulfur dioxygenase. Neurologic signs and symptoms include progressively delayed development, hypotonia, seizures, and abnormal movements. We report on the clinical, electroencephalographic and MRI findings of a baby with a severe early onset encephalopathy associated with novel ETHE1 gene mutation. This is the first case described in literature with an early pure epileptic onset, presenting with West syndrome.

  6. Legal Responsibilities of Physicians When They Diagnose Hepatic Encephalopathy.

    PubMed

    Vierling, John M

    2015-08-01

    Both covert hepatic encephalopathy (CHE) and overt hepatic encephalopathy (OHE) impair the ability to operate machinery. The legal responsibilities of US physicians who diagnose and treat patients with hepatic encephalopathy vary among states. It is imperative that physicians know the laws regarding reporting in their state. OHE represents a neuropsychiatric impairment that meets general reporting criteria. The medical advisory boards of the states have not identified OHE as a reportable condition. In the absence of validated diagnostic guidelines, physicians are not obligated to perform tests for CHE. There is a need for explicit guidance from professional associations regarding this issue.

  7. Spiroplasma spp. from transmissible spongiform encephalopathy brains or ticks induce spongiform encephalopathy in ruminants.

    PubMed

    Bastian, Frank O; Sanders, Dearl E; Forbes, Will A; Hagius, Sue D; Walker, Joel V; Henk, William G; Enright, Fred M; Elzer, Philip H

    2007-09-01

    Spiroplasma, small motile wall-less bacteria, are linked by molecular and serological studies to the transmissible spongiform encephalopathies (TSEs), which include scrapie in sheep, chronic wasting disease (CWD) in deer and Creutzfeldt-Jakob disease in humans. In this study, two experiments were undertaken to determine the role of spiroplasma in the pathogenesis of TSE. In experiment 1, Spiroplasma mirum, a rabbit tick isolate that had previously been shown to experimentally induce spongiform encephalopathy in rodents, was inoculated intracranially (IC) into ruminants. S. mirum-inoculated deer manifested clinical signs of TSE after 1.5 to 5.5 months incubation. The deer, as well as sheep and goats, inoculated with S. mirum developed spongiform encephalopathy in a dose-dependent manner. In experiment 2, spiroplasma closely related to S. mirum were isolated from TSE-affected brains via passage in embryonated eggs, and propagated in cell-free M1D media. Spiroplasma spp. isolates from scrapie-affected sheep brain and from CWD-affected deer brain inoculated IC into sheep and goats induced spongiform encephalopathy closely resembling natural TSE in these animals. These data show spiroplasma to be consistently associated with TSE, and able experimentally to cause TSE in ruminant animal models, therein questioning the validity of studies that have concluded the prion, a miss-folded protease-resistant protein that builds up in TSE brains during the course of the disease, to be the sole causal agent. The spiroplasma infection models reported here will be important for investigating factors involved in the pathogenesis of TSE since ruminants are the natural hosts.

  8. Hypoxic ischemic encephalopathy in a case of intranuclear rod myopathy without any prenatal sentinel event.

    PubMed

    Kawase, Koya; Nishino, Ichizo; Sugimoto, Mari; Kouwaki, Masanori; Koyama, Norihisa; Yokochi, Kenji

    2015-02-01

    Intranuclear rod myopathy (IRM), a variant of nemaline myopathy, is characterized by the presence of nemaline bodies in myonuclei. We report a case of IRM presenting with hypoxic ischemic encephalopathy (HIE). There were no prenatal complications caused by fetal brain injury. Although no nemaline bodies were observed in the cytoplasm, intranuclear rods were observed in some fibers under light and electron microscopy. Molecular analysis identified a heterozygous variant, c.449C>T (p.Thr150Ile), in ACTA1. On magnetic resonance imaging at 9days of age, injuries to the basal ganglia, thalamus, and brainstem consistent with perinatal HIE were seen. Respiratory insufficiency at birth was strongly suspected to be the cause of HIE. Our case highlights that a patient with a congenital neuromuscular disorder who presents with severe respiratory dysfunction requiring substantial resuscitative efforts at birth can be complicated by HIE without any prenatal sentinel event. Prenatal detection of neuromuscular disorders, careful management of delivery, and neonatal resuscitation and adequate respiratory management are important in preventing irreversible brain injury in these patients. PMID:24787270

  9. Management of hepatic encephalopathy in the hospital.

    PubMed

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

    2014-02-01

    Hepatic encephalopathy (HE) develops in up to 50% of patients with cirrhosis and is a feature of decompensated cirrhosis. With the goal of reviewing the evidence for treatment and prevention of overt hepatic encephalopathy, pubmed was searched using search terms hepatic encephalopathy AND treatment, limited to human studies from January 1, 2003, through December 1, 2013, and supplemented by key references. The inpatient incidence of HE is approximately 23,000 annually, and management of these patients is common for internists and subspecialists. Treatment of the hospitalized patient with HE has changed in recent years. Treatment entails 2 phases: induction and maintenance of remission. Most cases of significant HE are precipitated by infection, gastrointestinal bleeding, medications, or other culprits. All patients should be evaluated for secondary triggers of HE, and treatment should be initiated with a nonabsorbable disaccharide (ie, lactulose) in most patients. Rifaximin (off label) can be added in patients not responding to lactulose. Neomycin is a less preferred alternative to rifaximin owing to its adverse effect profile. Other therapies, including zinc, L-ornithine-L-aspartate, and branched-chain amino acids, can be considered for patients not responding to disaccharides and nonabsorbable antibiotics. Large portosystemic shunts may be embolized in patients with medically refractory recurrent or severe HE with otherwise well-compensated cirrhosis. Molecular Adsorbent Recirculating System is now available for patients with severe HE who do not respond to medical therapy. It is critically important that patients hospitalized with significant HE continue maintenance therapy at the time of dismissal to prevent further episodes. Patients with a first-time episode of HE can be administered lactulose, and careful instructions should be provided to patients and caregivers about dose titration to achieve 3 bowel movements daily. Patients with recurrent HE episodes

  10. Chronic Traumatic Encephalopathy: The Impact on Athletes.

    PubMed

    Galgano, Michael A; Cantu, Robert; Chin, Lawrence S

    2016-01-01

    Chronic traumatic encephalopathy (CTE) is a devastating neuropsychological condition afflicting a small percentage of athletes partaking in high-impact sports. The onset of symptoms lags years behind the inciting events. Repetitive minor head injuries are felt to be the main etiology behind CTE. Routine radiographic imaging generally is unremarkable in cases of CTE. Functional magnetic resonance imaging (fMRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) are advanced MRI-based sequences that have shown promise in detecting early radiographic findings that may be reflective of CTE. Progressive neuronal loss is the histopathological hallmark of this neurodegenerative disease. Strategizing earlier detection techniques is paramount in delivering optimal care to athletes afflicted with CTE. PMID:27088064

  11. Nonconvulsive status epilepticus disguising as hepatic encephalopathy.

    PubMed

    Jo, Yong Min; Lee, Sung Wook; Han, Sang Young; Baek, Yang Hyun; Ahn, Ji Hye; Choi, Won Jong; Lee, Ji Young; Kim, Sang Ho; Yoon, Byeol A

    2015-04-28

    Nonconvulsive status epilepticus has become an important issue in modern neurology and epileptology. This is based on difficulty in definitively elucidating the condition and its various clinical phenomena and on our inadequate insight into the intrinsic pathophysiological processes. Despite nonconvulsive status epilepticus being a situation that requires immediate treatment, this disorder may not be appreciated as the cause of mental status impairment. Although the pathophysiology of nonconvulsive status epilepticus remains unknown, this disorder is thought to lead to neuronal damage, so its identification and treatment are important. Nonconvulsive status epilepticus should be considered in the differential diagnosis of patients with liver cirrhosis presenting an altered mental status. We report a case of a 52-year-old male with liver cirrhosis presenting an altered mental status. He was initially diagnosed with hepatic encephalopathy but ultimately diagnosed with nonconvulsive status epilepticus by electroencephalogram.

  12. Does this patient have hypertensive encephalopathy?

    PubMed

    Christopoulou, Foteini; Rizos, Evangelos C; Kosta, Paraskevi; Argyropoulou, Maria I; Elisaf, Moses

    2016-05-01

    A 63-year-old man was admitted to our hospital for further investigation and management of brain metastases. The patient was initially presented with a 4-day history of confusion. On the day of admission, the patient was confused, agitated, disorientated in place and time, and had visual disturbances. His blood pressure was repeatedly recorded high, with levels of systolic blood pressure between 170-210 mm Hg. A brain magnetic resonance imaging showed areas of high signal on T2 and fluid-attenuated inversion recovery images, located bilaterally in the white matter of the occipital regions and unilateral in the left frontal lobe, suggestive of posterior reversible encephalopathy syndrome. Aggressive treatment of hypertension resulted in complete resolution of the clinical and radiologic features of the syndrome. PMID:26896240

  13. Neuroprotective Strategies after Neonatal Hypoxic Ischemic Encephalopathy

    PubMed Central

    Dixon, Brandon J.; Reis, Cesar; Ho, Wing Mann; Tang, Jiping; Zhang, John H.

    2015-01-01

    Neonatal hypoxic ischemic encephalopathy (HIE) is a devastating disease that primarily causes neuronal and white matter injury and is among the leading cause of death among infants. Currently there are no well-established treatments; thus, it is important to understand the pathophysiology of the disease and elucidate complications that are creating a gap between basic science and clinical translation. In the development of neuroprotective strategies and translation of experimental results in HIE, there are many limitations and challenges to master based on an appropriate study design, drug delivery properties, dosage, and use in neonates. We will identify understudied targets after HIE, as well as neuroprotective molecules that bring hope to future treatments such as melatonin, topiramate, xenon, interferon-beta, stem cell transplantation. This review will also discuss some of the most recent trials being conducted in the clinical setting and evaluate what directions are needed in the future. PMID:26389893

  14. Pathogenesis, Diagnosis, and Treatment of Hepatic Encephalopathy

    PubMed Central

    Atluri, Dileep K; Prakash, Ravi; Mullen, Kevin D

    2011-01-01

    Hepatic encephalopathy (HE) is a neuropsychiatric disorder seen in patients with advanced liver disease or porto-systemic shunts. Based on etiology and severity of HE, the World Congress of Gastroenterology has divided HE into categories and sub-categories. Many user-friendly computer-based neuropsychiatric tests are being validated for diagnosing covert HE. Currently, emphasis is being given to view HE deficits as a continuous spectrum rather than distinct stages. Ammonia is believed to play crucial role in pathogenesis of HE via astrocyte swelling and cerebral edema. However, evidence has been building up which supports the synergistic role of oxidative stress, inflammation and neurosteroids in pathogenesis of HE. At present, treatment of HE aims at decreasing the production and intestinal absorption of ammonia. But as the role of new pathogenetic mechanisms becomes clear, many potential new treatment strategies may become available for clinician. PMID:25755319

  15. Chronic traumatic encephalopathy and other neurodegenerative proteinopathies.

    PubMed

    Tartaglia, Maria Carmela; Hazrati, Lili-Naz; Davis, Karen D; Green, Robin E A; Wennberg, Richard; Mikulis, David; Ezerins, Leo J; Keightley, Michelle; Tator, Charles

    2014-01-01

    "Chronic traumatic encephalopathy" (CTE) is described as a slowly progressive neurodegenerative disease believed to result from multiple concussions. Traditionally, concussions were considered benign events and although most people recover fully, about 10% develop a post-concussive syndrome with persisting neurological, cognitive and neuropsychiatric symptoms. CTE was once thought to be unique to boxers, but it has now been observed in many different athletes having suffered multiple concussions as well as in military personal after repeated blast injuries. Much remains unknown about the development of CTE but its pathological substrate is usually tau, similar to that seen in Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD). The aim of this "perspective" is to compare and contrast clinical and pathological CTE with the other neurodegenerative proteinopathies and highlight that there is an urgent need for understanding the relationship between concussion and the development of CTE as it may provide a window into the development of a proteinopathy and thus new avenues for treatment.

  16. Chronic Traumatic Encephalopathy: The Impact on Athletes

    PubMed Central

    Cantu, Robert; Chin, Lawrence S.

    2016-01-01

    Chronic traumatic encephalopathy (CTE) is a devastating neuropsychological condition afflicting a small percentage of athletes partaking in high-impact sports. The onset of symptoms lags years behind the inciting events. Repetitive minor head injuries are felt to be the main etiology behind CTE. Routine radiographic imaging generally is unremarkable in cases of CTE. Functional magnetic resonance imaging (fMRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) are advanced MRI-based sequences that have shown promise in detecting early radiographic findings that may be reflective of CTE. Progressive neuronal loss is the histopathological hallmark of this neurodegenerative disease. Strategizing earlier detection techniques is paramount in delivering optimal care to athletes afflicted with CTE. PMID:27088064

  17. Leucine metabolism in patients with Hepatic Encephalopathy

    SciTech Connect

    McGhee, A.S.; Kassouny, M.E.; Matthews, D.E.; Millikan, W.

    1986-03-01

    A primed continuous infusion of (/sup 15/N, 1-/sup 13/C)leucine was used to determine whether increased oxidation and/or protein synthesis of leucine occurs in patients with cirrhosis. Five controls and patients were equilibrated on a metabolic balance diet (0.6 g protein per kg ideal body weight (IBW)). An additional four patients were equilibrated in the same manner with the same type of diet with a protein level of 0.75 g per kg IBW. Plasma leucine and breath CO/sub 2/ enrichments were measured by mass spectrometry. Protein synthesis and leucine metabolism were identical in controls and patients when both were fed a diet with 0.6 g protein/kg IBW. Results indicate that systemic derangements of leucine metabolism are not the cause of Hepatic Encephalopathy.

  18. Subacute necrotising encephalopathy in an Alaskan husky.

    PubMed

    Wakshlag, J J; de Lahunta, A; Robinson, T; Cooper, B J; Brenner, O; O'Toole, T D; Olson, J; Beckman, K B; Glass, E; Reynolds, A J

    1999-12-01

    A 29-month-old female Alaskan husky was presented recumbent, tetraparetic and in a state of dementia, with blindness and cranial nerve deficits. The dog's progress was followed for over two months, as the signs resolved to an non-progressive mild hypermetria with slight proprioceptive ataxia, a diminished menace response and inability to prehend food. Magnetic resonance imaging (MRI) revealed bilateral cavitation extending from the thalamus to the medulla, with less pronounced degenerative lesions in the caudate nucleus, putamen and claustrum. Cerebrospinal fluid lactate and pyruvate concentrations were in their normal ranges. Necropsy and histological examination confirmed the MRI findings as well as neuronal degeneration of the cerebellar cortex in the vermis and degenerative changes in the neocortex at the depths of the cerebral sulci. In view of the similarity of lesions to subacute necrotising encephalomyelopathy, known as Leigh's disease in humans, a tentative diagnosis of a mitochondrial encephalopathy was made.

  19. Prion biology relevant to bovine spongiform encephalopathy.

    PubMed

    Novakofski, J; Brewer, M S; Mateus-Pinilla, N; Killefer, J; McCusker, R H

    2005-06-01

    Bovine spongiform encephalopathy (BSE) and chronic wasting disease (CWD) of deer and elk are a threat to agriculture and natural resources, as well as a human health concern. Both diseases are transmissible spongiform encephalopathies (TSE), or prion diseases, caused by autocatalytic conversion of endogenously encoded prion protein (PrP) to an abnormal, neurotoxic conformation designated PrPsc. Most mammalian species are susceptible to TSE, which, despite a range of species-linked names, is caused by a single highly conserved protein, with no apparent normal function. In the simplest sense, TSE transmission can occur because PrPsc is resistant to both endogenous and environmental proteinases, although many details remain unclear. Questions about the transmission of TSE are central to practical issues such as livestock testing, access to international livestock markets, and wildlife management strategies, as well as intangible issues such as consumer confidence in the safety of the meat supply. The majority of BSE cases seem to have been transmitted by feed containing meat and bone meal from infected animals. In the United Kingdom, there was a dramatic decrease in BSE cases after neural tissue and, later, all ruminant tissues were banned from ruminant feed. However, probably because of heightened awareness and widespread testing, there is growing evidence that new variants of BSE are arising "spontaneously," suggesting ongoing surveillance will continue to find infected animals. Interspecies transmission is inefficient and depends on exposure, sequence homology, TSE donor strain, genetic polymorphism of the host, and architecture of the visceral nerves if exposure is by an oral route. Considering the low probability of interspecies transmission, the low efficiency of oral transmission, and the low prion levels in nonnervous tissues, consumption of conventional animal products represents minimal risk. However, detection of rare events is challenging, and TSE

  20. Quantitative Risk Assessment of Bovine Spongiform Encephalopathy

    NASA Astrophysics Data System (ADS)

    Tsutsui, Toshiyuki; Kasuga, Fumiko

    Bovine spongiform encephalopathy (BSE) is a progressive neurological disease of cattle affecting the central nervous system and was first diagnosed in the United Kingdom (UK) in 1986 (Wells et al., 1987). This disease is one of the transmissible spongiform encephalopathy (TSE) which includes Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep. The causative agent of TSE is considered to be an abnormal form of prion protein. However, the details of its pathogenic mechanism have not been fully identified. Scrapie, which causes neurological symptoms in sheep and goats, has existed in the UK for 200 years (Hoinville, 1996) and spread across the rest of the world in the 1900s (Detwiler & Baylis, 2003). There has been no report so far that scrapie can be transmitted to humans. Initially, BSE was also considered as a disease affecting only animals. However, a variant type of Creutzfeldt-Jakob disease (vCJD) was first reported in the UK, and exposure to a BSE agent was suspected (Collinge, Sidle, Meads, Ironside, & Hill, 1996). vCJD is clinically and pathologically different from the sporadic type of CJD, and age at clinical onset of vCJD is younger than sporadic type (Will et al., 1996). Since the UK government announced the possible association between BSE and vCJD in 1996, BSE has become a huge public health concern all over the world. Of particular concern about vCJD, the fatal disease in younger age, distorted consumer confidence in beef safety, and as a result reduced beef consumption has been seen in many BSE-affected countries.

  1. Hepatic encephalopathy in acute-on-chronic liver failure.

    PubMed

    Lee, Guan-Huei

    2015-10-01

    The presence of hepatic encephalopathy (HE) within 4 weeks is part of the criteria for defining acute-on-chronic liver failure (ACLF). The pathophysiology of HE is complex, and hyperammonemia and cerebral hemodynamic dysfunction appear to be central in the pathogenesis of encephalopathy. Recent data also suggest that inflammatory mediators may have a significant role in modulating the cerebral effect of ammonia. Multiple prospective and retrospective studies have shown that hepatic encephalopathy in ACLF patients is associated with higher mortality, especially in those with grade III-IV encephalopathy, similar to that of acute liver failure (ALF). Although significant cerebral edema detected by CT in ACLF patients appeared to be less common, specialized MRI imaging was able to detect cerebral edema even in low grade HE. Ammonia-focused therapy constitutes the basis of current therapy, as in the treatment of ALF. Emerging treatment strategies focusing on modulating the gut-liver-circulation-brain axis are discussed.

  2. Brain proton magnetic resonance spectroscopy for hepatic encephalopathy

    NASA Astrophysics Data System (ADS)

    Ong, Chin-Sing; McConnell, James R.; Chu, Wei-Kom

    1993-08-01

    Liver failure can induce gradations of encephalopathy from mild to stupor to deep coma. The objective of this study is to investigate and quantify the variation of biochemical compounds in the brain in patients with liver failure and encephalopathy, through the use of water- suppressed, localized in-vivo Proton Magnetic Resonance Spectroscopy (HMRS). The spectral parameters of the compounds quantitated are: N-Acetyl Aspartate (NAA) to Creatine (Cr) ratio, Choline (Cho) to Creatine ratio, Inositol (Ins) to Creatine ratio and Glutamine-Glutamate Amino Acid (AA) to Creatine ratio. The study group consisted of twelve patients with proven advanced chronic liver failure and symptoms of encephalopathy. Comparison has been done with results obtained from five normal subjects without any evidence of encephalopathy or liver diseases.

  3. Genetics Home Reference: MECP2-related severe neonatal encephalopathy

    MedlinePlus

    ... onset encephalopathy with microcephaly Patient Support and Advocacy Resources (2 links) Rare Disease Clinical Research Network: Rett Syndrome, MECP2 Duplication and Rett-Related Disorders Natural History Study RettSyndrome.org GeneReviews (1 link) MECP2- ...

  4. [Follow-up of newborns with hypoxic-ischaemic encephalopathy].

    PubMed

    Martínez-Biarge, M; Blanco, D; García-Alix, A; Salas, S

    2014-07-01

    Hypothermia treatment for newborn infants with hypoxic-ischemic encephalopathy reduces the number of neonates who die or have permanent neurological deficits. Although this therapy is now standard of care, neonatal hypoxic-ischaemic encephalopathy still has a significant impact on the child's neurodevelopment and quality of life. Infants with hypoxic-ischaemic encephalopathy should be enrolled in multidisciplinary follow-up programs in order to detect impairments, to initiate early intervention, and to provide counselling and support for families. This article describes the main neurodevelopmental outcomes after term neonatal hypoxic-ischaemic encephalopathy. We offer recommendations for follow-up based on the infant's clinical condition and other prognostic indicators, mainly neonatal neuroimaging. Other aspects, such as palliative care and medico-legal issues, are also briefly discussed.

  5. Fetal asphyctic preconditioning modulates the acute cytokine response thereby protecting against perinatal asphyxia in neonatal rats

    PubMed Central

    2013-01-01

    Background Perinatal asphyxia (PA) is a major cause of brain damage and neurodevelopmental impairment in infants. Recent investigations have shown that experimental sublethal fetal asphyxia (FA preconditioning) protects against a subsequent more severe asphyctic insult at birth. The molecular mechanisms of this protection have, however, not been elucidated. Evidence implicates that inflammatory cytokines play a protective role in the induction of ischemic tolerance in the adult brain. Accordingly, we hypothesize that FA preconditioning leads to changes in the fetal cytokine response, thereby protecting the newborn against a subsequent asphyctic insult. Methods In rats, FA preconditioning was induced at embryonic day 17 by clamping the uterine vasculature for 30 min. At term birth, global PA was induced by placing the uterine horns, containing the pups, in a saline bath for 19 min. We assessed, at different time points after FA and PA, mRNA and protein expression of several cytokines and related receptor mRNA levels in total hemispheres of fetal and neonatal brains. Additionally, we measured pSTAT3/STAT3 levels to investigate cellular responses to these cytokines. Results Prenatally, FA induced acute downregulation in IL-1β, TNF-α and IL-10 mRNA levels. At 96 h post FA, IL-6 mRNA and IL-10 protein expression were increased in FA brains compared with controls. Two hours after birth, all proinflammatory cytokines and pSTAT3/STAT3 levels decreased in pups that experienced FA and/or PA. Interestingly, IL-10 and IL-6 mRNA levels increased after PA. When pups were FA preconditioned, however, IL-10 and IL-6 mRNA levels were comparable to those in controls. Conclusions FA leads to prenatal changes in the neuroinflammatory response. This modulation of the cytokine response probably results in the protective inflammatory phenotype seen when combining FA and PA and may have significant implications for preventing post-asphyctic perinatal encephalopathy. PMID:23351591

  6. Etiological analysis of presumed perinatal stroke.

    PubMed

    Kocaman, Canan; Yilmaz, Yuksel

    2012-02-01

    This study aimed to investigate the maternal, pre- and perinatal, and prothrombotic factors with congenital hemiparesis due to presumed perinatal stroke (PPS). Prothrombotic risk factors including protein C and S, antithrombin III, lipoprotein (a), homocystein, factor VIII levels; anticardiolipin antibodies and lupus anticoagulant; methylenetetrahydrofolate reductase mutations, factor V Leiden, prothrombin G20210A mutations were investigated. Arterial ischemic stroke was detected in 60% and periventricular venous infarction in 40%. At least one prothrombotic risk factor was present in 69%, two in 17%, and three or more in 8.5% of cases. The most common combination was methylenetetrahydrofolate reductase C677T and factor V Leiden heterozygosity. The etiology and pathogenesis of PPS is still unclear. According to this study, most of the patients with PPS might have one or more prothrombotic risk factors and certain prenatal risk factors including intrauterine growth retardation, twin gestation and preeclampsia might be related to PPS. PMID:21561729

  7. Perinatal nurses' perceptions of competency assessments.

    PubMed

    Maddox, Brenda L; Waller-Wise, Renece; Weed, Latricia D

    2014-10-01

    Competency assessment should be a changing and continuing process. In addition, it should be appropriate for the organization and the nursing staff. Nursing educators are challenged to provide a competency assessment process that is relevant and meaningful. This qualitative research study describes perinatal nurses' perceptions of a change from a traditional testing competency assessment to a hands-on competency assessment. The setting was a medical center in southeastern Alabama. Thirteen nurses participated in the study. Focus groups were used to explore the new assessment method. Three themes were identified: I am learning, multidimensional learning together, and increasing professional confidence. As the medical center perinatal nursing competency assessment program continues to improve, the expectation is for other departments to assess and revise their competency assessment program.

  8. Early progressive encephalopathy in boys and MECP2 mutations.

    PubMed

    Kankirawatana, P; Leonard, H; Ellaway, C; Scurlock, J; Mansour, A; Makris, C M; Dure, L S; Friez, M; Lane, J; Kiraly-Borri, C; Fabian, V; Davis, M; Jackson, J; Christodoulou, J; Kaufmann, W E; Ravine, D; Percy, A K

    2006-07-11

    MECP2 mutations mainly occur in females with Rett syndrome. Mutations have been described in 11 boys with progressive encephalopathy: seven of nine with affected sisters and two de novo. The authors report four de novo occurrences: three pathogenic and one potentially pathogenic. Common features include failure to thrive, respiratory insufficiency, microcephaly, and abnormal motor control. MECP2 mutations should be assessed in boys with progressive encephalopathy and one or more of respiratory insufficiency, abnormal movements or tone, and intractable seizures.

  9. Late onset arginase deficiency presenting with encephalopathy and midbrain hyperintensity.

    PubMed

    Maramattom, Boby Varkey; Raja, Rajat; Balagopal, Anuroop

    2016-01-01

    Urea cycle disorders (UCD) are very rare metabolic disorders that present with encephalopathy and hyperammonemia. Of the UCDs, Arginase deficiency (ARD) is the rarest and presents in childhood with a progressive spastic diplegia or seizures. Acute presentation in adulthood is extremely unusual.[1] We present the first case of adult onset ARD presenting with encephalopathy and diffusion weighted MRI findings that resembled a moustache in the midbrain. PMID:27570396

  10. Late onset arginase deficiency presenting with encephalopathy and midbrain hyperintensity

    PubMed Central

    Maramattom, Boby Varkey; Raja, Rajat; Balagopal, Anuroop

    2016-01-01

    Urea cycle disorders (UCD) are very rare metabolic disorders that present with encephalopathy and hyperammonemia. Of the UCDs, Arginase deficiency (ARD) is the rarest and presents in childhood with a progressive spastic diplegia or seizures. Acute presentation in adulthood is extremely unusual.[1] We present the first case of adult onset ARD presenting with encephalopathy and diffusion weighted MRI findings that resembled a moustache in the midbrain. PMID:27570396

  11. Spongiform encephalopathy in a captive puma (Felis concolor).

    PubMed

    Willoughby, K; Kelly, D F; Lyon, D G; Wells, G A

    1992-11-01

    A captive adult puma developed ataxia, a hypermetric gait and whole body tremor. The signs progressed over a period of six weeks. Histopathological examination following euthanasia demonstrated spongiform encephalopathy, gliosis and mild non-suppurative meningoencephalitis. Immunostaining with a polyclonal antiserum revealed prion protein (PrP) associated with these changes in sections of cervical spinal cord and medulla. This is the first confirmed case of a scrapie-like spongiform encephalopathy described in a non-domestic cat in the United Kingdom.

  12. Perinatal mortality in Matlab, Bangladesh: a community-based study.

    PubMed

    Fauveau, V; Wojtyniak, B; Mostafa, G; Sarder, A M; Chakraborty, J

    1990-09-01

    Perinatal deaths, comprising stillbirths and deaths during the first week of life, were monitored over the eight-year period 1979 to 1986 in a rural Bangladeshi population of 196,000. The perinatal mortality rate was 75 per 1000 total births. The rate was 13% higher in males than females. Stillbirth and early neonatal mortality rates were 37 and 38 per 1000 total births, respectively. The major causes of perinatal deaths are presented, as well as some of the maternal determinants. During the period under study, perinatal mortality declined regularly and significantly over time in an area covered by an intensive Family Planning and Health Services programme, but not in the adjacent control area. This raises the issue of the impact of such a programme upon perinatal mortality, and the need to include a strong maternity care component into primary healthcare strategies if further reductions of perinatal mortality are to be achieved. PMID:2262255

  13. Perinatal mortality attributable to complications of childbirth in Matlab, Bangladesh.

    PubMed Central

    Kusiako, T.; Ronsmans, C.; Van der Paal, L.

    2000-01-01

    Very few population-based studies of perinatal mortality in developing countries have examined the role of intrapartum risk factors. In the present study, the proportion of perinatal deaths that are attributable to complications during childbirth in Matlab, Bangladesh, was assessed using community-based data from a home-based programme led by professional midwives between 1987 and 1993. Complications during labour and delivery--such as prolonged or obstructed labour, abnormal fetal position, and hypertensive diseases of pregnancy--increased the risk of perinatal mortality fivefold and accounted for 30% of perinatal deaths. Premature labour, which occurred in 20% of pregnancies, accounted for 27% of perinatal mortality. Better care by qualified staff during delivery and improved care of newborns should substantially reduce perinatal mortality in this study population. PMID:10859856

  14. Perinatal Risk Factors for Mild Motor Disability

    ERIC Educational Resources Information Center

    Hands, Beth; Kendall, Garth; Larkin, Dawne; Parker, Helen

    2009-01-01

    The aetiology of mild motor disability (MMD) is a complex issue and as yet is poorly understood. The aim of this study was to identify the prevalence of perinatal risk factors in a cohort of 10-year-old boys and girls with (n = 362) and without (n = 1193) MMD. Among the males with MMD there was a higher prevalence of postpartum haemorrhage,…

  15. Specific ultrasonographic features of perinatal lethal hypophosphatasia.

    PubMed

    Zankl, Andreas; Mornet, Etienne; Wong, Shell

    2008-05-01

    Prenatal diagnosis of perinatal lethal hypophosphatasia (PL-HPH) by ultrasonography is difficult as PL-HPH must be differentiated from other skeletal dysplasias with short long bones and poor mineralization of the skeleton, such as osteogenesis imperfecta type II and achondrogenesis/hypochondrogenesis. Here we present a case of molecularly confirmed PL-HPH and illustrate specific ultrasonographic findings that help to distinguish PL-HPH from similar conditions. PMID:18386808

  16. Mechanisms of perinatal arterial ischemic stroke

    PubMed Central

    Fernández-López, David; Natarajan, Niranjana; Ashwal, Stephen; Vexler, Zinaida S

    2014-01-01

    The incidence of perinatal stroke is high, similar to that in the elderly, and produces a significant morbidity and severe long-term neurologic and cognitive deficits, including cerebral palsy, epilepsy, neuropsychological impairments, and behavioral disorders. Emerging clinical data and data from experimental models of cerebral ischemia in neonatal rodents have shown that the pathophysiology of perinatal brain damage is multifactorial. These studies have revealed that, far from just being a smaller version of the adult brain, the neonatal brain is unique with a very particular and age-dependent responsiveness to hypoxia–ischemia and focal arterial stroke. In this review, we discuss fundamental clinical aspects of perinatal stroke as well as some of the most recent and relevant findings regarding the susceptibility of specific brain cell populations to injury, the dynamics and the mechanisms of neuronal cell death in injured neonates, the responses of neonatal blood–brain barrier to stroke in relation to systemic and local inflammation, and the long-term effects of stroke on angiogenesis and neurogenesis. Finally, we address translational strategies currently being considered for neonatal stroke as well as treatments that might effectively enhance repair later after injury. PMID:24667913

  17. Action plan to reduce perinatal mortality.

    PubMed

    Bhakoo, O N; Kumar, R

    1990-01-01

    The government of India has set a goal of reducing perinatal mortality from its current rate of 48/1000 to 30-35/1000 by the year 2000. Perinatal deaths result from maternal malnutrition, inadequate prenatal care, complications of delivery, and infections in the postpartum period. Since reductions in perinatal mortality require attention to social, economic, and behavioral factors, as well as improvements in the health care delivery system, a comprehensive strategy is required. Social measures, such as raising the age at marriage to 18 years for females, improving the nutritional status of adolescent girls, reducing the strenuousness of work during pregnancy, improving female literacy, raising women's status in the society and thus in the family, and poverty alleviation programs, would all help eliminate the extent of complications of pregnancy. Measures required to enhance infant survival include improved prenatal care, prenatal tetanus toxoid immunization, use of sterile disposable cord care kits, the provision of mucus extractors and resuscitation materials to birth attendants, the creation of neonatal care units in health facilities, and more efficient referral of high-risk newborns and mothers. Since 90% of births in rural India take place at home priority must be given to training traditional birth attendants in the identification of high risk factors during pregnancy, delivery, and the newborn period. PMID:12316585

  18. Ethics in perinatal medicine: A global perspective.

    PubMed

    Chervenak, Frank A; McCullough, Laurence B

    2015-10-01

    This article describes the professional responsibility model of perinatal ethics, which requires the perinatologist in all cases to identify and balance beneficence-based and autonomy-based obligations to the pregnant patient, beneficence-based obligations to the fetal patient, and beneficence-based obligations to the neonatal patient. We explain how this model avoids the clinical failure of both fetal and maternal rights-based reductionism, i.e., insistence either on unlimited fetal rights or on unlimited maternal rights, respectively. The professional responsibility model of perinatal ethics provides the basis for the transnational clinical ethical concept of healthcare justice, which requires that beneficence-based obligations to all patients be routinely fulfilled by providing them with an evidence-based standard of care. We then show how healthcare justice can be used to identify and address ethically unacceptable allocation of healthcare resources. The professional responsibility model of perinatal ethics creates an important role for the perinatologist as responsible advocate for pregnant, fetal, and neonatal patients. PMID:26049210

  19. Behavioural outcomes of perinatal maternal fluoxetine treatment.

    PubMed

    McAllister, B B; Kiryanova, V; Dyck, R H

    2012-12-13

    During and following pregnancy, women are at considerable risk of experiencing depression. For treatment, selective serotonin reuptake inhibitor drugs, such as fluoxetine, are commonly prescribed, yet the potential effects of perinatal exposure to these drugs on the brain and behaviour have not been examined in humans beyond childhood. This is despite abundant evidence from studies using rodents indicating that altered serotonin levels early in life affect neurodevelopment and behavioural outcomes. These reported effects on behaviour are inconsistent, however, and the testing of females has often been overlooked. In the present study, the behavioural outcomes of female mice perinatally (embryonic day 15 to postnatal day 12) treated with fluoxetine (25mg/kg/day) via a non-stressful method of maternal administration were assessed using a battery of tests. Maternal treatment resulted in subtle alterations in anxiety-like and depression-like behaviour in early adulthood, with a decrease in both types of behaviour as well as body weight. Though altered anxiety and depression have previously been reported in this area of research, decreased anxiety is a novel finding. While there was little effect of perinatal maternal fluoxetine treatment on many of the behaviours assessed, the capacity to alter "emotional" behaviours in mice has implications with regard to research on human infant fluoxetine exposure.

  20. Perinatal nicotine-induced transgenerational asthma.

    PubMed

    Rehan, Virender K; Liu, Jie; Sakurai, Reiko; Torday, John S

    2013-10-01

    Asthma is a major public health hazard worldwide. Its transgenerational inheritance has been inferred from epidemiological studies. More recently, using nicotine as a proxy for maternal smoking, we have demonstrated that an asthma-like phenotype can be inherited by rat offspring for up to two generations, i.e., multigenerationally, after the initial intrauterine exposure. We hypothesized that asthma transmission to offspring following perinatal nicotine exposure is not restricted up to F2 generation, but it also extends to subsequent generations. To test this hypothesis, using a well-established rat model of nicotine exposure-induced childhood asthma, we determined if perinatal nicotine exposure of F0 gestating dams would transmit asthma transgenerationally to F3 offspring. We now extend our findings to third-generation offspring, including abnormal pulmonary function, particularly as it relates to the occurrence in the upper airway exclusively in males, and to its effects on molecular functional markers (fibronectin and peroxisome proliferator-activated receptor γ), previously shown to be consistent with the asthma phenotype, herein expressed in fibroblasts isolated from the lung. These data, for the first time, demonstrate the transgenerational transmission of the asthma phenotype to F3 offspring following perinatal nicotine exposure of F0 dams. PMID:23911437

  1. Dual role of astrocytes in perinatal asphyxia injury and neuroprotection.

    PubMed

    Romero, J; Muñiz, J; Logica Tornatore, T; Holubiec, M; González, J; Barreto, G E; Guelman, L; Lillig, C H; Blanco, E; Capani, F

    2014-04-17

    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. PMID:24172702

  2. Dual role of astrocytes in perinatal asphyxia injury and neuroprotection.

    PubMed

    Romero, J; Muñiz, J; Logica Tornatore, T; Holubiec, M; González, J; Barreto, G E; Guelman, L; Lillig, C H; Blanco, E; Capani, F

    2014-04-17

    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.

  3. New uses of legacy systems: examples in perinatal care.

    PubMed

    Margolis, A; Vázquez, R; Mendoza, G; Zignago, A; López, A; Lucián, H

    1999-01-01

    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.

  4. New uses of legacy systems: examples in perinatal care.

    PubMed Central

    Margolis, A.; Vázquez, R.; Mendoza, G.; Zignago, A.; López, A.; Lucián, H.

    1999-01-01

    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

  5. The neuropathology of acquired pre- and perinatal brain injuries.

    PubMed

    Folkerth, Rebecca D

    2007-02-01

    Acquired pre- and perinatal brain injuries comprise a significant proportion of perinatal neuropathology. They are associated with placental abnormalities, maternal factors, multiple gestations, and preterm labor, as well as with the later development of cerebral palsy and developmental delay. The patterns of perinatal brain injury depend on the etiology (often hypoxic-ischemic) and the timing relative to the development of the fetal nervous system, since the vulnerabilities of gray and white matter differ across postconceptional age and by neuroanatomic site. Nevertheless, characteristic features allow determination of the approximate age and cause of each pattern of injury in the perinatal brain. PMID:17455862

  6. The perinatal safety nurse: exemplar of transformational leadership.

    PubMed

    Raab, Cheryl; Palmer-Byfield, Renée

    2011-01-01

    There is increased attention to the issue of patient safety in the care of pregnant women and their infants. The Joint Commission has issued sentinel event alerts regarding infant and maternal morbidity and mortality. Hospitals and healthcare systems are implementing perinatal patient safety programs to minimize the risk of preventable patient harm. This article describes the role of the perinatal patient safety nurse as one aspect of a comprehensive initiative to promote patient safety for women who give birth. Nurses and organizations offering perinatal care are encouraged to incorporate the role of perinatal patient safety nurse in their patient safety efforts. PMID:21743356

  7. Topiramate increases the risk of valproic acid-induced encephalopathy.

    PubMed

    Noh, Young; Kim, Dong Wook; Chu, Kon; Lee, Soon-Tae; Jung, Keun-Hwa; Moon, Hye-Jin; Lee, Sang Kun

    2013-01-01

    Metabolic encephalopathy is a rare but serious complication of valproic acid (VPA) therapy that usually presents with impaired consciousness or increased seizure frequency. Although it has been suggested that topiramate (TPM) increases the risk of VPA-induced encephalopathy, the additional risk in patients receiving TPM therapy has not been evaluated. We reviewed all adult patients who took VPA between January 2005 and February 2009 at the Seoul National University Hospital and identified patients with VPA-induced encephalopathy based on clinical and electroencephalography (EEG) data. Information on sex, age, serum ammonia level, serum VPA level, liver function test, and EEG was collected from patient registry and medical data. We enrolled 8,372 patients who received VPA therapy and 1,236 patients who received VPA/TPM combination therapy. We identified 11 patients with VPA-induced encephalopathy (0.13%), 7 of whom received a combination therapy of VPA and TPM. The odds ratio of VPA-induced encephalopathy with TPM over that without TPM was 10.16. There were no significant differences in sex distribution, number of antiepileptic agents, ammonia level, VPA serum level, underlying diseases, dosage of VPA, duration of VPA treatment, treatment of encephalopathy, and outcomes between the two groups. Our study showed that the prevalence of VPA-induced encephalopathy is approximately 0.1% among patients treated with VPA and that the risk of this condition, although still low, can increase by approximately 10 times in the presence of TPM therapy. Based on these results, we suggest that TPM should be carefully used in patients receiving VPA treatment.

  8. 78 FR 11207 - Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-15

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration Transmissible Spongiform Encephalopathies Advisory Committee... be open to the public. Name of Committee: Transmissible Spongiform Encephalopathies...

  9. The Neuropathology of Chronic Traumatic Encephalopathy

    PubMed Central

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

    2015-01-01

    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

  10. Benign encephalopathy of pregnancy. Preliminary clinical observations.

    PubMed

    Poser, C M; Kassirer, M R; Peyser, J M

    1986-01-01

    A survey of 67 pregnancies in 51 professional women (physicians, psychologists, nurses, administrators, etc.) revealed the occurrence of symptoms of cognitive dysfunction such as forgetfulness, disorientation, confusion and reading difficulties in 28 pregnancies occurring in 21 women. These were unrelated to such factors as age of delivery, percentage weight gain, the baby's sex or birth weight, alcohol consumption, smoking, a history of migraine or allergy or other symptoms occurring during pregnancy such as sleepiness and lack of concentration, irritability, loss of interest in job or nightmares. Nor was there any correlation with hypertension, proteinuria, glycosuria, ketonuria, anemia, or morning sickness. Furthermore, these cognitive disturbances were not related to depression or sleep deprivation. Despite these symptoms, none of the women suffering from them were forced to interrupt their professional activities during pregnancy. The syndrome of benign encephalopathy of pregnancy should be recognized so that simple precautions can be taken to prevent any interference with professional or other activities. The etiology of the syndrome is unknown.

  11. The why and wherefore of hepatic encephalopathy

    PubMed Central

    Grover, Vijay PB; Tognarelli, Joshua M; Massie, Nicolas; Crossey, Mary ME; Cook, Nicola A; Taylor-Robinson, Simon D

    2015-01-01

    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

  12. A critical review of chronic traumatic encephalopathy.

    PubMed

    Iverson, Grant L; Gardner, Andrew J; McCrory, Paul; Zafonte, Ross; Castellani, Rudy J

    2015-09-01

    Chronic traumatic encephalopathy (CTE) has been described in the literature as a neurodegenerative disease with: (i) localized neuronal and glial accumulations of phosphorylated tau (p-tau) involving perivascular areas of the cerebral cortex, sulcal depths, and with a preference for neurons within superficial cortical laminae; (ii) multifocal axonal varicosities and axonal loss involving deep cortex and subcortical white matter; (iii) relative absence of beta-amyloid deposits; (iv) TDP-43 immunoreactive inclusions and neurites; and (v) broad and diverse clinical features. Some of the pathological findings reported in the literature may be encountered with age and other neurodegenerative diseases. However, the focality of the p-tau cortical findings in particular, and the regional distribution, are believed to be unique to CTE. The described clinical features in recent cases are very similar to how depression manifests in middle-aged men and with frontotemporal dementia as the disease progresses. It has not been established that the described tau pathology, especially in small amounts, can cause complex changes in behavior such as depression, substance abuse, suicidality, personality changes, or cognitive impairment. Future studies will help determine the extent to which the neuropathology is causally related to the diverse clinical features. PMID:26183075

  13. Posterior Reversible Encephalopathy Syndrome Complicating Traumatic Pancreatitis

    PubMed Central

    Sigurtà, Anna; Terzi, Valeria; Regna-Gladin, Caroline; Fumagalli, Roberto

    2016-01-01

    Abstract We are reporting a case of posterior reversible encephalopathy syndrome (PRES) developed in an unusual clinical scenario without the presence of the most described symptoms. PRES is a neurological and radiological syndrome described in many different clinical conditions. In children it has been mostly reported in association with hematological and renal disorders. Our patient was a 15 years old boy, admitted to our intensive care unit for pancreatitis after blunt abdominal trauma. During the stay in the intensive care unit, he underwent multiple abdominal surgical interventions for pancreatitis complications. He had a difficult management of analgesia and sedation, being often agitated with high arterial pressure, and he developed a bacterial peritonitis. After 29 days his neurological conditions abruptly worsened with neuroimaging findings consistent with PRES. His clinical conditions progressively improved after sedation and arterial pressure control. He was discharged at home with complete resolution of the neurological and imaging signs 2 months later. The pathophysiology of PRES is controversial and involves disordered autoregulation ascribable to hypertension and endothelial dysfunction. In this case both hypertension and endothelial activation, triggered by sepsis and pancreatitis, could represent the culprits of PRES onset. Even if there is no specific treatment for this condition, a diagnosis is mandatory to start antihypertensive and supportive treatment. We are therefore suggesting to consider PRES in the differential diagnosis of a neurological deterioration preceded by hypertension and/or septic state, even without other “typical” clinical features. PMID:27258506

  14. The why and wherefore of hepatic encephalopathy.

    PubMed

    Grover, Vijay Pb; Tognarelli, Joshua M; Massie, Nicolas; Crossey, Mary Me; Cook, Nicola A; Taylor-Robinson, Simon D

    2015-01-01

    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

  15. Mycophenolate-Induced Posterior Reversible Encephalopathy Syndrome.

    PubMed

    Khajuria, Bhavik; Khajuria, Mansi; Agrawal, Yashwant

    2016-01-01

    A 29-year-old woman presented with diffuse anasarca and shortness of breath. Workup revealed a creatinine of 3.3 and a glomerular filtration rate of 17. The patient was also found to be pancytopenic with evidence of hemolytic anemia. A renal biopsy showed evidence of stage IV lupus nephritis with rapidly progressive glomerulonephritis. Her lupus was further classified as ANA negative and anti-dsDNA positive. Mycophenolate and triweekly hemodialysis were started along with a steroid burst of methylprednisolone 1 g for 3 days followed by prednisone 60 mg daily. Four days after discharge, the patient represented with a witnessed 3-minute seizure involving bowel incontinence, altered mental status, and tongue biting. She was given 2 mg intravenous lorazepam and loaded with 1000 mg levetiracetam for seizure prophylaxis. Magnetic resonance imaging of the head revealed bilateral posterior hemispheric subcortical edema, and the diagnosis of posterior reversible encephalopathy syndrome was made. Mycophenolate was immediately discontinued and replaced with cyclophosphamide. Strict blood pressure control below 140/90 mm Hg was maintained initially with intravenous nicardipine drip and then transitioned to oral nifedipine, clonidine, losartan, and minoxidil. A repeat head magnetic resonance imaging 8 days later showed resolved subcortical edema consistent with the patient's improved mental status. No permanent neurologic sequelae were recorded as a result of this hospital episode. PMID:25933141

  16. Sodium Benzoate for Treatment of Hepatic Encephalopathy

    PubMed Central

    Misel, Michael L.; Patton, Heather; Mendler, Michel

    2013-01-01

    Hepatic encephalopathy (HE) is a serious but usually reversible neuropsychiatric complication of cirrhosis, inborn errors of metabolism involving disorders of the urea cycle, and noncirrhotic portosystemic shunting that most commonly arises from a transjugular intrahepatic portosystemic shunting procedure. Symptoms can include alterations in cognitive function, neuromuscular activity, and consciousness, as well as sleep disorders and mood changes. HE is associated with significant morbidity and mortality and, if not properly treated, will lead to increased hospital admissions and healthcare costs. Although the standard therapies of lactulose and rifaximin (Xifaxan, Salix) are effective for most patients, these drugs may be associated with significant adverse effects and expense and, in some patients, inadequate therapeutic response. A need for adjunctive therapies exists. Drugs that target serum and tissue ammonia metabolism and elimination may be important adjuncts to drugs that reduce ammonia production and absorption from the gastrointestinal tract for patients with severe or persistent overt symptoms of HE. Sodium benzoate is an inexpensive adjunctive agent that can be used in addition to lactulose and rifaximin and may provide an option for some select patients with refractory HE who have failed to respond to standard therapies or who cannot afford them. Although sodium benzoate does not share the same adverse effect profiles of standard therapies for HE, its efficacy has not been well established. Given the significant dose-dependent sodium content of this therapy, it may not be appropriate for patients with significant fluid retention or kidney dysfunction. PMID:24711766

  17. Antibody-Mediated Autoimmune Encephalopathies and Immunotherapies.

    PubMed

    Gastaldi, Matteo; Thouin, Anaïs; Vincent, Angela

    2016-01-01

    Over the last 15 years it has become clear that rare but highly recognizable diseases of the central nervous system (CNS), including newly identified forms of limbic encephalitis and other encephalopathies, are likely to be mediated by antibodies (Abs) to CNS proteins. The Abs are directed against membrane receptors and ion channel-associated proteins that are expressed on the surface of neurons in the CNS, such as N-methyl D-aspartate receptors and leucine-rich, glioma inactivated 1 protein and contactin-associated protein like 2, that are associated with voltage-gated potassium channels. The diseases are not invariably cancer-related and are therefore different from the classical paraneoplastic neurological diseases that are associated with, but not caused by, Abs to intracellular proteins. Most importantly, the new antibody-associated diseases almost invariably respond to immunotherapies with considerable and sometimes complete recovery, and there is convincing evidence of their pathogenicity in the relatively limited studies performed so far. Treatments include first-line steroids, intravenous immunoglobulins, and plasma exchange, and second-line rituximab and cyclophosphamide, followed in many cases by steroid-sparing agents in the long-term. This review focuses mainly on N-methyl D-aspartate receptor- and voltage-gated potassium channel complex-related Abs in adults, the clinical phenotypes, and treatment responses. Pediatric cases are referred to but not reviewed in detail. As there have been very few prospective studies, the conclusions regarding immunotherapies are based on retrospective studies. PMID:26692392

  18. Cortical Reorganization of Language Functioning Following Perinatal Left MCA Stroke

    ERIC Educational Resources Information Center

    Tillema, Jan-Mendelt; Byars, Anna W.; Jacola, Lisa M.; Schapiro, Mark B.; Schmithorst, Vince J.; Szaflarski, Jerzy P.; Holland, Scott K.

    2008-01-01

    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…

  19. 21 CFR 884.2740 - Perinatal monitoring system and accessories.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Perinatal monitoring system and accessories. 884.2740 Section 884.2740 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN... Monitoring Devices § 884.2740 Perinatal monitoring system and accessories. (a) Identification. A...

  20. Modifying CBT for Perinatal Depression: What Do Women Want?

    ERIC Educational Resources Information Center

    O'Mahen, Heather; Fedock, Gina; Henshaw, Erin; Himle, Joseph A.; Forman, Jane; Flynn, Heather A.

    2012-01-01

    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…

  1. A Primer for Nurses on Perinatal/Neonatal Stroke.

    PubMed

    Becker, Jill S

    2015-01-01

    Perinatal or neonatal stroke is not uncommon, but diagnosis is often missed. Perinatal nurses are often the first health professionals in the position to observe the most typical symptom of stroke in a newborn, which is focal seizure. Etiology, symptoms and outcomes are reviewed and discussed through the context of the author's personal story.

  2. Perinatal Generalized Anxiety Disorder: Assessment and Treatment.

    PubMed

    Misri, Shaila; Abizadeh, Jasmin; Sanders, Shawn; Swift, Elena

    2015-09-01

    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

  3. Perinatal risk factors for acute myeloid leukemia.

    PubMed

    Crump, Casey; Sundquist, Jan; Sieh, Weiva; Winkleby, Marilyn A; Sundquist, Kristina

    2015-12-01

    Infectious etiologies have been hypothesized for acute leukemias because of their high incidence in early childhood, but have seldom been examined for acute myeloid leukemia (AML). We conducted the first large cohort study to examine perinatal factors including season of birth, a proxy for perinatal infectious exposures, and risk of AML in childhood through young adulthood. A national cohort of 3,569,333 persons without Down syndrome who were born in Sweden in 1973-2008 were followed up for AML incidence through 2010 (maximum age 38 years). There were 315 AML cases in 69.7 million person-years of follow-up. We found a sinusoidal pattern in AML risk by season of birth (P < 0.001), with peak risk among persons born in winter. Relative to persons born in summer (June-August), incidence rate ratios for AML were 1.72 (95 % CI 1.25-2.38; P = 0.001) for winter (December-February), 1.37 (95 % CI 0.99-1.90; P = 0.06) for spring (March-May), and 1.27 (95 % CI 0.90-1.80; P = 0.17) for fall (September-November). Other risk factors for AML included high fetal growth, high gestational age at birth, and low maternal education level. These findings did not vary by sex or age at diagnosis. Sex, birth order, parental age, and parental country of birth were not associated with AML. In this large cohort study, birth in winter was associated with increased risk of AML in childhood through young adulthood, possibly related to immunologic effects of early infectious exposures compared with summer birth. These findings warrant further investigation of the role of seasonally varying perinatal exposures in the etiology of AML.

  4. Incarceration, Maternal Hardship, and Perinatal Health Behaviors

    PubMed Central

    Dumont, Dora M.; Wildeman, Christopher; Lee, Hedwig; Gjelsvik, Annie; Valera, Pamela A.; Clarke, Jennifer G.

    2014-01-01

    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

  5. Perinatal Generalized Anxiety Disorder: Assessment and Treatment

    PubMed Central

    Abizadeh, Jasmin; Sanders, Shawn; Swift, Elena

    2015-01-01

    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

  6. Perinatal Generalized Anxiety Disorder: Assessment and Treatment.

    PubMed

    Misri, Shaila; Abizadeh, Jasmin; Sanders, Shawn; Swift, Elena

    2015-09-01

    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.

  7. Perinatal lung function and invasive antenatal procedures

    PubMed Central

    Yuksel, B.; Greenough, A.; Naik, S.; Cheeseman, P.; Nicolaides, K. H.

    1997-01-01

    BACKGROUND: Second trimester amniocentesis has been associated with an excess of perinatal lung function abnormalities. Early amniocentesis might have a similar adverse effect, as could other invasive investigations carried out in the first trimester. METHODS: Plethysmographic measurements of thoracic gas volume (TGV) and airway resistance (Raw), from which specific conductance (sGaw) was calculated, were made in the perinatal period in non-sedated infants. In addition, functional residual capacity (FRC) was measured using a helium gas dilution technique. Measurements were made in 47 infants whose mothers had undergone early amniocentesis, 19 whose mothers had undergone chorion villus sampling, and 25 controls whose mothers had undergone no invasive antenatal procedures. RESULTS: The infants of mothers who had undergone early amniocentesis had higher TGV (95% CI - 6.3 to 1.1 ml/kg) and Raw values (95% CI -10.68 to -5.23 cm H2O/l/s) and lower sGaw (0.11 to 0.84 l/cm H2O.s) and FRC (-5.17 to - 0.87 ml/kg) values than the controls. Infants whose mothers had undergone chorion villus sampling also differed significantly from the controls with higher Raw (-7.59 to -1.99 cm H2O/l/s) and lower sGaw values (0.11 to 0.24 l/cm H2O.s), and had lower Raw values than those in the early amniocentesis group (not significant). Logistic regression analysis, taking into account possible risk factors for abnormal lung function, showed that the procedures performed in the first trimester were independently associated with a high airways resistance. CONCLUSION: These results suggest that invasive procedures performed in the first trimester of pregnancy have an adverse effect on perinatal lung function. 


 PMID:9059482

  8. Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie.

    PubMed

    Jacobs, J G; Sauer, M; van Keulen, L J M; Tang, Y; Bossers, A; Langeveld, J P M

    2011-01-01

    With increased awareness of the diversity of transmissible spongiform encephalopathy (TSE) strains in the ruminant population, comes an appreciation of the need for improved methods of differential diagnosis. Exposure to bovine spongiform encephalopathy (BSE) has been associated with the human TSE, variant Creutzfeldt-Jakob disease, emphasizing the necessity in distinguishing low-risk TSE types from BSE. TSE type discrimination in ruminants such as cattle, sheep, goats and deer, requires the application of several prion protein (PrP)-specific antibodies in parallel immunochemical tests on brain homogenates or tissue sections from infected animals. This study uses in a single incubation step, three PrP-specific antibodies and fluorescent Alexa dye-labelled anti-mouse Fabs on a Western blot. The usual amount of brain tissue needed is 0.5 mg. This multiplex application of antibodies directed towards three different PrP epitopes enabled differential diagnosis of all established main features of classical scrapie, BSE and Nor98-like scrapie in sheep and goats, as well as the currently known BSE types C, H and L in cattle. Moreover, due to an antibody-dependent dual PrP-banding pattern, for the first time CH1641 scrapie of sheep can be reliably discriminated from the other TSE isolate types in sheep.

  9. Seeing more clearly through the fog of encephalopathy.

    PubMed

    Kaplan, Peter W; Sutter, Raoul

    2013-10-01

    Patients with acute confusional states (often referred to as encephalopathy or delirium) pose diagnostic and management challenges for treating physicians. Encephalopathy is associated with a high morbidity and mortality rate, and the diagnosis rests on clinical grounds but may also be supported by the finding of electroencephalographic (EEG) evidence for diffuse cerebral dysfunction. The myriad cerebral transmitter and metabolic disruptions are generated by systemic organ system failures, principal among which are those of the liver, kidneys, lungs, heart, and endocrine system, along with the effects of exogenous toxins and medications. In most cases, several of these organ failures together contribute to the confusional state, frequently in the context of a diffuse cerebral atrophy that affects the aging brain. This special issue of the Journal of Clinical Neurophysiology is dedicated to exploring the electrophysiology of these conditions. It reviews the pathophysiology, psychiatric manifestations, clinical and imaging correlations of the many causes and types of encephalopathy. A literature review of the EEG abnormalities in the various types of encephalopathy provides an overview that ranges from paraneoplastic causes, through organ system failures, postcardiorespiratory arrest, to postoperative delirium. The issue is supplemented by tables of relevant clinical correlations, graphs, Venn diagrams, and the use of mathematical modeling used to explain how defects in the neuronal interplay might generate the EEG patterns seen in encephalopathy. We hope that this assembly will act as a springboard for further discussion and investigation into the EEG underpinnings, clinical correlations, diagnosis. and prognostication of these common and morbid disturbances of brain function.

  10. Preventing Workplace Injuries Among Perinatal Nurses.

    PubMed

    Harolds, Laura; Hurst, Helen

    2016-01-01

    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.

  11. Preventing Workplace Injuries Among Perinatal Nurses.

    PubMed

    Harolds, Laura; Hurst, Helen

    2016-01-01

    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. PMID:26902445

  12. Prenatal and perinatal risk factors of schizophrenia.

    PubMed

    Meli, Giampiero; Ottl, Birgit; Paladini, Angela; Cataldi, Luigi

    2012-12-01

    Schizophrenia could be considered the most severe of all psychiatric disorders. It shows a heterogeneous clinical picture and presents an etiopathogenesis that is not cleared sufficiently. Even if the etiopathogenesis remains a puzzle, there is a scientific consensus that it is an expression of interaction between genotype and environmental factors. In the present article, following a study of literature and the accumulated evidence, the role of prenatal and perinatal factors in the development of schizophrenia will be revised and synthesized. We think that better knowledge of the risk factors could be helpful not only for better comprehension of the pathogenesis but especially to optimize interventions for prevention of the disorder. PMID:22646662

  13. Some observations on perinatal mortality in rural health centre.

    PubMed

    Damodar; Mathur, H N; Sharma, P N

    1983-01-01

    A 4-year study of perinatal mortality in Rural Health Training Centre, Vallabhnagar, affiliated to R.N.T. Medical College, Udaipur was conducted. The chief objective of the study was to analyze underlying causes of perinatal deaths. The perinatal mortality rate was calculated to be 74.19/100 births. Age and parity of mother and sex of the child did not affect perinatal mortality significantly. Antenatal care of mother had a significant role in determining fetal outcome and 1st week survival. Fate of the newborn was substantially affected by birth weight less than 2 kg. Training of "dais" in view of identification of "at risk" cases and nutrition education, better facilities in terms of personnel and equipment, and improvement in referral services emerged as necessary steps needed to plan strategy for lowering perinatal mortaltiy in rural areas. PMID:6680112

  14. Perinatal mortality in a rural district of south India.

    PubMed

    Chandrashekar, S; Rao, R S; Chakladar, B K; Krishnan, L; Nair, N S

    1998-01-01

    Perinatal mortality is one of the most sensitive indices of maternal and child health. The perinatal mortality rate is an indicator of the extent of pregnancy wastage as well as of the quality and quantity of health care available to the mother and the newborn. A community based prospective study carried out on 13,214 births in South Kanara district between Oct. 1991-Sept. 1992 revealed a perinatal mortality rate (PNMR) of 44.65/1000 births. Among the various factors influencing perinatal mortality, breech deliveries and babies of multiple pregnancies had a very high perinatal mortality rate of 180.81/1000 births (adjusted odd's ratio: 4.90) and 128/1000 births (adjusted odd's ratio: 2.64). The previous bad obstetric history of the mother, parity and sex of the newborn were among the other important factors influencing the PNMR. PMID:10773926

  15. Perinatal depression: a review of US legislation and law.

    PubMed

    Rhodes, Ann M; Segre, Lisa S

    2013-08-01

    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. PMID:23740222

  16. Risk of suicidal ideation in adolescents with both self-asphyxial risk-taking behavior and non-suicidal self-injury.

    PubMed

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

    2011-08-01

    This study examined adolescent participation in self-asphyxial risk-taking behaviors (SAB), sometimes known as the "choking game," and its relationship with other adolescent risk behaviors, including non-suicidal self-injury (NSSI). Researchers proposed that participation in SAB and NSSI would be associated with suicidal behavior, disordered eating, and substance use. Using a large community-based sample, results revealed preliminary associations between SAB and other risk-taking behaviors. Adolescents who had engaged in both SAB and NSSI reported more concurrent risk behaviors than adolescents who participated in only one of the behaviors or neither behavior. Results indicate that greater awareness of SAB is important, and continued research can evaluate the possible link between the behavior and risk for suicide.

  17. New mutations in DYNC2H1 and WDR60 genes revealed by whole-exome sequencing in two unrelated Sardinian families with Jeune asphyxiating thoracic dystrophy.

    PubMed

    Cossu, Carla; Incani, Federica; Serra, Maria Luisa; Coiana, Alessandra; Crisponi, Giangiorgio; Boccone, Loredana; Rosatelli, Maria Cristina

    2016-04-01

    Jeune asphyxiating thoracic dystrophy (JATD; Jeune syndrome, MIM 208500) is a rare autosomal recessive chondrodysplasia, phenotypically overlapping with short-rib polydactyly syndromes (SRPS). JATD typical hallmarks include skeletal abnormalities such as narrow chest, shortened ribs, limbs shortened bones, extra fingers and toes (polydactyly), as well as extraskeletal manifestations (renal, liver and retinal disease). To date, disease-causing mutations have been found in several genes, highlighting a marked genetic heterogeneity that prevents a molecular diagnosis of the disease in most families. Here, we report the results of whole-exome sequencing (WES) carried out in four JATD cases, belonging to three unrelated families of Sardinian origin. The exome analysis allowed to identify mutations not previously reported in the DYNC2H1 (MIM 603297) and WDR60 (MIM 615462) genes, both codifying for ciliary intraflagellar transport components whose mutations are known to cause Jeune syndrome.

  18. New mutations in DYNC2H1 and WDR60 genes revealed by whole-exome sequencing in two unrelated Sardinian families with Jeune asphyxiating thoracic dystrophy.

    PubMed

    Cossu, Carla; Incani, Federica; Serra, Maria Luisa; Coiana, Alessandra; Crisponi, Giangiorgio; Boccone, Loredana; Rosatelli, Maria Cristina

    2016-04-01

    Jeune asphyxiating thoracic dystrophy (JATD; Jeune syndrome, MIM 208500) is a rare autosomal recessive chondrodysplasia, phenotypically overlapping with short-rib polydactyly syndromes (SRPS). JATD typical hallmarks include skeletal abnormalities such as narrow chest, shortened ribs, limbs shortened bones, extra fingers and toes (polydactyly), as well as extraskeletal manifestations (renal, liver and retinal disease). To date, disease-causing mutations have been found in several genes, highlighting a marked genetic heterogeneity that prevents a molecular diagnosis of the disease in most families. Here, we report the results of whole-exome sequencing (WES) carried out in four JATD cases, belonging to three unrelated families of Sardinian origin. The exome analysis allowed to identify mutations not previously reported in the DYNC2H1 (MIM 603297) and WDR60 (MIM 615462) genes, both codifying for ciliary intraflagellar transport components whose mutations are known to cause Jeune syndrome. PMID:26874042

  19. Electroencephalogram of Age-Dependent Epileptic Encephalopathies in Infancy and Early Childhood

    PubMed Central

    Wong-Kisiel, Lily C.; Nickels, Katherine

    2013-01-01

    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

  20. 78 FR 72859 - Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-04

    ... spongiform encephalopathy (BSE) risk designations for 14 regions. The OIE recognizes these regions as being... ``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...

  1. Single Sustained Inflation followed by Ventilation Leads to Rapid Cardiorespiratory Recovery but Causes Cerebral Vascular Leakage in Asphyxiated Near-Term Lambs

    PubMed Central

    Sobotka, Kristina S.; Hooper, Stuart B.; Crossley, Kelly J.; Ong, Tracey; Schmölzer, Georg M.; Barton, Samantha K.; McDougall, Annie R. A.; Miller, Suzie L.; Tolcos, Mary; Klingenberg, Claus; Polglase, Graeme R.

    2016-01-01

    Background A sustained inflation (SI) rapidly restores cardiac function in asphyxic, bradycardic newborns but its effects on cerebral haemodynamics and brain injury are unknown. We determined the effect of different SI strategies on carotid blood flow (CaBF) and cerebral vascular integrity in asphyxiated near-term lambs. Methods Lambs were instrumented and delivered at 139 ± 2 d gestation and asphyxia was induced by delaying ventilation onset. Lambs were randomised to receive 5 consecutive 3 s SI (multiple SI; n = 6), a single 30 s SI (single SI; n = 6) or conventional ventilation (no SI; n = 6). Ventilation continued for 30 min in all lambs while CaBF and respiratory function parameters were recorded. Brains were assessed for gross histopathology and vascular leakage. Results CaBF increased more rapidly and to a greater extent during a single SI (p = 0.01), which then decreased below both other groups by 10 min, due to a higher cerebral oxygen delivery (p = 0.01). Blood brain barrier disruption was increased in single SI lambs as indicated by increased numbers of blood vessel profiles with plasma protein extravasation (p = 0.001) in the cerebral cortex. There were no differences in CaBF or cerebral oxygen delivery between the multiple SI and no SI lambs. Conclusions Ventilation with an initial single 30 s SI improves circulatory recovery, but is associated with greater disruption of blood brain barrier function, which may exacerbate brain injury suffered by asphyxiated newborns. This injury may occur as a direct result of the initial SI or to the higher tidal volumes delivered during subsequent ventilation. PMID:26765258

  2. Measles-associated encephalopathy in children with renal transplants.

    PubMed

    Turner, A; Jeyaratnam, D; Haworth, F; Sinha, M D; Hughes, E; Cohen, B; Jin, L; Kidd, I M; Rigden, S P A; MacMahon, E

    2006-06-01

    Two children, boys of 8 and 13 years, presented with measles-associated encephalopathy several years after kidney transplantation for congenital nephrotic syndrome. In the absence of prior clinical measles, the neurological symptoms initially eluded diagnosis, but retrospective analysis of stored samples facilitated the diagnosis of measles-associated encephalopathy without recourse to biopsy of deep cerebral lesions. Each had received a single dose of measles mumps and rubella vaccine before 12 months of age. Prior vaccination, reduction of immunosuppression and treatment with intravenous immunoglobulin and ribavirin may have contributed to their survival. Persistent measles virus RNA shedding, present in one child, was not controlled by treatment with i.v. ribavirin. Two years later, both patients continue to have functioning allografts with only minimal immunosuppression. These cases illustrate the difficulty in diagnosing measles-associated encephalopathy in the immunocompromised host, even in the era of molecular diagnostics, and highlight the renewed threat of neurological disease in communities with incomplete herd immunity.

  3. Useful Tests for Hepatic Encephalopathy in Clinical Practice

    PubMed Central

    Nabi, Eiman; Bajaj, Jasmohan S

    2014-01-01

    Hepatic encephalopathy (HE) is a serious complication of liver disease and portosystemic shunting that represents a continuum of neuropsychiatric changes and altered consciousness. It is classified as overt hepatic encephalopathy (OHE) when clinically apparent or as covert hepatic encephalopathy (CHE) in its mildest form. Progression of CHE to OHE and its impact of quality of life make its early diagnosis imperative. Several diagnostic techniques ranging from simple clinical scales to sophisticated computerized tests exist yet diagnosis remains a challenge due to the time, cost and personnel involved. Psychometric tests appear promising due to their high sensitivity and low cost but results are variable depending on age and education. The pros and cons of current diagnostic methods for overt and covert HE are reviewed along with strategy to CHE testing. PMID:24357348

  4. Perinatal testicular torsion and medicolegal considerations.

    PubMed

    Massoni, F; Troili, G M; Pelosi, M; Ricci, S

    2014-06-01

    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. PMID:24826979

  5. Predictors of neonatal encephalopathy in full-term infants.

    PubMed Central

    Adamson, S. J.; Alessandri, L. M.; Badawi, N.; Burton, P. R.; Pemberton, P. J.; Stanley, F.

    1995-01-01

    OBJECTIVE--Preliminary investigation of the contribution of adverse antepartum and intrapartum factors to neonatal encephalopathy in singleton neonates born full term. DESIGN--Matched case-control study based on incidence density sampling of controls. SETTING--Two major teaching hospitals (one paediatric and one obstetric) and three peripheral maternity hospitals in Perth, Western Australia (population 1.2 million). SUBJECTS--89 cases, all the full term singleton neonates born during an eight month period in 1992 who fulfilled one or more of six criteria during the first week of life (seizures, abnormal conscious state, persistent hypertonia or hypotonia, and feeding or respiratory difficulties of central origin). One full term control infant without neonatal encephalopathy was matched to each case by sex, hospital of delivery, time of day and day of the week of birth, and maternal health insurance status. MAIN OUTCOME MEASURES--Odds ratio estimates of relative risk of neonatal encephalopathy associated with antepartum and intrapartum factors. RESULTS--Estimated incidence of moderate or severe encephalopathy in first week of life was 3.75 per 1000 full term live births. Thirteen cases and no controls had evidence suggestive of important intrapartum hypoxia, and in only five of these cases was the neurological condition at birth attributed to events during the intrapartum period. Univariate conditional logistic regression analysis identified significant differences between cases and controls for maternal vaginal bleeding in pregnancy, maternal thyroxine treatment, congenital abnormalities, induction of labour, interval from membrane rupture to delivery, maternal pyrexia in labour, augmentation of labour, abnormal intrapartum cardiotocograms, and meconium in labour. Family history of convulsions also approached significance. CONCLUSIONS--Our preliminary results suggest that intrapartum hypoxia, according to currently used criteria, was not the cause of neonatal

  6. Gut microbiota: its role in hepatic encephalopathy.

    PubMed

    Rai, Rahul; Saraswat, Vivek A; Dhiman, Radha K

    2015-03-01

    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

  7. Prophylaxis and reversal of ifosfamide encephalopathy with methylene-blue.

    PubMed

    Küpfer, A; Aeschlimann, C; Wermuth, B; Cerny, T

    1994-03-26

    The antineoplastic ifosfamide produces dose-dependent signs of neurotoxicity. After ifosfamide overdose in a patient, we found excessive urinary excretion of glutaric acid and sarcosine, which is compatible with glutaric aciduria type II, a defect in mitochondrial fatty acid oxidation that results from defective electron transfer to flavoproteins. We therefore used the electron-accepting drug methylene-blue as an antidote for ifosfamide encephalopathy. In one patient, ifosfamide neurotoxicity was rapidly reversed by methylene-blue 50 mg intravenously. In another patient with previous episodes of ifosfamide encephalopathy, methylene-blue was administered orally prophylactically. No symptoms of neurotoxicity were noted.

  8. Toxic encephalopathy due to colchicine--Gloriosa superba poisoning.

    PubMed

    Gooneratne, Inuka Kishara; Weeratunga, Praveen; Caldera, Manjula; Gamage, Ranjanie

    2014-10-01

    Gloriosa superba, a flowering plant widespread in South and Southeast Asia, is implicated in many cases of self-poisoning. Colchicine is concentrated in the seeds and tubers and this mediates its toxicity. We describe a 28-year-old woman who developed delayed encephalopathy after eating G superba tubers. MR scan of brain showed bilateral symmetrical T2 basal ganglia hyperintensities in the caudate and lentiform nuclei. The delay in onset of encephalopathy is attributable to a direct-effect colchicine, probably mediated through its effect on microtubular transport.

  9. [Fever-induced refractory epileptic encephalopathy of children].

    PubMed

    Sivkova, S N; Bogdanov, E I; Zaĭkova, F M; Morozova, E A; Aiupova, V A; Zabbarova, A T; Shaĭmardanova, R M

    2013-01-01

    Fever-induced refractory epileptic encephalopathy of school-age children is a rare epileptic syndrome that causes difficulties in diagnosis at the initial stage of disease. It is characterized by sudden onset with multifocal refractory status epilepticus in previously healthy children with normal development. Later, children suffer from resistant focal epilepsy in the combination with cognitive deficit and behavioral difficulties. Authors describe a clinical case of fever-induced refractory epileptic encephalopathy of school-age children in a child of 7 years old. Aspects of etiology, pathogenesis, clinical manifestation, differential diagnosis, treatment and prognosis of the disease are discussed.

  10. Fatal Encephalopathy Associated with Influenza in a Health Care Professional.

    PubMed

    Kitano, Kiyoshi; Furuta, Kiyoshi; Kanai, Shinichiro; Takei, Yo-Ichi

    2016-01-01

    A 41-year-old nurse was referred to our hospital with a fever and disturbed consciousness. She tested positive for influenza antigen. CT and MRI findings revealed low density and intensity areas in the right occipital and lateral lobes with remarkable brain edema, which led to a diagnosis of influenza encephalopathy. Influenza A antibodies in the serum were below the detection limit despite the patient receiving previous vaccination three months earlier. A PCR analysis revealed that the influenza HA gene was classified into clade 3C.2a, subclass AH3N2. The present case indicates the potential development of encephalopathy in adults under certain conditions. PMID:26935378

  11. Neonatal Encephalopathy: Update on Therapeutic Hypothermia and Other Novel Therapeutics.

    PubMed

    McAdams, Ryan M; Juul, Sandra E

    2016-09-01

    Neonatal encephalopathy (NE) is a major cause of neonatal mortality and morbidity. Therapeutic hypothermia (TH) is standard treatment for newborns at 36 weeks of gestation or greater with intrapartum hypoxia-related NE. Term and late preterm infants with moderate to severe encephalopathy show improved survival and neurodevelopmental outcomes at 18 months of age after TH. TH can increase survival without increasing major disability, rates of an IQ less than 70, or cerebral palsy. Neonates with severe NE remain at risk of death or severe neurodevelopmental impairment. This review discusses the evidence supporting TH for term or near term neonates with NE. PMID:27524449

  12. Apgar scores at 10 min and outcomes at 6–7 years following hypoxic-ischaemic encephalopathy

    PubMed Central

    Natarajan, Girija; Shankaran, Seetha; Laptook, Abbot R; Pappas, Athina; Bann, Carla M; McDonald, Scott A; Das, Abhik; Higgins, Rosemary D; Hintz, Susan R; Vohr, Betty R

    2014-01-01

    Aim To determine the association between 10 min Apgar scores and 6–7-year outcomes in children with perinatal hypoxic-ischaemic encephalopathy (HIE) enrolled in the National Institute of Child Health and Human Development Neonatal Research Network (NICHD NRN) whole body cooling randomised controlled trial (RCT). Methods Evaluations at 6–7 years included the Wechsler Preschool and Primary Scale of Intelligence III or Wechsler Intelligence Scale for Children IV and Gross Motor Functional Classification Scale. Primary outcome was death/moderate or severe disability. Logistic regression was used to examine the association between 10 min Apgar scores and outcomes after adjusting for birth weight, gestational age, gender, outborn status, hypothermia treatment and centre. Results In the study cohort (n=174), 64/85 (75%) of those with 10 min Apgar score of 0–3 had death/disability compared with 40/89 (45%) of those with scores >3. Each point increase in 10 min Apgar scores was associated with a significantly lower adjusted risk of death/disability, death, death/IQ <70, death/cerebral palsy (CP) and disability, IQ<70 and CP among survivors (all p<0.05). Among the 24 children with a 10 min Apgar score of 0, five (20.8%) survived without disability. The risk-adjusted probabilities of death/disability were significantly lower in cooled infants with Apgar scores of 0–3; there was no significant interaction between cooling and Apgar scores (p=0.26). Conclusions Among children with perinatal HIE enrolled in the NICHD cooling RCT, 10 min Apgar scores were significantly associated with school-age outcomes. A fifth of infants with 10 min Apgar score of 0 survived without disability to school age, suggesting the need for caution in limiting resuscitation to a specified duration. PMID:23896791

  13. Evaluation and Management of Hepatic Encephalopathy: Current Status and Future Directions

    PubMed Central

    Suraweera, Duminda; Sundaram, Vinay; Saab, Sammy

    2016-01-01

    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

  14. A Perinatal Health Framework for Women with Physical Disabilities

    PubMed Central

    Mitra, Monika; Long-Bellil, Linda M.; Smeltzer, Suzanne C.; Iezzoni, Lisa I.

    2015-01-01

    Background Studies suggest that women with disabilities experience health and health care disparities before, during, and after pregnancy. However, existing perinatal health and health care frameworks do not address the needs and barriers faced by women with physical disabilities around the time of pregnancy. A new framework that addresses the perinatal disparities among women with physical disabilities is needed. Objective To propose a framework for examining perinatal health and health care disparities among women with physical disabilities. Methods We developed a perinatal health framework guided by the International Classification of Functioning, Disability and Health (ICF) and the integrated perinatal health framework by Misra et al. Results The proposed framework uses a life span perspective in a manner that directly addresses the multiple determinants specific to women with physical disabilities around the time of pregnancy. The framework is based on longitudinal and integrated perspectives that take into account women's functional status and environment over their life course. Conclusion The perinatal health framework for women with physical disabilities was developed to inform the way researchers and health care professionals address disparities in perinatal health and health care among women with physical disabilities. PMID:26189010

  15. Encephalopathy after persistent vomiting: Three cases of non-alcohol-related Wernicke's encephalopathy.

    PubMed

    Antel, K; Singh, N; Chisholm, B; Heckmann, J M

    2015-06-01

    Wernicke's encephalopathy (WE) is a medical emergency. Although WE is commonly viewed in the context of alcoholism, it can be caused by thiamine deficiency secondary to persistent vomiting. Non-alcohol-related WE may be more catastrophic in onset and less likely to present with the classic features than WE with alcoholism as a cause. We describe three cases of WE due to persistent vomiting without alcoholism in patients with hyperemesis gravidarum, drug-induced hyperlactataemia, and an acute gastrointestinal illness in an already malnourished individual. Our cases highlight the importance of recognising WE when undernutrition, which may be caused by gastrointestinal disease or surgery, or malignancy, is compounded by vomiting. Expert guidelines suggest that WE must be considered in the emergency room in any individual with disturbed consciousness of unknown cause. Treatment is with parenteral thiamine before glucose administration. PMID:26716155

  16. The Greek National Perinatal Survey. II: Socioeconomic factors and perinatal mortality in Greece.

    PubMed

    Tzoumaka-Bakoula, C; Lekea-Karanika, V; Matsaniotis, N S; Golding, J

    1989-01-01

    Information concerning all 10,859 singleton deliveries in Greece in April 1983, were analysed to assess the contribution of socioeconomic factors to the perinatal mortality rate. Statistically significant associations were initially found with parental education, parental ages, duration of marriage, paternal occupation and parity. There was no association with maternal smoking habit, maternal occupation during pregnancy, type of health insurance or housing conditions. Once logistic regression analyses had taken account of the strong parity effect (P less than 0.0001), only a moderate association with maternal age (P less than 0.05) remained statistically significant, together with a marginally significant (P less than 0.05) association with maternal education level. Mothers who were moderately well educated had the lowest risk of loosing their baby. It is concluded that traditional measures of social deprivation appeared to have little effect on perinatal mortality in Greece in 1983. PMID:2710679

  17. [Perinatal Information System. Incorporation latency and impact on perinatal clinical registry].

    PubMed

    Simini, F; Fernández, A; Sosa, C; Díaz Rossello, J L

    2001-10-01

    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.

  18. [Perinatal Information System. Incorporation latency and impact on perinatal clinical registry].

    PubMed

    Simini, F; Fernández, A; Sosa, C; Díaz Rossello, J L

    2001-10-01

    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. PMID:11816526

  19. Neonatal thyroid function: influence of perinatal factors.

    PubMed Central

    Franklin, R C; Carpenter, L M; O'Grady, C M

    1985-01-01

    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

  20. Perinatal mortality at pre-Columbian Teotihuacan.

    PubMed

    Storey, R

    1986-04-01

    The skeletal population of 166 individuals from a low-status apartment compound of the pre-Columbian city of Teotihuacan contained 52 perinatal individuals. The most perilous time of the lifespan was around birth, as revealed by life table analysis. Femur length was not increasing during the last month of gestation, and individuals were probably shorter somatically at birth than modern standards or historic-period Arikara skeletal controls. The possibility of intrauterine growth retardation is investigated through paleo-pathological indicators of prenatal growth arrest. The evidence of prenatal stress and the high rate of mortality at birth seem to indicate that this New World preindustrial urban population faced similar health and nutritional stresses as Old World preindustrial cities.

  1. Cytomegalovirus myelitis in perinatally acquired HIV.

    PubMed Central

    Güngör, T; Funk, M; Linde, R; Jacobi, G; Horn, M; Kreuz, W

    1993-01-01

    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

  2. Metabolic effects of perinatal asphyxia in the rat cerebral cortex.

    PubMed

    Souza, Samir Khal; Martins, Tiago Leal; Ferreira, Gustavo Dias; Vinagre, Anapaula Sommer; Silva, Roselis Silveira Martins da; Frizzo, Marcos Emilio

    2013-03-01

    We reported previously that intrauterine asphyxia acutely affects the rat hippocampus. For this reason, the early effects of this injury were studied in the cerebral cortex, immediately after hysterectomy (acute condition) or following a recovery period at normoxia (recovery condition). Lactacidemia and glycemia were determined, as well as glycogen levels in the muscle, liver and cortex. Cortical tissue was also used to assay the ATP levels and glutamate uptake. Asphyxiated pups exhibited bluish coloring, loss of movement, sporadic gasping and hypertonia. However, the appearance of the controls and asphyxiated pups was similar at the end of the recovery period. Lactacidemia and glycemia were significantly increased by asphyxia in both the acute and recovery conditions. Concerning muscle and hepatic glycogen, the control group showed significantly higher levels than the asphyxic group in the acute condition and when compared with groups of the recovery period. In the recovery condition, the control and asphyxic groups showed similar glycogen levels. However, in the cortex, the control groups showed significantly higher glycogen levels than the asphyxic group, in both the acute and recovery conditions. In the cortical tissue, asphyxia reduced ATP levels by 70 % in the acute condition, but these levels increased significantly in asphyxic pups after the recovery period. Asphyxia did not affect glutamate transport in the cortex of both groups. Our results suggest that the cortex uses different energy resources to restore ATP after an asphyxia episode followed by a reperfusion period. This strategy could sustain the activity of essential energy-dependent mechanisms. PMID:23196669

  3. Bovine spongiform encephalopathy in Sweden: an H-type variant

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  4. Neuropsychiatric Manifestation of Hashimoto's Encephalopathy in an Adolescent and Treatment.

    PubMed

    Ransing, Ramdas Sarjerao; Mishra, Kshirod Kumar; Sarkar, Dipayan

    2016-01-01

    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

  5. Recurring hyperammonemic encephalopathy induced by bacteria usually not producing urease

    PubMed Central

    2014-01-01

    Background Hyperammonemic encephalopathy may occur when urease-positive bacteria in the urinary tract produce ammonium which directly enters systemic circulation. Predisposing conditions such as a neurogenic bladder can increase both urinary tract infection and urine stagnation. Case presentation We describe the case of a 66 years old woman with a neurogenic bladder who twice developed hyperammonemic encephalopathy following urinary tract infection. During the second episode Escherichia coli and Enterococcus faecalis have been isolated in the urine. The neurologic examination showed psychomotor slowing, weak photomotor reflex, nystagmus in the lateral gaze and asterixis. The EEG showed triphasic waves which disappeared along with clinical recovery. Conclusion Escherichia coli and Enterococcus faecalis are commonly considered urease-negative bacteria. Although frequently involved in urinary tract infections, their role in causing hyperammonemic encephalopathy have not been previously reported. Moreover, despite only one case with a neurogenic bladder have been described so far, our is the first patient with reoccurring hyperammonemic encephalopathy secondary to urinary tract infections. PMID:24884855

  6. Acute encephalopathy and tubulointerstitial nephritis associated with Yersinia pseudotuberculosis.

    PubMed

    Kaito, Hiroshi; Kamei, Koichi; Ogura, Masao; Kikuchi, Eriko; Hoshino, Hideki; Nakagawa, Satoshi; Matsuoka, Kentaro; Abe, Jun; Ito, Shuichi

    2012-12-01

    We report the case of a 28-month-old boy with encephalopathy and acute tubulointerstitial nephritis possibly associated with Yersinia pseudotuberculosis (Yp) infection. He was transferred to our center because of impairment of renal function and altered consciousness. He had fever for 5 days after recurrent vomiting and diarrhea. Computed tomography scan was normal, but electroencephalogram (EEG) analyses showed generalized slow wave patterns. Continuous hemodialysis was undergone and then his renal function was improved, but altered consciousness persisted. Single photon emission computed tomography (SPECT) revealed abnormally low signals at entire field, which suggested that he was suffered from encephalopathy. Phenobarbital administration and post-encephalopathy rehabilitation were started, and he recovered in fully premorbid state with normal EEG and SPECT findings on the 33rd hospital day. Various bacterial cultures were negative, but both Yp antibody and Yp-derived mitogen (YPM) antibody, the antibody of a specific Yp exotoxin, had an extremely high titer. This is the first report of encephalopathy potentially caused by Yp, indicated by the presence of a high Yp and YPM antibody titer.

  7. A rare case of glycine encephalopathy unveiled by valproate therapy.

    PubMed

    Subramanian, Velusamy; Kadiyala, Pramila; Hariharan, Praveen; Neeraj, E

    2015-01-01

    Glycine encephalopathy (GE) or nonketotic hyperglycinemia is an autosomal recessive disorder due to a primary defect in glycine cleavage enzyme system. It is characterized by elevated levels of glycine in plasma and cerebrospinal fluid usually presenting with seizures, hypotonia, and developmental delay. In our case, paradoxical increase in seizure frequency on starting sodium valproate led us to diagnose GE. PMID:26167219

  8. Neuropsychiatric Manifestation of Hashimoto's Encephalopathy in an Adolescent and Treatment

    PubMed Central

    Ransing, Ramdas Sarjerao; Mishra, Kshirod Kumar; Sarkar, Dipayan

    2016-01-01

    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

  9. Collagen defects in lethal perinatal osteogenesis imperfecta.

    PubMed

    Bateman, J F; Chan, D; Mascara, T; Rogers, J G; Cole, W G

    1986-12-15

    Quantitative and qualitative abnormalities of collagen were observed in tissues and fibroblast cultures from 17 consecutive cases of lethal perinatal osteogenesis imperfecta (OI). The content of type I collagen was reduced in OI dermis and bone and the content of type III collagen was also reduced in the dermis. Normal bone contained 99.3% type I and 0.7% type V collagen whereas OI bone contained a lower proportion of type I, a greater proportion of type V and a significant amount of type III collagen. The type III and V collagens appeared to be structurally normal. In contrast, abnormal type I collagen chains, which migrated slowly on electrophoresis, were observed in all babies with OI. Cultured fibroblasts from five babies produced a mixture of normal and abnormal type I collagens; the abnormal collagen was not secreted in two cases and was slowly secreted in the others. Fibroblasts from 12 babies produced only abnormal type I collagens and they were also secreted slowly. The slower electrophoretic migration of the abnormal chains was due to enzymic overmodification of the lysine residues. The distribution of the cyanogen bromide peptides containing the overmodified residues was used to localize the underlying structural abnormalities to three regions of the type I procollagen chains. These regions included the carboxy-propeptide of the pro alpha 1(I)-chain, the helical alpha 1(I) CB7 peptide and the helical alpha 1(I) CB8 and CB3 peptides. In one baby a basic charge mutation was observed in the alpha 1(I) CB7 peptide and in another baby a basic charge mutation was observed in the alpha 1(I) CB8 peptide. The primary defects in lethal perinatal OI appear to reside in the type I collagen chains. Type III and V collagens did not appear to compensate for the deficiency of type I collagen in the tissues.

  10. Ethical issues in perinatal mental health.

    PubMed

    Miller, Laura J

    2009-06-01

    The principles of autonomy, beneficence, nonmaleficence, and justice can guide clinicians in finding ethical approaches to the treatment of women who have psychiatric disorders during preconception, pregnancy, and postpartum. Table 1 summarizes some clinical dilemmas in perinatal mental health care, the ethical conundrums posed by these situations, and guiding principles or tools that can help clinicians resolve ethical conflicts. The concept of relational ethics helps resolve apparent mother-offspring ethical conflicts, and the practice of preventive ethics helps anticipate and reduce the risk of ethical dilemmas and adverse clinical outcomes. These central principles suggest the following guidelines in caring for perinatal women: In situations that seem to pit the needs of a pregnant or postpartum woman against the needs of her fetus or baby, reframe the problem to find a solution that most benefits the mother-baby dyad while posing the least risk to the dyad. In evaluating a pregnant woman's ability to make autonomous, informed decisions about medical care, assess her ability to decide on behalf of both herself and her fetus. When explaining the risks of treatments such as psychotropic medication during pregnancy, avoid errors of omission by also explaining the risks of withholding the treatments. Apply the principle of justice to ensure that women are not stigmatized by having psychiatric disorders or by being pregnant. When screening for maternal psychiatric symptoms, ensure that the benefits of screening outweigh the ethical costs by designing effective follow-up systems for helping women who have positive screens. When treating women of reproductive age for psychiatric disorders, proactively discuss family planning and, when appropriate, the anticipated risks of the illness and the treatment during future pregnancies. Offer preventive interventions to reduce these risks. PMID:19486812

  11. Metformin Inhibits Glutaminase Activity and Protects against Hepatic Encephalopathy

    PubMed Central

    Ampuero, Javier; Ranchal, Isidora; Nuñez, David; Díaz-Herrero, María del Mar; Maraver, Marta; del Campo, José Antonio; Rojas, Ángela; Camacho, Inés; Figueruela, Blanca; Bautista, Juan D.; Romero-Gómez, Manuel

    2012-01-01

    Aim To investigate the influence of metformin use on liver dysfunction and hepatic encephalopathy in a retrospective cohort of diabetic cirrhotic patients. To analyze the impact of metformin on glutaminase activity and ammonia production in vitro. Methods Eighty-two cirrhotic patients with type 2 diabetes were included. Forty-one patients were classified as insulin sensitizers experienced (metformin) and 41 as controls (cirrhotic patients with type 2 diabetes mellitus without metformin treatment). Baseline analysis included: insulin, glucose, glucagon, leptin, adiponectin, TNFr2, AST, ALT. HOMA-IR was calculated. Baseline HE risk was calculated according to minimal hepatic encephalopathy, oral glutamine challenge and mutations in glutaminase gene. We performed an experimental study in vitro including an enzymatic activity assay where glutaminase inhibition was measured according to different metformin concentrations. In Caco2 cells, glutaminase activity inhibition was evaluated by ammonia production at 24, 48 and 72 hours after metformina treatment. Results Hepatic encephalopathy was diagnosed during follow-up in 23.2% (19/82): 4.9% (2/41) in patients receiving metformin and 41.5% (17/41) in patients without metformin treatment (logRank 9.81; p = 0.002). In multivariate analysis, metformin use [H.R.11.4 (95% CI: 1.2–108.8); p = 0.034], age at diagnosis [H.R.1.12 (95% CI: 1.04–1.2); p = 0.002], female sex [H.R.10.4 (95% CI: 1.5–71.6); p = 0.017] and HE risk [H.R.21.3 (95% CI: 2.8–163.4); p = 0.003] were found independently associated with hepatic encephalopathy. In the enzymatic assay, glutaminase activity inhibition reached 68% with metformin 100 mM. In Caco2 cells, metformin (20 mM) decreased glutaminase activity up to 24% at 72 hours post-treatment (p<0.05). Conclusions Metformin was found independently related to overt hepatic encephalopathy in patients with type 2 diabetes mellitus and high risk of hepatic encephalopathy. Metformin

  12. CADASIL: Migraine, Encephalopathy, Stroke and Their Inter-Relationships

    PubMed Central

    Markus, Hugh Stephen

    2016-01-01

    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

  13. SCN8A encephalopathy: Research progress and prospects.

    PubMed

    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

    2016-07-01

    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. PMID:27270488

  14. The burden of hepatic encephalopathy in Latin America.

    PubMed

    Dávalos Moscol, Milagros; Bustios Sanchez, Carla

    2011-06-01

    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.

  15. Risk of Remote Seizures After Perinatal Ischemic Stroke.

    PubMed

    Ritacco, David G

    2016-08-01

    Investigators from the University of California San Francisco and Kaiser Permanente Medical Centers in Oakland report outcome of perinatal arterial ischemic stroke (PAIS) in a population-based cohort, with particular attention to the incidence of seizures. PMID:27649623

  16. High-tech, high-touch perinatal home care.

    PubMed

    Dahlberg, N L; Blazek, D; Wikoff, B; Tuckwell, B L; Koloroutis, M

    1995-05-01

    Perinatal home care for women experiencing a high-risk pregnancy often requires the use of technologies for safe home management. Home care professionals need to integrate high technology with high touch to ensure the best results.

  17. Contribution of congenital malformation to perinatal mortality in Lagos, Nigeria.

    PubMed

    Abudu, O O; Uguru, V; Olude, O

    1988-08-01

    Over a 17-month period we prospectively recorded identifiable congenital malformations at delivery in singleton births in our hospital. Despite the prevailing religious and cultural belief we carried out autopsies in 41% of the perinatal deaths that occurred during the study period. Out of a total of 63 (21/1000 singleton births) congenital malformations discovered, 21 (33%) were identified at autopsy only. About 16% of total perinatal deaths were due to congenital malformation. Cardiovascular malformations accounted for about 40% of perinatal deaths from congenital malformations followed by central nervous system malformation (23.3%), gastrointestinal malformations (20%), musculo-skeletal malformations (6.7%); renal malformations (3.3%) and others (6.7%). No relationship between maternal age, parity and congenital malformation was found. The results from this study suggest that with the use of autopsy, teratology may contribute significantly to the prevailing high perinatal mortality in Lagos more than was previously thought. PMID:2905300

  18. Optimizing the treatment of mood disorders in the perinatal period.

    PubMed

    Meltzer-Brody, Samantha; Jones, Ian

    2015-06-01

    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.

  19. Prevention of perinatal transmission of human immunodeficiency virus.

    PubMed

    Chappell, Catherine A; Cohn, Susan E

    2014-12-01

    The reproductive health needs of all women of childbearing age should routinely address effective and appropriate contraception, safer sex practices, and elimination of alcohol, illicit drugs and tobacco should pregnancy occur. Combined antepartum, intrapartum, and infant antiretroviral (ARV) prophylaxis are recommended because ARV drugs reduce perinatal transmission by several mechanisms, including lowering maternal viral load and providing infant pre- and post-exposure prophylaxis. Scheduled cesarean delivery at 38 weeks with IV AZT decreases the risk of perinatal transmission if the HIV RNA is greater than 1000 copies/mL or if HIV levels are unknown near the time of delivery. Oral AZT should generally be given for at least 6 weeks to all infants perinatally exposed to HIV to reduce perinatal transmission of HIV. PMID:25455313

  20. Genetics of perinatal brain injury in the preterm infant.

    PubMed

    Baier, Ronald John

    2006-05-01

    Due to developmental immaturity of the central nervous system, effects of an adverse intrauterine environment and need for intensive care postnatally, preterm infants are at high risk of sustaining brain injury in the perinatal period. Infants who suffer brain injury in the perinatal period are at risk for long-term neurodevelopmental sequelae. Clinical and experimental data supports a significant role for inflammatory mediators in the pathophysiology of perinatal brain injury. Abnormalities in coagulation proteins in the sick preterm newborn may accentuate the risk for intraventricular hemorrhage. Polymorphisms in TNF alpha , IL-1 beta , IL-4, IL-6 and IL-10 as well as mutations in coagulation proteins have been investigated as potential candidate genes to modify risk and or severity of perinatal brain injury. Preliminary evidence suggests a role for cytokine genes as risk modifiers for IVH and PVL.

  1. Interrelationship Between Broadband NIRS Measurements of Cerebral Cytochrome C Oxidase and Systemic Changes Indicates Injury Severity in Neonatal Encephalopathy.

    PubMed

    Bale, Gemma; Mitra, Subhabrata; de Roever, Isabel; Chan, Marcus; Caicedo-Dorado, Alexander; Meek, Judith; Robertson, Nicola; Tachtsidis, Ilias

    2016-01-01

    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. PMID:27526141

  2. Implementation in Buenos Aires City of a program to prevent neurological damage caused by hypoxic-ischemic encephalopathy: Therapeutic hypothermia.

    PubMed

    Valera, Mariana; Berazategui, Juan Pablo; Saa, Gladys; Olmo Herrera, Carolina; Sepúlveda, Teresa; Buraschi, María Fernanda; Gacio, Sebastián; Villalba, Cristina; Beloso, Inés; Basso, Graciela; Carlo, Waldemar; Tavosnanska, Jorge

    2015-10-01

    Therapeutic hypothermia is the standard of care for hypoxic-ischemic encephalopathy (HIE). This treatment was implemented at a regional level by the perinatal network of the City of Buenos Aires. The following are the objectives of this article: 1. To describe the implementation of the network's hypothermia treatment program; 2. To report treatment-associated complications, adverse events and mortality. The program was implemented in stages: 1) 2009-2010. Training and instruction on how to use the equipment. 2) 20102014. Treatment and follow-up of patients with moderate or severe HIE. Up to October 2014, 27 newborn infants received hypothermia treatment with moderate (n= 15) and severe (n= 12) HIE. None of the patients died during treatment. Three newborn infants were lost to follow-up. Among the 16 survivors older than one year old, three have severe neurological disability. Program implementation was plausible. It is imperative to train health care providers on how to identify patients with HIE. PMID:26294149

  3. Pathogenesis and new candidate treatments for infantile spasms and early life epileptic encephalopathies: A view from preclinical studies.

    PubMed

    Galanopoulou, Aristea S; Moshé, Solomon L

    2015-07-01

    Early onset and infantile epileptic encephalopathies (EIEEs) are usually associated with medically intractable or difficult to treat epileptic seizures and prominent cognitive, neurodevelopmental and behavioral consequences. EIEEs have numerous etiologies that contribute to the inter- and intra-syndromic phenotypic variability. Etiologies include structural and metabolic or genetic etiologies although a significant percentage is of unknown cause. The need to better understand their pathogenic mechanisms and identify better therapies has driven the development of animal models of EIEEs. Several rodent models of infantile spasms have emerged that recapitulate various aspects of the disease. The acute models manifest epileptic spasms after induction and include the NMDA rat model, the NMDA model with prior prenatal betamethasone or perinatal stress exposure, and the γ-butyrolactone induced spasms in a mouse model of Down syndrome. The chronic models include the tetrodotoxin rat model, the aristaless related homeobox X-linked (Arx) mouse models and the multiple-hit rat model of infantile spasms. We will discuss the main features and findings from these models on target mechanisms and emerging therapies. Genetic models have also provided interesting data on the pathogenesis of Dravet syndrome and proposed new therapies for testing. The genetic associations of many of the EIEEs have also been tested in rodent models as to their pathogenicity. Finally, several models have tested the impact of subclinical epileptiform discharges on brain function. The impact of these advances in animal modeling for therapy development will be discussed. PMID:25968935

  4. Pathogenesis and new candidate treatments for infantile spasms and early life epileptic encephalopathies: a view from preclinical studies

    PubMed Central

    Galanopoulou, Aristea S.; Moshé, Solomon L.

    2015-01-01

    Early onset and infantile epileptic encephalopathies (EIEEs) are usually associated with medically intractable or difficult to treat epileptic seizures and prominent cognitive, neurodevelopmental and behavioral consequences. EIEEs have numerous etiologies that contribute to the inter- and intra-syndromic phenotypic variability. Etiologies include structural and metabolic or genetic etiologies although a significant percentage is of unknown cause. The need to better understand their pathogenic mechanisms and identify better therapies has driven the development of animal models of EIEEs. Several rodent models of infantile spasms have emerged that recapitulate various aspects of the disease. The acute models manifest epileptic spasms after induction and include the NMDA rat model, the NMDA model with prior prenatal betamethasone or perinatal stress exposure, and the γ-butyrolactone induced spasms in a mouse model of Down syndrome. The chronic models include the tetrodotoxin rat model, the aristaless related homeobox X-linked (Arx) mouse models and the multiple-hit rat model of infantile spasms. We will discuss the main features and findings from these models on target mechanisms and emerging therapies. Genetic models have also provided interesting data on the pathogenesis of Dravet syndrome and proposed new therapies for testing. The genetic associations of many of the EIEEs have also been tested in rodent models as to their pathogenicity. Finally, several models have tested the impact of subclinical epileptiform discharges on brain function. The impact of these advances in animal modeling for therapy development will be discussed. PMID:25968935

  5. Epigenetics and fetal adaptation to perinatal events: diversity through fidelity.

    PubMed

    Joss-Moore, L A; Metcalfe, D B; Albertine, K H; McKnight, R A; Lane, R H

    2010-04-01

    Perinatal insults, including fetal undernutrition and hypoxia, are associated with an increased susceptibility to several adult-onset metabolic disorders. These include cardiovascular disease, insulin resistance, and obesity. However, the mechanisms driving the long-term phenotypic consequences have only recently begun to be elucidated. A primary mechanism accounting for perinatal adaptation is the epigenetic modification of chromatin. In this context, epigenetic modifications to chromatin are thought to arise in response to a perinatal insult in an effort to modulate gene expression and maximize fetal survival. In this symposium report, we discuss epigenetics as a mechanism by which perinatal adaptations can be made by the developing fetus. We examine the benefits of using multiple in vivo models to understand the interrelation of signals that come together and result in perinatal adaptation. Epigenetic effects on IGF-1 arising from a perinatal insult are discussed, as are the difficulties and challenges associated with this complex field. In conclusion, epigenetics provides a means of modulating gene transcription, thus allowing fetal adaptation to a broad variety of conditions. PMID:19854998

  6. Clinical manifestations and treatment response of steroid in pediatric Hashimoto encephalopathy.

    PubMed

    Yu, Hee Joon; Lee, Jeehun; Seo, Dae Won; Lee, Munhyang

    2014-07-01

    Hashimoto encephalopathy is a steroid-responsive encephalopathy associated with elevated titers of antithyroid antibodies. Clinical symptoms are characterized by behavioral and cognitive changes, speech disturbance, seizures, myoclonus, psychosis, hallucination, involuntary movements, cerebellar signs, and coma. The standard treatment is the use of corticosteroids along with the treatment of any concurrent dysthyroidism. Other options are immunoglobulins and plasmapheresis. We described symptoms and outcomes on 3 teenage girls with Hashimoto encephalopathy. Presenting symptoms were seizure or altered mental status. One patient took levothyroxine due to hypothyroidism before presentation of Hashimoto encephalopathy. After confirmation of elevated antithyroid antibodies, all patients were treated with steroids. One patient needed plasmapheresis because of the lack of response to steroids and immunoglobulins. Hashimoto encephalopathy should be considered in any patient presenting with acute or subacute unexplained encephalopathy and seizures. Even though the use of steroids is the first line of treatment, plasmapheresis can rescue steroid-resistant patients.

  7. Exclusion of the Ellis-van Creveld region on chromosome 4p16 in some families with asphyxiating thoracic dystrophy and short-rib polydactyly syndromes.

    PubMed

    Krakow, D; Salazar, D; Wilcox, W R; Rimoin, D L; Cohn, D H

    2000-08-01

    Ellis-van Creveld syndrome (EVC) is a relatively rare, usually non-lethal, autosomal recessive skeletal dysplasia characterized by short stature, polydactyly, cardiac and renal anomalies. Linkage analysis has localized the disease gene to chromosome 4p16, with the markers at loci D4S827 and D4S3135 defining the centromeric and telomeric limits of the linked interval, respectively. There has been long-term speculation that asphyxiating thoracic dystrophy (ATD) and the short-rib polydactyly syndromes (SRP) represent the severe end of the EVC disease spectrum. We performed linkage analysis using markers from the EVC region in seven families manifesting either ATD or SRP type III. In two of the families, one segregating ATD and one SRP kindred, linkage of the phenotype to the EVC region was excluded. In the other five families linkage of the phenotype to the EVC region could not be excluded, but the families were too small for linkage to the region to be established. The exclusion of the EVC region in ATD and SRP III families suggests that locus heterogeneity exists within the short-rib dysplasia (with and without polydactyly) group of disorders.

  8. 20-Hydroxyeicosatetraenoic Acid Inhibition by HET0016 Offers Neuroprotection, Decreases Edema, and Increases Cortical Cerebral Blood Flow in a Pediatric Asphyxial Cardiac Arrest Model in Rats

    PubMed Central

    Shaik, Jafar Sadik B; Poloyac, Samuel M; Kochanek, Patrick M; Alexander, Henry; Tudorascu, Dana L; Clark, Robert SB; Manole, Mioara D

    2015-01-01

    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. PMID:26058691

  9. D-cycloserine 24 and 48 hours after asphyxial cardiac arrest has no effect on hippocampal CA1 neuropathology

    PubMed Central

    Combs, Vélvá M; Crispell, Heather D; Drew, Kelly L

    2014-01-01

    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). PMID:25099755

  10. Long-term increase in coherence between the basal ganglia and motor cortex after asphyxial cardiac arrest and resuscitation in developing rats

    PubMed Central

    Aravamuthan, Bhooma R.; Shoykhet, Michael

    2016-01-01

    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

  11. Perinatal epidemiological risk factors for preeclampsia.

    PubMed

    Bobić, Mirna Vuković; Habek, Dubravko; Habek, Jasna Čerkez

    2015-03-01

    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.

  12. Ethical issues of perinatal human gene therapy.

    PubMed

    Fletcher, J C; Richter, G

    1996-01-01

    This paper examines some key ethical issues raised by trials of human gene therapy in the perinatal period--i.e., in infants, young children, and the human fetus. It describes five resources in ethics for researchers' considerations prior to such trials: (1) the history of ethical debate about gene therapy, (2) a literature on the relevance of major ethical principles for clinical research, (3) a body of widely accepted norms and practices, (4) knowledge of paradigm cases, and (5) researchers' own professional integrity. The paper also examines ethical concerns that must be met prior to any trial: benefits to and safety of subjects, informed assent of children and informed parental permission, informed consent of pregnant women in fetal gene therapy, protection of privacy, and concerns about fairness in the selection of subjects. The paper criticizes the position that cases of fetal gene therapy should be restricted only to those where the pregnant woman has explicitly refused abortion. Additional topics include concerns about genetic enhancement and germ-line gene therapy.

  13. Perinatal induction of Cre recombination with tamoxifen.

    PubMed

    Lizen, Benoit; Claus, Melissa; Jeannotte, Lucie; Rijli, Filippo M; Gofflot, Françoise

    2015-12-01

    Temporal control of site-specific recombination is commonly achieved by using a tamoxifen-inducible form of Cre or Flp recombinases. Although powerful protocols of induction have been developed for gene inactivation at adult stages or during embryonic development, induction of recombination at late gestational or early postnatal stages is still difficult to achieve. In this context, using the ubiquitous CMV-CreER(T2) transgenic mice, we have tested and validated two procedures to achieve recombination just before and just after birth. The efficiency of recombination was evaluated in the brain, which is known to be more problematic to target. For the late gestation treatment with tamoxifen, different protocols of complementary administration of progesterone and estrogen were tested. However, delayed delivery and/or mortality of pups due to difficult delivery were always observed. To circumvent this problem, pups were collected from tamoxifen-treated pregnant dams by caesarian section at E18.5 and given to foster mothers. For postnatal treatment, different dosages of tamoxifen were administered by intragastric injection to the pups during 3 or 4 days after birth. The efficiency of these treatments was analyzed at P7 using a transgenic reporter line. They were also validated with the Hoxa5 conditional allele. In conclusion, we have developed efficient procedures that allow achieving efficient recombination of floxed alleles at perinatal stages. These protocols will allow investigating the late/adult functions of many developmental genes, whose characterization has been so far restricted to embryonic development. PMID:26395370

  14. Normoammonemic encephalopathy: solely valproate induced or multiple mechanisms?

    PubMed Central

    Budhdeo, Sanjay; Marquette, Malcolm; Singh, Deepwant; Rajagopal, Vivek

    2014-01-01

    A 77-year-old woman presented with subacute onset progressive confusion, aggression, auditory hallucinations and delusions. In the preceding months, the patient had a number of admissions with transient unilateral hemiparesis with facial droop, and had been started on valproate for presumed hemiplegic migraine. Valproate was withdrawn soon after admission and her cognitive abilities have gradually improved over 3 months of follow-up. Valproate levels taken prior to withdrawal were subtherapeutic and the patient was normoammonaemic. EEG undertaken during inpatient stay showed changes consistent with encephalopathy, and low titre N-methyl-d-aspartate (NMDA) receptor antibodies were present in this patient. The possible aetiologies of valproate-induced encephalopathy and NMDA receptor-associated encephalitis present a diagnostic dilemma. We present a putative combinatorial hypothesis to explain this patient's symptoms. PMID:24614773

  15. Pathophysiology of septic encephalopathy--an unsolved puzzle.

    PubMed

    Flierl, Michael A; Rittirsch, Daniel; Huber-Lang, Markus S; Stahel, Philip F

    2010-01-01

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

  16. Severe valproate induced hyperammonemic encephalopathy successfully managed with peritoneal dialysis.

    PubMed

    Kumar, Amandeep; Suri, Ashish; Sharma, Bhawani S

    2014-07-01

    Valproic acid (VPA) is a commonly used drug for epilepsy, psychiatric disorders and migraine and is frequently used in neurosurgical intensive care units. Though most of its side-effects are mild and transient, certain idiosyncratic side-effects have been attributed to VPA. Valproate induced hyperammonemia (VIH) is one such side-effect. VIH can produce symptoms of encephalopathy known as valproate induced hyperammonemic encephalopathy (VHE). VIH and VHE usually respond to withdrawal of VPA. However, in some cases VHE can be unresponsive to supportive measures and severe enough to be life-threatening. In such cases, dialysis can be used to rapidly reverse hyperammonemia and VHE and can prove to be a lifesaving measure. We report such a case of VIH and life-threatening VHE in a postoperative neurosurgical patient that was managed successfully with peritoneal dialysis.

  17. Nutritional support in the treatment of chronic hepatic encephalopathy.

    PubMed

    Milke García, María del Pilar

    2011-06-01

    The prevalence of under nutrition in cirrhotic patients is 61% and it usually progresses as the disease becomes more advanced. The deterioration in the nutritional status and its associated metabolic derangements has raised doubts about the benefits of severe and prolonged protein restriction as a treatment for hepatic encephalopathy. However, the practice of dietary protein restriction for patients with liver cirrhosis is deeply embedded among medical practitioners and dietitians. To date, no solid conclusions may be drawn about the benefit of protein restriction. However, the negative effects of protein restriction are clear, that is, increased protein catabolism, the release of amino acids from the muscle, and possible worsening of hepatic encephalopathy. In conclusion, chronic protein restriction causes progressive and harmful protein depletion and must be avoided. PMID:22228881

  18. [Diabetic encephalopathy: an underexposed complication of diabetes mellitus].

    PubMed

    Brands, A M A; Henselmans, J M L; de Haan, E H F; Biessels, G J

    2003-01-01

    Diabetes mellitus seems to be associated with gradually developing end-organ damage to the central nervous system. This relatively unknown complication of both diabetes type 1 and type 2 can be referred to as 'diabetic encephalopathy'. Measurable manifestations are electrophysiological and structural changes and limitations in the cognitive functioning. The mechanisms responsible for this diabetic encephalopathy are only partially known. Chronic metabolic and vascular changes seem to play an important role. The effects of diabetes on the brain are most distinct in the elderly. This may be the consequence of interactions between the mechanisms that underlie the ageing of the brain, dementia and the origin of diabetic complications. At present there are few leads for the targeted diagnostics and treatment of individual patients.

  19. Hepatic Encephalopathy: From the Pathogenesis to the New Treatments

    PubMed Central

    Cordoba, Juan

    2014-01-01

    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

  20. [RENDU-OSLER DISEASE: A RARE CAUSE OF AMMONIA ENCEPHALOPATHY].

    PubMed

    Dumont, R; Loly, J-P; Delwaide, J; Louis, E

    2016-02-01

    Hereditary Hemorrhagic Telangiectasia (HHT) also known as Rendu-Osler disease is a group of related disorders inherited in an autosomal dominant fashion and characterized by the development of arteriovenous malformations (AVM) in the skin, mucous membranes, and/or internal organs such as the brain, lungs, and liver. The prevalence of liver involvement is clinically estimated between 8 and 31 percent. It can be revealed by the following clinical signs : ascites, edema of the lower extremities, abdominal pain, dyspnea, and, rarely, hepatic encephalopathy and gastrointestinal bleeding associated with portal hypertension. This case illustrates the highlight of liver damage revealed by an ammonia encephalopathy associated with iconographic anomalies on ultrasonography and magnetic resonance liver as part of Rendu-Osler disease. PMID:27141651

  1. Laboratory activities involving transmissible spongiform encephalopathy causing agents

    PubMed Central

    Leunda, Amaya; Van Vaerenbergh, Bernadette; Baldo, Aline; Roels, Stefan; Herman, Philippe

    2013-01-01

    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

  2. Vogt-Koyanagi-Harada syndrome presenting with encephalopathy

    PubMed Central

    Naeini, Alireza E.; Daneshmand, Dana; Khorvash, Farzin; Chitsaz, Ahmad

    2014-01-01

    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

  3. Contributions of Microdialysis to New Alternative Therapeutics for Hepatic Encephalopathy

    PubMed Central

    Rivera-Espinosa, Liliana; Floriano-Sánchez, Esaú; Pedraza-Chaverrí, José; Coballase-Urrutia, Elvia; Sampieri, Aristides; Ortega-Cuellar, Daniel; Cárdenas-Rodríguez, Noemí; Carmona-Aparicio, Liliana

    2013-01-01

    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

  4. Contributions of microdialysis to new alternative therapeutics for hepatic encephalopathy.

    PubMed

    Rivera-Espinosa, Liliana; Floriano-Sánchez, Esaú; Pedraza-Chaverrí, José; Coballase-Urrutia, Elvia; Sampieri, Aristides Iii; Ortega-Cuellar, Daniel; Cárdenas-Rodríguez, Noemí; Carmona-Aparicio, Liliana

    2013-08-05

    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.

  5. Guest Editorial: Managing Bovine Spongiform Encephalopathy Risk in the United States.

    PubMed

    McCarty, Rick

    2002-01-01

    The United States has built a triple firewall system to prevent the introduction or emergence of bovine spongiform encephalopathy. The firewalls are preventing bovine spongiform encephalopathy's introduction through bans on imports of animals and animal products, actively looking for bovine spongiform encephalopathy in the US cattle herd, and banning ruminant animal feed supplements found to carry the infectious agent and spread the disease in England. PMID:11984425

  6. Brainstem variant of posterior reversible encephalopathy syndrome: A case report.

    PubMed

    Tortora, Fabio; Caranci, Ferdinando; Belfiore, Maria Paola; Manzi, Francesca; Pagliano, Pasquale; Cirillo, Sossio

    2015-12-01

    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.

  7. Subcortical arteriosclerotic encephalopathy: a clinical and radiological investigation.

    PubMed Central

    Loizou, L A; Kendall, B E; Marshall, J

    1981-01-01

    Subcortical arteriosclerotic encephalopathy (Binswanger's type) was diagnosed in a group of patients with hypertension or arteriosclerosis, who showed acute and subacute neurological deficits, dementia, reduced cerebral blood flow, and white matter low attenuation with mild atrophy and infarcts as the predominant CT scan features. This set of clinical and radiological criteria could be used to make the diagnosis in life, as confirmed neuropathologically in one patient. Images PMID:7241156

  8. Epileptic encephalopathies: Optimizing seizure control and developmental outcome.

    PubMed

    Jehi, Lara; Wyllie, Elaine; Devinsky, Orrin

    2015-10-01

    Cognitive and developmental outcomes in patients with epileptic encephalopathy are hypothesized to result from an interplay between the underlying epileptic pathologic substrate and the acquired consequences of frequent and repetitive seizures and epileptiform discharges that often straddle the interictal and ictal boundaries. This article briefly reviews the evidence related to this assumption, presents critical questions that need to be answered to clarify this relationship, and advances a set of concrete steps that may help improve developmental patient outcomes. PMID:26293588

  9. [Transmissible spongiform encephalopathies: neuroinfections with unconventional immune reactions].

    PubMed

    Mitrová, E

    1997-04-01

    Transmissible spongiform encephalopathies (TSE) as well as the properties of the major component of the infectious agent-prion, and the most important human and animal prion diseases are characterized. Considering the recent biochemical and molecular biological data, possible explanations of natural resistance, species barrier and lack of the immune response to the unconventional infectious particles are presented. Finally the importance of immunoblotting and immunostaining as the most specific confirmation of TSE diagnosis is underlined. (Ref. 11.)

  10. Approach to clinical syndrome of jaundice and encephalopathy in tropics.

    PubMed

    Anand, Anil C; Garg, Hitendra K

    2015-03-01

    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.

  11. Acute febrile encephalopathy in adults from Northwest India

    PubMed Central

    Bhalla, Ashish; Suri, Vika; Varma, Subhash; Sharma, Navneet; Mahi, Sushil; Singh, Paramjeet; Khandelwal, Niranjan K

    2010-01-01

    Background: Acute onset fever with altered mentation is a common problem encountered by the physician practicing in tropical countries. Central nervous system (CNS) infections are the most common cause resulting in fever with altered mentation in children. Aim: In this study, we have tried to analyze the cause of encephalopathy following short febrile illness in adults presenting to a tertiary care center in Northwestern part of India. Setting and Design: A prospective observational study carried out in a tertiary care center in the Northwestern India over a period of 1 year. Material and Methods A total of 127 patients with fever of less than 2 weeks duration along with alteration in mentation were studied prospectively over a period of 12 months. The demographic variables were recorded in detail. In addition to routine investigations, cerebrospinal fluid analysis, noncontrast- and contrast-enhanced computed tomography, along with magnetic resonance imaging were performed in all the subjects. Statistical Analysis The results were analyzed using SPSS statistical software. The values were expressed as mean with standard deviation for contiguous variable as percentage for the others. Results and Conclusion Out of these, 70% had primary CNS infection as the etiology. A total of 33% patients had meningitis, 29.9% had evidence of meningoencephalitis, and 12.7% were diagnosed as sepsis-associated encephalopathy. These were followed by cerebral malaria, leptospirosis, and brain abscess as the cause of febrile encephalopathy in adults. Among the noninfectious causes, acute disseminated encephalomyelitis, cortical venous thrombosis, and neuroleptic malignant syndrome were documented in 2.36% each. In 11% of the patients, the final diagnosis could not be made in spite of the extensive investigations. Our study demonstrates that acute febrile encephalopathy in adults is a heterogeneous syndrome with primary CNS infections being the commonest etiology. PMID:20930964

  12. Hypothermia for Hypoxic Ischemic Encephalopathy in Infants ≥ 36 weeks

    PubMed Central

    Higgins, Rosemary D.; Shankaran, Seetha

    2009-01-01

    Hypoxic ischemic encephalopathy is a serious condition affecting infants which can result in death and disability. This is a summary of pathogenesis of HIE, animal studies of cooling for hypoxic and ischemic models, human hypothermia trials, and the American Academy of Pediatrics publication on hypothermia for HIE. Hypothermia for neonatal HIE is continuing to evolve as a therapy. Studies, gaps in knowledge and opportunities for research are presented herein. PMID:19762176

  13. Non-typhoidal Salmonella encephalopathy involving lipopolysaccharide in cattle.

    PubMed

    Xiong, N; Brewer, M T; Anderson, K L; Carlson, S A

    2013-02-22

    This study assessed the involvement of lipopolysaccharide (LPS) in the non-typhoidal Salmonella encephalopathy (NTSE) caused by a unique isolate of Salmonella enterica serovar Saint-paul (SstpNPG). NTSE was prevented by genetic (deletion of murE) or pharmacologic (polymyxin) disruption of LPS on SstpNPG although the disruption of LPS did not deter brain penetration of the strain. This is the first study to demonstrate that LPS is involved in the manifestations of NTSE. PMID:22939987

  14. Perinatally infected adolescents living with human immunodeficiency virus (perinatally human immunodeficiency virus)

    PubMed Central

    Cruz, Maria Leticia S; Cardoso, Claudete A

    2015-01-01

    The availability of highly potent antiretroviral treatment during the last decades has transformed human immunodeficiency virus (HIV) infection into a chronic disease. Children that were diagnosed during the first months or years of life and received treatment, are living longer and better and are presently reaching adolescence and adulthood. Perinatally HIV-infected adolescents (PHIV) and young adults may present specific clinical, behavior and social characteristics and demands. We have performed a literature review about different aspects that have to be considered in the care and follow-up of PHIV. The search included papers in the MEDLINE database via PubMed, located using the keywords “perinatally HIV-infected” AND “adolescents”. Only articles published in English or Portuguese from 2003 to 2014 were selected. The types of articles included original research, systematic reviews, and quantitative or qualitative studies; case reports and case series were excluded. Results are presented in the following topics: “Puberal development and sexual maturation”, “Growth in weight and height”, “Bone metabolism during adolescence”, “Metabolic complications”, “Brain development, cognition and mental health”, “Reproductive health”, “Viral drug resistance” and “Transition to adult outpatient care”. We hope that this review will support the work of pediatricians, clinicians and infectious diseases specialists that are receiving these subjects to continue treatment. PMID:26279988

  15. [Perinatal Depression: The Meaning of the Paradigm Shift from "Postnatal" to "Perinatal"].

    PubMed

    Kamo, Toshiko

    2015-01-01

    Psychiatry regarding pregnancy, childbirth, and child-rearing is changing rapidly. In this paper, the meaning of the paradigm shift from postnantal to perinatal depression along with the changing treatment are discussed. Since the late 20 century, several large-scale epidemiological surveys on the incidence and outcomes of postnatal depression have concluded not only that postpartum depression is likely to occur at a high frequency, such as 10-15%, but that the subsequent maternal mortality rate as the number of deaths from suicide is higher than deaths due to obstetric medical conditions. Additionally, evidence of the negative impact of a mother's depression on the physical and mental development of children has been accumulated as well. Several studies regarding depression during pregnancy, such as on the relatively high frequency of prenatal depression or negative consequence of interrupted pharmacological treatment, should also be highlighted. These movements seemed to reflect the change in special attributes of depressive disorders and bipolar disorders, in that the term perinatal onset came to be preferred instead of postnatal, used in DSM-IV. Comprehensive treatment guidelines for depression applicable for all women with the potential for pregnancy, delivery, and lactation are needed as the next step.

  16. Persistent portosystemic shunts after deceased donor liver transplant causing episodic hepatic encephalopathy despite good graft function

    PubMed Central

    Barritt, A. Sidney; Fried, Michael W.; Hayashi, Paul H.

    2011-01-01

    We describe two cases of post liver transplant encephalopathy caused by persistent portosystemic shunts despite good graft function. Such recurrence of encephalopathy due to persistent shunting has not been reported in the deceased donor liver transplant literature. Our patients had episodic hepatic encephalopathy concordant with elevated serum ammonia levels due to well documented persistent portosystemic shunts. In one of our cases, the shunt was obliterated via coil embolization. This patient's encephalopathy resolved completely and has not recurred over seven months of follow up. The second patient has declined an intervention, but has remained symptom free on maintenance lactulose and rifaximin. PMID:19655248

  17. Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders.

    PubMed

    Latt, N; Dore, G

    2014-09-01

    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.

  18. Wernicke's encephalopathy in a patient with masticator and parapharyngeal space abscess: a case report

    PubMed Central

    2016-01-01

    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

  19. Acute encephalopathy of Bacillus cereus mimicking Reye syndrome.

    PubMed

    Ichikawa, Kazushi; Gakumazawa, Masayasu; Inaba, Aya; Shiga, Kentaro; Takeshita, Saoko; Mori, Masaaki; Kikuchi, Nobuyuki

    2010-09-01

    We present an 11-year-old boy diagnosed as having acute encephalopathy and liver failure with the underlying condition of a metabolic dysfunction. He developed convulsions and severe consciousness disturbance following gastroenteritis after the ingestion of some fried rice. He showed excessive elevation of transaminases, non-ketotic hypoglycemia and hyperammonemia, which were presumed to reflect a metabolic dysfunction of the mitochondrial beta-oxidation, and he exhibited severe brain edema throughout the 5th hospital day. He was subjected to mild hypothermia therapy for encephalopathy, and treated with high-dose methylprednisolone, cyclosporine and continuous hemodiafiltration for liver failure, systemic organ damage and hyperammonemia. The patient recovered with the sequela of just mild intelligence impairment. In this case, Bacillus cereus, producing emetic toxin cereulide, was detected in a gastric fluid specimen, a stool specimen and the fried rice. It was suggested that the cereulide had toxicity to mitochondria and induced a dysfunction of the beta-oxidation process. The patient was considered as having an acute encephalopathy mimicking Reye syndrome due to food poisoning caused by cereulide produced by B. cereus.

  20. A neurotoxic alcohol exposure paradigm does not induce hepatic encephalopathy.

    PubMed

    Hashimoto, Joel G; Wiren, Kristine M; Wilhelm, Clare J

    2016-01-01

    Alcohol abuse is associated with neurological dysfunction, brain morphological deficits and frank neurotoxicity. Although these disruptions may be a secondary effect due to hepatic encephalopathy, no clear evidence of causality is available. This study examined whether a 72h period of alcohol intoxication known to induce physical dependence, followed by a single withdrawal, was sufficient to induce signs of hepatic encephalopathy in male and female mice. Animals were continuously intoxicated via alcohol vapor inhalation, a procedure previously shown to induce significant neurotoxicity in female mice. At peak synchronized withdrawal (8h following the end of alcohol exposure), blood samples were taken and levels of several liver-regulated markers and brain swelling were characterized. Glutathione levels were also determined in the medial frontal cortex (mFC) and hippocampus. Results revealed elevated levels of cholesterol, albumin, alkaline phosphatase (ALP), alanine aminotransferase (ALT) and decreased levels of blood urea nitrogen and total bilirubin in alcohol-exposed male and female groups compared to controls. Brain water weight was not affected by alcohol exposure, though males tended to have slightly more water weight overall. Alcohol exposure led to reductions in tissue levels of glutathione in both the hippocampus and mFC which may indicate increased oxidative stress. Combined, these results suggest that hepatic encephalopathy does not appear to play a significant role in the neurotoxicity observed following alcohol exposure in this model. PMID:27268733

  1. The Treatment of Hepatic Encephalopathy in the Cirrhotic Patient

    PubMed Central

    Sanyal, Arun J.; Mullen, Kevin D.; Bass, Nathan M.

    2010-01-01

    Cirrhosis of the liver is a rising epidemic in the United States, affecting 2 out of every 1,000 adults. It is responsible for the deaths of more than 27,000 people each year. The primary diseases that underlie cirrhosis include viral hepatitis, alcoholic liver disease, and nonalcoholic fatty liver disease. Monitoring the extent of fibrosis and aggressively treating the underlying disease is essential for maintaining quality of life and preventing the complications of cirrhosis. As patients progress toward end-stage liver disease, the most common complications include portal hypertension, the development of esophageal varices, and hepatic encephalopathy. Esophageal varices can lead to hemorrhaging, a dangerous complication that is fatal in 30–50% of patients during the first occurrence. Hepatic encephalopathy is another serious complication of end-stage liver disease, as it significantly reduces patient quality of life and places heavy economic and caregiving burdens upon the patient's family. In this clinical roundtable monograph, the latest advances in the monitoring of liver disease and the management of portal hypertension and hepatic encephalopathy are discussed. PMID:20567582

  2. Uncommon cause of acute encephalopathy in liver cirrhosis.

    PubMed

    Dieuvil, Monique; Malaty, John

    2016-01-01

    A 49-year-old woman with a medical history of alcoholic cirrhosis status post-transjugular intrahepatic portosystemic shunt (post-TIPS) in 2012, and ongoing alcohol abuse, presented to the hospital, with haematuria. CT intravenous pyelogram (IVP) was normal except for 'a large intrahepatic cystic mass adjacent to the TIPS, causing intrahepatic biliary duct dilation'. The patient also presented with acute encephalopathy, jaundice, right upper quadrant abdominal pain and hyperbilirubinaemia (total bilirubin of 8.1 mg/dL with direct bilirubin of 3.0 mg/dL). She remained encephalopathic despite adequate treatment for alcohol withdrawal, hepatic encephalopathy and enterococcus urinary tract infection. MRI of the abdomen later confirmed presence of an obstructing biloma. The biloma, drained by CT-guided percutaneous drains, demonstrated an Escherichia coli and ESBL Klebsiella infection. The patient's encephalopathy completely resolved after treatment of the infected biloma. With adequate drainage, her hyperbilirubinaemia resolved to her post-TIPS baseline (total bilirubin of 3.7 mg/dL with direct bilirubin of 3.3 mg/dL). PMID:27194673

  3. Dexamethasone therapy for preventing delayed encephalopathy after carbon monoxide poisoning.

    PubMed

    Li, Q; Song, J J; Zhang, H Y; Fu, K; Lan, H B; Deng, Y

    2015-01-01

    We investigated dexamethasone therapy for preventing delayed encephalopathy after carbon monoxide (CO) poisoning. Eighty healthy male rats were exposed to CO and randomly divided into four groups: hyperbaric oxygen treatment (H), treatment (D), combined hyperbaric and dexamethasone treatment (C), and a control (M) group in which the rats inhaled CO to coma in the hyperbaric oxygen chamber, then were removed without further treatment. Twelve rats were put into the hyperbaric oxygen chamber and treated with air for 60 min (N) group. An eight arm maze was used to evaluate cognitive and memory abilities of these mice. Serum myelin basic protein (MBP) levels were evaluated using ELISA, and magnetic resonance imaging was used to observe brain demyelination and morbidity associated with delayed encephalopathy. A sample of the hippocampus from each group was examined by light microscopy. Cognitive and memory functions decreased in the control group M. Three days after CO poisoning, the serum MBP level of each group increased significantly. On Day 10 after CO poisoning, the MBP levels in groups C and D decreased significantly, but returned to normal on Day 18. MBP levels in the M and H groups were elevated at all time points. Brain MRIs showed significant differences among C, D, H and control M groups. Hematoxylin & eosin staining of the hippocampus showed greater damage in the control M and H groups. Early dexamethasone treatment may be useful for preventing delayed encephalopathy after CO poisoning and may reduce serum MBP levels.

  4. Perinatal origin of adult self-destructive behavior.

    PubMed

    Jacobson, B; Eklund, G; Hamberger, L; Linnarsson, D; Sedvall, G; Valverius, M

    1987-10-01

    The study was undertaken to test whether obstetric procedures are of importance for eventual adult behavior of the newborn, as ecological data from the United States seem to indicate. Birth record data were gathered for 412 forensic victims comprising suicides, alcoholics and drug addicts born in Stockholm after 1940, and who died there in 1978-1984. The births of the victims were unevenly distributed among six hospitals. Comparison with 2,901 controls, and mutual comparison of categories, showed that suicides involving asphyxiation were closely associated with asphyxia at birth, suicides by violent mechanical means were associated with mechanical birth trauma and drug addiction was associated with opiate and/or barbiturate administration to mothers during labor. Irrespective of the mechanism transferring the birth trauma to adulthood--which might be analogous to imprinting--the results show that obstetric procedures should be carefully evaluated and possibly modified to prevent eventual self-destructive behavior.

  5. Mapping Perinatal Nursing Process Measurement Concepts to Standard Terminologies.

    PubMed

    Ivory, Catherine H

    2016-07-01

    The use of standard terminologies is an essential component for using data to inform practice and conduct research; perinatal nursing data standardization is needed. This study explored whether 76 distinct process elements important for perinatal nursing were present in four American Nurses Association-recognized standard terminologies. The 76 process elements were taken from a valid paper-based perinatal nursing process measurement tool. Using terminology-supported browsers, the elements were manually mapped to the selected terminologies by the researcher. A five-member expert panel validated 100% of the mapping findings. The majority of the process elements (n = 63, 83%) were present in SNOMED-CT, 28% (n = 21) in LOINC, 34% (n = 26) in ICNP, and 15% (n = 11) in CCC. SNOMED-CT and LOINC are terminologies currently recommended for use to facilitate interoperability in the capture of assessment and problem data in certified electronic medical records. Study results suggest that SNOMED-CT and LOINC contain perinatal nursing process elements and are useful standard terminologies to support perinatal nursing practice in electronic health records. Terminology mapping is the first step toward incorporating traditional paper-based tools into electronic systems. PMID:27081756

  6. Mapping Perinatal Nursing Process Measurement Concepts to Standard Terminologies.

    PubMed

    Ivory, Catherine H

    2016-07-01

    The use of standard terminologies is an essential component for using data to inform practice and conduct research; perinatal nursing data standardization is needed. This study explored whether 76 distinct process elements important for perinatal nursing were present in four American Nurses Association-recognized standard terminologies. The 76 process elements were taken from a valid paper-based perinatal nursing process measurement tool. Using terminology-supported browsers, the elements were manually mapped to the selected terminologies by the researcher. A five-member expert panel validated 100% of the mapping findings. The majority of the process elements (n = 63, 83%) were present in SNOMED-CT, 28% (n = 21) in LOINC, 34% (n = 26) in ICNP, and 15% (n = 11) in CCC. SNOMED-CT and LOINC are terminologies currently recommended for use to facilitate interoperability in the capture of assessment and problem data in certified electronic medical records. Study results suggest that SNOMED-CT and LOINC contain perinatal nursing process elements and are useful standard terminologies to support perinatal nursing practice in electronic health records. Terminology mapping is the first step toward incorporating traditional paper-based tools into electronic systems.

  7. Perinatal 192 IgG-Saporin as Neuroteratogen.

    PubMed

    Petrosini, Laura; De Bartolo, Paola; Cutuli, Debora; Gelfo, Francesca

    2016-01-01

    The immunotoxin 192 IgG-saporin selectively destroys basal forebrain cholinergic neurons that provide cholinergic input to the hippocampus, entire cortical mantle, amygdala, and olfactory bulb. Perinatal immunotoxic lesions by 192 IgG-saporin induce long-lasting cholinergic depletion mimicking a number of developmental disorders reported in humans. The perinatal injection of 192 IgG-saporin induces several brain modifications, which are observed in neocortex and hippocampus at short and long term. These plastic changes involve both structural (alterations in brain volume, neuronal morphology, and neurogenesis) and molecular (modulations of the levels of neurotransmitters and other proteins related to neurodegeneration) levels. Moreover, the perinatal injection of 192 IgG-saporin may interact with the brain plastic capacity to react to other injuries. Perinatal 192 IgG-saporin lesions allowed investigating the role of the basal forebrain cholinergic system in modulating behavioral functions in developing as well as adult rats. After perinatal cholinergic depletion, rats display reduced ultrasonic vocalizations as neonates, learning and exploratory deficits as juveniles, altered discriminative abilities, impulsive and perseverative behaviors, and memory deficits as adults. Overall, these findings underline the importance of cholinergic system integrity for the development of specific structural and functional features. PMID:26695170

  8. Low perinatal autopsy rate in Malaysia: time for a change.

    PubMed

    Tan, Geok Chin; Hayati, Abdul Rahman; Khong, Teck Yee

    2010-01-01

    Our objectives were to determine the perinatal autopsy rate in a tertiary hospital in Malaysia and to quantify the value of the perinatal autopsy. All stillbirths, miscarriages, therapeutic abortions, and neonatal deaths between January 1, 2004, and August 31, 2009, were identified from the archives. The autopsy findings were compared with the clinical diagnoses. The autopsy reports were also reviewed to determine if it would be possible to improve the quality of the autopsies. There were 807 perinatal deaths, of which 36 (4.5%) included an autopsy. There were ethnic differences in the rate of autopsy, with the lowest rate among the Malays. The autopsy provided the diagnosis, changed the clinical diagnosis, or revealed additional findings in 58.3% of cases. Ancillary testing, such as microbiology, chromosomal analysis, and biochemistry, could improve the quality of the autopsy. This study provides further data on the perinatal autopsy rate from an emerging and developing country. It reaffirms the value of the perinatal autopsy. Attempts must be made to improve on the low autopsy rate while recognizing that the performance of autopsies can be enhanced through the use of ancillary testing.

  9. Endophenotypes in Schizophrenia for the Perinatal Period: Criteria for Validation

    PubMed Central

    Ross, Randal G.; Freedman, Robert

    2015-01-01

    Endophenotypes are disease-associated phenotypes that are thought to reflect the neurobiological or other mechanisms that underlie the more overt symptoms of a psychiatric illness. Endophenotypes have been critical in understanding the genetics, neurobiology, and treatment of schizophrenia. Because psychiatric illnesses have multiple causes, including both genetic and nongenetic risk factors, an endophenotype linked to one of the mechanisms may be expressed more frequently than the disease itself. However, in schizophrenia research, endophenotypes have almost exclusively been studied in older adolescents or adults who have entered or passed through the age of risk for the disorder. Yet, schizophrenia is a neurodevelopmental disorder where prenatal development starts a cascade of brain changes across the lifespan. Endophenotypes have only minimally been utilized to explore the perinatal development of vulnerability. One major impediment to the development of perinatally-useful endophenotypes has been the established validity criteria. For example, the criterion that the endophenotype be more frequently present in those with disease than those without is difficult to demonstrate when there can be a decades-long period between endophenotype measurement and the age of greatest risk for onset of the disorder. This article proposes changes to the endophenotype validity criteria appropriate to perinatal research and reviews how application of these modified criteria helped identify a perinatally-usable phenotype of risk for schizophrenia, P50 sensory gating, which was then used to propose a novel perinatal primary prevention intervention. PMID:25943124

  10. [Posterior reversible encephalopathy syndrome of the midbrain and hypothalamus - a case report of uremic encephalopathy presenting with hypersomnia].

    PubMed

    Shiga, Yuji; Kanaya, Yuhei; Kono, Ryuhei; Takeshima, Shinichi; Shimoe, Yutaka; Kuriyama, Masaru

    2016-01-01

    We report the case of a 73-year-old woman presenting with hypersomnia and loss of appetite. She suffered from diabetic nephropathy without receiving dialysis, in addition to hypertension, which was well controlled without marked fluctuation. There were no objective neurological findings. Her laboratory findings showed renal failure with 3.7 mg/dl of serum creatinine and decreased serum sodium and potassium. Brain magnetic resonance imaging (MRI) showed posterior reversible encephalopathy syndrome (PRES) with vasogenic edema, which was distributed in the dorsal midbrain, medial thalamus, and hypothalamus. After we addressed the electrolyte imbalance and dehydration, her symptoms and MRI findings gradually improved, but faint high signals on MRI were still present 3 months later. Orexin in the cerebrospinal fluid was decreased on admission, but improved 6 months later. We diagnosed uremic encephalopathy with atypical form PRES showing functional disturbance of the hypothalamus. PMID:26640128

  11. Experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle.

    PubMed

    Hamir, Amir N; Kehrli, Marcus E; Kunkle, Robert A; Greenlee, Justin J; Nicholson, Eric M; Richt, Jürgen A; Miller, Janice M; Cutlip, Randall C

    2011-05-01

    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.

  12. Complementary and alternative medicine therapies for perinatal depression.

    PubMed

    Deligiannidis, Kristina M; Freeman, Marlene P

    2014-01-01

    Complementary and alternative medicine therapies are increasingly sought out by people with psychiatric disorders. In this chapter, we review the evidence for several commonly used CAM therapies (i.e. omega-3 fatty acids, folate, S-adenosyl-methionine, 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 postpartum, but the safety and efficacy of these relative to standard treatments must still be systematically determined. Evidence-based use of complementary and alternative medicine therapies treatments for perinatal depression is discussed. Adequately powered systematic studies are necessary to determine the role of complementary and alternative medicine therapies in the treatment of perinatal depression.

  13. Perinatal Programming of Asthma: The Role of Gut Microbiota

    PubMed Central

    Azad, Meghan B.; Kozyrskyj, Anita L.

    2012-01-01

    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

  14. Interrelationship of carcinogen and glucocorticoid in perinatal enzyme induction.

    PubMed

    Leakey, J E; Wishart, G J; Dutton, G J

    1982-01-01

    Polycyclic aromatic hydrocarbons such as 3-methylcholanthrene can interrelate certain of their effects with those of glucocorticoid in the fetomaternal unit and the infant. We describe the induction by both classes of compounds in perinatal rat liver of enzymes that modify their biologic activity, and present further evidence for glucocorticoid-mimetic effects of 3-methylcholanthrene. We suggest that some degree of fetal teratogenicity and imprinting of postnatal events can arise from these mimetic effects, which should be taken into consideration along with the more direct perinatal toxicity of these mutagens.

  15. The experience of the implementation of perinatal audit in Moldova.

    PubMed

    Stratulat, P; Curteanu, A; Caraus, T; Petrov, V; Gardosi, J

    2014-09-01

    The Beyond the Numbers project in Moldova implemented perinatal mortality audit as a means to improve maternity and newborn care. Key activities for this project included training in audit, the setting up of audit committees, implementation of the review of cases and dissemination of information. During the project, a significant reduction was noted of perinatal deaths at term (from 37 weeks gestation and birthweight of ≥2500 g) by 1.5 per 1000; from 5.1 per 1000 in 2006 to 3.6 per 1000 in 2013.

  16. Early intervention after perinatal stroke: Opportunities and challenges

    PubMed Central

    Basu, Anna P

    2014-01-01

    Perinatal stroke is the commonest cause of hemiplegic cerebral palsy. No standardised early intervention exists despite evidence for a critical time window for activity-dependent plasticity to mould corticospinal tract development in the first few years of life. Intervention during this unique period of plasticity could mitigate the consequences of perinatal stroke to an extent not possible with later intervention, by preserving the normal pattern of development of descending motor pathways. This article outlines the broad range of approaches currently under investigation. Improved early detection and outcome prediction remain important goals, despite significant progress in this area. PMID:24528276

  17. Prevalence and associated harm of engagement in self-asphyxial behaviours (‘choking game’) in young people: a systematic review

    PubMed Central

    Busse, H; Harrop, T

    2015-01-01

    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

  18. Localized Cerebral Energy Failure in DNA Polymerase Gamma-Associated Encephalopathy Syndromes

    ERIC Educational Resources Information Center

    Tzoulis, Charalampos; Neckelmann, Gesche; Mork, Sverre J.; Engelsen, Bernt E.; Viscomi, Carlo; Moen, Gunnar; Ersland, Lars; Zeviani, Massimo; Bindoff, Laurence A.

    2010-01-01

    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…

  19. Stability properties of PrPSc from cattle with experimental transmissible spongiform encephalopathies

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  20. Experimental Inoculation of Spiroplasma mirum and Transmissible Mink Encephalopathy (TME) into Raccoons (Procyon lotor)

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  1. Ammonia and hepatic encephalopathy: the more things change, the more they remain the same.

    PubMed

    Shawcross, D L; Olde Damink, S W M; Butterworth, R F; Jalan, R

    2005-09-01

    Ammonia is thought to be central in the pathogenesis of hepatic encephalopathy and has been of importance to generations dating back to the early Egyptians. Hippocrates 2500 years ago described 'encephalopathy' simply translated as 'inside head suffering.' Over 1500 papers have been written on hepatic encephalopathy since 1966, but only a minority of these actually refer to the original observation of hepatic encephalopathy and the link with ammonia made by Marcel Nencki and Ivan Pavlov in 1893 with very little acknowledgement being made to the early landmark studies which described the importance of the muscle and kidneys in maintaining ammonia homeostasis as well as the liver and gut. Furthermore, infection was recognized as being an important modulator of brain function by the ancient Greek physicians and philosophers. This review focuses upon the original experiments of Nencki and Pavlov and describes how they fit into what we understand about the pathophysiology and treatment of hepatic encephalopathy today.

  2. A Qualitative Study of Physician Perspectives on Prognostication in Neonatal Hypoxic Ischemic Encephalopathy.

    PubMed

    Rasmussen, Lisa Anne; Bell, Emily; Racine, Eric

    2016-10-01

    Hypoxic ischemic encephalopathy is the most frequent cause of neonatal encephalopathy and yields a great degree of morbidity and mortality. From an ethical and clinical standpoint, neurological prognosis is fundamental in the care of neonates with hypoxic ischemic encephalopathy. This qualitative study explores physician perspectives about neurological prognosis in neonatal hypoxic ischemic encephalopathy. This study aimed, through semistructured interviews with neonatologists and pediatric neurologists, to understand the practice of prognostication. Qualitative thematic content analysis was used for data analysis. The authors report 2 main findings: (1) neurological prognosis remains fundamental to quality-of-life predictions and considerations of best interest, and (2) magnetic resonance imaging is presented to parents with a greater degree of certainty than actually exists. Further research is needed to explore both the parental perspective and, prospectively, the impact of different clinical approaches and styles to prognostication for neonatal hypoxic ischemic encephalopathy.

  3. Ammonia and hepatic encephalopathy: the more things change, the more they remain the same.

    PubMed

    Shawcross, D L; Olde Damink, S W M; Butterworth, R F; Jalan, R

    2005-09-01

    Ammonia is thought to be central in the pathogenesis of hepatic encephalopathy and has been of importance to generations dating back to the early Egyptians. Hippocrates 2500 years ago described 'encephalopathy' simply translated as 'inside head suffering.' Over 1500 papers have been written on hepatic encephalopathy since 1966, but only a minority of these actually refer to the original observation of hepatic encephalopathy and the link with ammonia made by Marcel Nencki and Ivan Pavlov in 1893 with very little acknowledgement being made to the early landmark studies which described the importance of the muscle and kidneys in maintaining ammonia homeostasis as well as the liver and gut. Furthermore, infection was recognized as being an important modulator of brain function by the ancient Greek physicians and philosophers. This review focuses upon the original experiments of Nencki and Pavlov and describes how they fit into what we understand about the pathophysiology and treatment of hepatic encephalopathy today. PMID:16167195

  4. Should We Treat Minimal/Covert Hepatic Encephalopathy, and with What?

    PubMed

    Henderson, Phillip K; Herrera, Jorge L

    2015-08-01

    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. PMID:26195203

  5. [Speech impairment predisposes to cognitive deterioration in hepatic encephalopathy].

    PubMed

    Meparidze, M M; Kodua, T E; Lashkhi, K S

    2010-04-01

    Hepatic encephalopathy is a reversible neuro-psychiatric syndrome that complicates liver insufficiency. The changes are complex and disorders are detected in digestive and neural systems. Disturbed consciousness and intellectual deterioration, particularly communicative difficulties are observed: speech is slurred, voice monotonous, writing disturbances, amimic face and rigid posture. Difficulties of socialization and tendency to self-isolation are observed. Memory, attention and perception are decreased. We suppose that disorders of cognitive functions are determined by impairment of speech and other communicative abilities. According to the theories of linguistic determinism and linguistic relativity thought categories move through the mould of native language. That means, speech impairment causes misperception of the real world. To confirm this hypothesis we investigated 106 patients with following diseases: peptic ulcer - 46, fatty liver - 30, liver cirrhosis - 19, viral hepatitis - 11 and 19 controls. Brain magnetic resonance tomography was carried out and psychometric tests were performed to patients with symptoms of hepatic encephalopathy. Atrophic changes in frontal, temporal and insular area of brain cortex were revealed in most cases. Those regions are responsible for actor observation, imitation and emotion, i.e. for empathy and sociability. They are very sensitive to the increased levels of ammonia and glutamine. In case of early treatment only slight atrophic changes are presented but without treatment atrophic processes become stable and expressed by impairments of speech and entire communicative ability. Human beings are very much at the mercy of the particular language which has become the medium of expression for their society. They do not live in the objective world alone, or in the world of social activity alone. Accordingly, damage of speech in hepatic encephalopathy is primary and predisposes to cognitive dysfunction. PMID:20495225

  6. The science and questions surrounding chronic traumatic encephalopathy.

    PubMed

    Ban, Vin Shen; Madden, Christopher J; Bailes, Julian E; Hunt Batjer, H; Lonser, Russell R

    2016-04-01

    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. PMID:27032918

  7. The Role of Sarcopenia and Frailty in Hepatic Encephalopathy Management.

    PubMed

    Lucero, Catherine; Verna, Elizabeth C

    2015-08-01

    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. PMID:26195205

  8. The National Football League and chronic traumatic encephalopathy: legal implications.

    PubMed

    Korngold, Caleb; Farrell, Helen M; Fozdar, Manish

    2013-01-01

    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.

  9. [Encephalopathy in the rat following partial portosystemic anastomosis].

    PubMed

    Stella, G D; Vassanelli, P; Gonzato, P; Rizzi, B; Pelizzo, M R

    1973-01-01

    Gamma-aminobutyric acid (GABA) values were determined in 4 groups of Sprague-Dawley rats 45-50 days after end-to-side porto-cava (PC), mesentericosplenic-cava (MSC) and mesenteric-cava (MC) anastomosis, and a false operation (FO) respectively. Values were significantly increased in the anastomosed animals. If, as may be supposed, this finding points to altered cerebral metabolism, its observation in the MSC and MC groups militates against the general view that encephalopathy in the experimental animal only occurs as a result of total deviation of the portal flow.

  10. The science and questions surrounding chronic traumatic encephalopathy.

    PubMed

    Ban, Vin Shen; Madden, Christopher J; Bailes, Julian E; Hunt Batjer, H; Lonser, Russell R

    2016-04-01

    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.

  11. Acute Liver Failure and Hepatic Encephalopathy After Cleft Palate Repair.

    PubMed

    Kocaaslan, Nihal Durmuş; Tuncer, Fatma Betul; Tutar, Engin; Celebiler, Ozhan

    2015-09-01

    Paracetamol is the most commonly used analgesic after cleft palate repair. It has rarely caused acute hepatic failure at therapeutic or supratherapeutic doses. Only one case of therapeutic paracetamol toxicity after cleft palate repair had been reported previously. Here, we present a similar patient who developed acute liver failure and hepatic encephalopathy after an uncomplicated cleft palate surgery. Lack of large prospective trials in young children due to ethical concerns increases the value of the case reports of acetaminophen toxicity at therapeutic doses. The dosing recommendations of paracetamol may need to be reconsidered after cleft palate surgery.

  12. Diagnosis and Management of Hepatic Encephalopathy in Fulminant Hepatic Failure.

    PubMed

    Kodali, Sudha; McGuire, Brendan M

    2015-08-01

    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.

  13. Current Concepts in the Pathophysiology and Management of Hepatic Encephalopathy

    PubMed Central

    2011-01-01

    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

  14. The hunter and the pianist: two hepatic encephalopathy tales.

    PubMed

    Montagnese, Sara; Cona, Giorgia; Schiff, Sami; Maresio, Giulio; Gatta, Angelo; Merkel, Carlo; Amodio, Piero

    2011-07-01

    Details of 2 patients with cirrhosis and hepatic encephalopathy whose hobby or job were possibly responsible for a selectively enhanced performance in 1 neuropsychiatric test are reported. Clinicians should be alert to the fact that personal inclinations and habits may impinge on both neuropsychological and psychophysic performance, thus producing a mismatch between the results of different mental status tests. A prospective study with accurate history taking, use of comprehensive assessment protocols, and modeling/critical interpretation of the test results is required to confirm this hypothesis.

  15. Vogt-Koyanagi-Harada syndrome presenting with encephalopathy

    PubMed Central

    Naeini, Alireza E.; Daneshmand, Dana; Khorvash, Farzin; Chitsaz, Ahmad

    2013-01-01

    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

  16. Contemporary Understanding and Management of Overt and Covert Hepatic Encephalopathy.

    PubMed

    NeSmith, Meghan; Ahn, Joseph; Flamm, Steven L

    2016-02-01

    Hepatic encephalopathy (HE) is a major complication of liver disease that leads to significant morbidity and mortality. Caring for hospitalized patients with HE is becoming more complex, and the economic burden of HE continues to rise. Defining and diagnosing HE, particularly covert HE (CHE), remain challenging. In this article, we review new tools and those currently under development for the diagnosis of CHE and the latest advances in the acute and long-term management of overt HE (OHE) and CHE. In particular, we review the latest data on the use of lactulose and rifaximin for treatment of OHE and summarize the data on adjunctive agents such as sodium benzoate and probiotics.

  17. Management of Hepatic Encephalopathy by Traditional Chinese Medicine

    PubMed Central

    Yao, Chun; Tang, Nong; Xie, Guoxiang; Zheng, Xiaojiao; Liu, Ping; Fu, Lei; Xie, Wu; Yao, Fan; Li, Houkai; Jia, Wei

    2012-01-01

    In spite of the impressive progress in the investigation of hepatic encephalopathy (HE), the complex mechanisms underlying the onset and deterioration of HE are still not fully understood. Currently, none of the existing theories provide conclusive explanations on the symptoms that link liver dysfunction to nervous system disorders and clinical manifestations. This paper summarized the diagnostic and therapeutic approaches used for HE in modern medicine and traditional Chinese medicine and provided future perspective in HE therapies from the viewpoint of holistic and personalized Chinese medicine. PMID:22567035

  18. Perinatal antidepressant use: understanding women's preferences and concerns.

    PubMed

    Battle, Cynthia L; Salisbury, Amy L; Schofield, Casey A; Ortiz-Hernandez, Samia

    2013-11-01

    Perinatal depression is prevalent and linked with a host of adverse consequences for women and newborns. Rates of engagement in depression treatment are, however, strikingly low among pregnant and postpartum women, with the majority of affected women receiving no mental health treatment. Research indicates that perinatal women are extremely reluctant to take antidepressant medications, yet the nature of women's concerns and treatment decision- making patterns have not been well documented. Developing a clearer understanding of women's treatment preferences and behaviors may help identify solutions to the under-treatment of perinatal depression. In this mixed methods study, we conducted in-depth interviews with 61 pregnant women, approximately half of whom were experiencing clinical levels of depression. In addition to assessing psychiatric diagnoses, symptoms, and functional impairment, we conducted qualitative interviews addressing women's preferences for depression treatment, concerns, and decision-making patterns. Consistent with prior reports, women were significantly more likely to voice a preference for non-pharmacologic depression treatments, as opposed to antidepressant medications. Many depressed women reported a great degree of uncertainty regarding how to treat their depression, and those with more severe depression symptoms were more likely to endorse decisional conflict. Analysis of qualitative comments yielded detailed information about the nature of women's concerns and preferences related to use of antidepressant medications and other aspects of treatment engagement. We discuss findings in the context of improving patient-centered care for perinatal depression.

  19. Perinatal Antidepressant Use: Understanding Women’s Preferences and Concerns

    PubMed Central

    BATTLE, CYNTHIA L.; SALISBURY, AMY L.; SCHOFIELD, CASEY A.; ORTIZ-HERNANDEZ, SAMIA

    2014-01-01

    Perinatal depression is prevalent and linked with a host of adverse consequences for women and newborns. Rates of engagement in depression treatment are, however, strikingly low among pregnant and postpartum women, with the majority of affected women receiving no mental health treatment. Research indicates that perinatal women are extremely reluctant to take antidepressant medications, yet the nature of women’s concerns and treatment decisionmaking patterns have not been well documented. Developing a clearer understanding of women’s treatment preferences and behaviors may help identify solutions to the under-treatment of perinatal depression. In this mixed methods study, we conducted in-depth interviews with 61 pregnant women, approximately half of whom were experiencing clinical levels of depression. In addition to assessing psychiatric diagnoses, symptoms, and functional impairment, we conducted qualitative interviews addressing women’s preferences for depression treatment, concerns, and decision-making patterns. Consistent with prior reports, women were significantly more likely to voice a preference for non-pharmacologic depression treatments, as opposed to antidepressant medications. Many depressed women reported a great degree of uncertainty regarding how to treat their depression, and those with more severe depression symptoms were more likely to endorse decisional conflict. Analysis of qualitative comments yielded detailed information about the nature of women’s concerns and preferences related to use of antidepressant medications and other aspects of treatment engagement. We discuss findings in the context of improving patient-centered care for perinatal depression. PMID:24241498

  20. Perinatal Mortality Counseling Program For Families Who Experience a Stillbirth.

    ERIC Educational Resources Information Center

    Kellner, Kenneth R.; And Others

    1981-01-01

    Discusses the emotional impact of a stillbirth on a family. The Perinatal Mortality Counseling Program (PMCP) at Shands Teaching Hospital, Gainesville, Florida, provides crisis intervention and support for these families, as well as serving a research function. Outlines the program, including its history, composition, procedures, and research.…

  1. Impaired Lung Mitochondrial Respiration Following Perinatal Nicotine Exposure in Rats.

    PubMed

    Cannon, Daniel T; Liu, Jie; Sakurai, Reiko; Rossiter, Harry B; Rehan, Virender K

    2016-04-01

    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.

  2. Visual Deficits and Improvements in Children after Perinatal Hypoxia.

    ERIC Educational Resources Information Center

    Groenendaal, F.; Van Hof-Van Duin, J.

    1992-01-01

    Study of the visual development of 38 infants, children, and youths who were neurologically impaired following perinatal hypoxia found that all children showed impairments of 1 or more visual functions, though visual development continued and visual improvements were demonstrated up to age 16. (Author/JDD)

  3. Perinatal mortality with particular reference to the Singapore situation.

    PubMed

    Tan, K L

    1984-04-01

    Perinatal mortality rates have been gradually declining in all countries. The initial decline mainly resulted from improvements in the late foetal mortality rates. Later with improvements in neonatal care, early neonatal mortality rates also improved. The developed countries have consistently shown better results than the developing countries, an indication of the higher standard of living, general health as well as the delivery of health care in these countries. In the Singapore situation, a rapid improvement in perinatal mortality was initially observed due to improvements in the late foetal mortality, followed later by reduction in the early neonatal mortality due to upgrading of neonatal intensive care. The perinatal mortality rate is lowest in the Chinese compared to the Indians and Malays, most likely due to the dietary practices of the three ethnic groups in Singapore; while the Chinese encourage extra nutrition in the pregnant female, the Malays and Indians tend to practise dietary restriction during this period. The improved nutrition of the pregnant mother is a factor in improving the perinatal mortality.

  4. Perinatal Mental Health: Supporting New Families through Vulnerability and Change.

    ERIC Educational Resources Information Center

    Fenichel, Emily, Ed.

    2002-01-01

    "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…

  5. 21 CFR 884.2740 - Perinatal monitoring system and accessories.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 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...

  6. 21 CFR 884.2740 - Perinatal monitoring system and accessories.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 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...

  7. 21 CFR 884.2740 - Perinatal monitoring system and accessories.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 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...

  8. Impaired Lung Mitochondrial Respiration Following Perinatal Nicotine Exposure in Rats.

    PubMed

    Cannon, Daniel T; Liu, Jie; Sakurai, Reiko; Rossiter, Harry B; Rehan, Virender K

    2016-04-01

    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. PMID:26899624

  9. Perinatal Staff Nurse Medical Device Use and Education.

    ERIC Educational Resources Information Center

    McConnell, Edwina A.

    1998-01-01

    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.…

  10. PREGNANCY AND PERINATAL HEALTH, BAMEN, INNER MONGOLIA, CHINA

    EPA Science Inventory

    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...

  11. Trends in Perinatal Care and Implications for Frontline Nurse Leaders.

    PubMed

    Crenshaw, Jeannette T; Adams, Ellise D; Amis, Debby

    2016-01-01

    The perinatal trends presented in this article are based on recent topics from conferences, journals, the media, as well as from input from perinatal nurses. Trends in patient care are influenced by evidence known for decades, new research, emerging and innovative concepts in healthcare, patient and family preferences, and the media. Trends discussed in this article are rethinking the due date, birth outside the hospital setting, obstetric hospitalists as birth attendants, nitrous oxide for pain in childbirth, hydrotherapy and waterbirth in the hospital setting, delayed cord clamping, disrupters of an optimal infant microbiome, skin-to-skin care during cesarean surgery, and breast-sleeping and the breast-feeding dyad. In addition, the authors developed implications for perinatal nurses related to each trend. The goal is to stimulate reflection on evidence that supports or does not support current practice and to stimulate future research by discussing some of the current trends that may influence the care that perinatal nurses provide during the birthing year. PMID:27465460

  12. Perinatal Pitocin as an Early ADHD Biomarker: Neurodevelopmental Risk?

    ERIC Educational Resources Information Center

    Kurth, Lisa; Haussmann, Robert

    2011-01-01

    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,…

  13. An Endangered Generation: Impact of Perinatal Drug Use.

    ERIC Educational Resources Information Center

    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…

  14. Assessing the knowledge of perinatal mental illness among student midwives.

    PubMed

    Phillips, Louise

    2015-11-01

    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.

  15. Prenatal and Perinatal Risk Factors for Autism in China

    ERIC Educational Resources Information Center

    Zhang, Xin; Lv, Cong-Chao; Tian, Jiang; Miao, Ru-Juan; Xi, Wei; Hertz-Picciotto, Irva; Qi, Lihong

    2010-01-01

    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…

  16. Prenatal and Perinatal Factors Associated with Intellectual Disability

    ERIC Educational Resources Information Center

    Bilder, Deborah A.; Pinborough-Zimmerman, Judith; Bakian, Amanda V.; Miller, Judith S.; Dorius, Josette T.; Nangle, Barry; McMahon, William M.

    2013-01-01

    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…

  17. Psychosocial impact of perinatal loss among Muslim women

    PubMed Central

    2012-01-01

    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

  18. [Perinatal mortality risk factors in a case-control study].

    PubMed

    Ruelas-Orozco, G; Guzmán, J; Malacara, J M

    1985-03-01

    This work describes a cross-sectional case-control study conducted in a marginal area of the city of Leon, Guanajuato, Mexico, to identify risk factors for perinatal mortality. 104 deaths identified in the civil register as occurring during 1982 in the study area were each matched to 2 controls selected from the same district and with birth dates within 30 days of the case. Perinatal mortality was defined as occurring between the 27th week of pregnancy and the 7th day after birth. 39 factors were stuided, including 10 socioeconomic factors, 6 maternal factors such as weight, height, and smoking, 10 factors concerning obstetrical history, 4 factors related to pathology during pregnancy, 6 factors referring to labor and delivery, and 2 concerning medical attention. In the univariate analysis, 18 factors were significant: unmarried or illiterate mother, maternal age under 17 or over 35, more than 7 previous births, previous perinatal death, less than 30 weeks or more than 200 weeks between pregnancies, hypertension, hemorrhage in the 2nd half of pregnancy, morning edema of pregnancy, no prenatal care, and birth attended by midwife. Some factors were eliminated because they were found to be dependent on a 2nd factor, and factors linked to perinatal events were also eliminated. A final model achieved after discriminant function analysis included 8 risk factors for perinatal mortality: 1) less than 30 weeks between pregnancies 2) more than 200 weeks between pregnancies 3) hypertension during pregnancy 4) maternal age under 18 5) maternal age over 35 6) unmarried mother 7) previous fetal deaths and 8) no prenatal care.

  19. Applying a science-based method to improve perinatal care: the institute for healthcare improvement perinatal improvement community.

    PubMed

    Bisognano, Maureen; Cherouny, Peter H; Gullo, Sue

    2014-10-01

    The Institute for Healthcare Improvement applies a systems-focused, science-based approach to improving perinatal care. This approach is based on the pioneering work in quality improvement and statistical process control performed by Walter Shewhart and W. Edwards Deming, and it uses the Model for Improvement, a simple and effective tool for accelerating improvement. In 2008, the Institute for Healthcare Improvement articulated a Triple Aim for improvement-better care, better health for populations, and lower per capita costs. The Triple Aim has become a guiding framework throughout health care and also guides much of the work of the Institute for Healthcare Improvement. The Institute for Healthcare Improvement's collaborative effort to improve perinatal care-the Perinatal Improvement Community-is an ideal example of work that pursues all three dimensions of the Triple Aim. The improvement method used in the community creates the foundation for the kind of cultural transformation that Perinatal Improvement Community leaders and participants have learned is necessary to make significant and lasting change. Using a systems-focused and science-based approach to improvement equips obstetricians and gynecologists with the knowledge, skills, and tools they need to improve the systems of care they work in so they can deliver the best evidence-based care to all of their patients, all of the time.

  20. [Elderly autopsy case of influenza-associated encephalopathy].

    PubMed

    Yoshimura, Hajime; Imai, Yukihiro; Beppu, Minako; Ohara, Nobuyuki; Kobayashi, Junya; Kuzuya, Akira; Yamagami, Hiroshi; Kawamoto, Michi; Kohara, Nobuo

    2008-10-01

    Influenza-associated encephalopathy (IAE) usually occurs in children aged <5 years. Adult cases of IAE are very rare and, thus far, no definite adult autopsy case has been reported. Here, we present the first definite adult autopsy case of IAE. A 76-year-old man presented with sudden coma a day after the onset of fever caused by infection with influenza type A virus. Soon after admission, his condition was complicated by DIC, shock, and multiple organ failure, and he was diagnosed with IAE. Oseltamivir administration and steroid pulse therapy were performed but these proved to be ineffective. The patient died about 24 hours after the onset of encephalopathy. The autopsy revealed massive brain edema and diffuse increase of amoeboid glias without inflammatory cell infiltration. Influenza type A/Hong Kong virus (H3) was isolated from his lungs. Serum IL-6 level was extremely high (35,800 pg/ml; normal, 0.221-4.62 pg/ml). The clinical course, and the laboratory and pathological findings of this adult case resembled those of a typical childhood-onset IAE, suggesting the same pathogenesis. During the influenza season, IAE should be taken into account for differential diagnosis in adult patients with altered mental status and fever.

  1. Abnormal cortical synaptic plasticity in minimal hepatic encephalopathy.

    PubMed

    Golaszewski, Stefan; Langthaler, Patrick B; Schwenker, Kerstin; Florea, Cristina; Christova, Monica; Brigo, Francesco; Trinka, Eugen; Nardone, Raffaele

    2016-07-01

    Minimal hepatic encephalopathy (MHE) represents the earliest stage of hepatic encephalopathy (HE). MHE is characterized by cognitive function impairment in the domains of attention, vigilance and integrative function, while obvious clinical manifestations are lacking. In the present study, we aimed at assessing whether subjects with MHE showed alterations in synaptic plasticity within the motor cortex. Previous findings suggest that learning in human motor cortex occurs through long-term potentiation (LTP)-like mechanisms. We employed therefore the paired associative stimulation (PAS) protocol by transcranial magnetic stimulation (TMS), which is able to induce LTP-like effects in the motor cortex of normal subjects. Fifteen patients with MHE and 15 age- and sex-matched cirrhotic patients without MHE were recruited. PAS consisted of 180 electrical stimuli of the right median nerve paired with a single TMS over the hotspot of right abductor pollicis brevis (APB) at an ISI of 25ms (PAS25). We measured motor evoked potentials (MEPs) before and after each intervention for up to 30min. In healthy subjects the PAS25 protocol was followed by a significant increase of the MEP amplitude. On the contrary, in patients with MHE the MEP amplitude was slightly reduced after PAS. These findings demonstrated that associative sensorimotor plasticity, an indirect probe for motor learning, is impaired in MHE patients. PMID:27475415

  2. Wernicke’s encephalopathy after sleeve gastrectomy: Literature review

    PubMed Central

    Pardo-Aranda, Fernando; Perez-Romero, Noelia; Osorio, Javier; Rodriguez-Santiago, Joaquín; Muñoz, Emilio; Puértolas, Noelia; Veloso, Enrique

    2016-01-01

    Objective To describe a case of Wernicke’s encephalopathy after laparoscopic sleeve gastrectomy. Setting Emergency Department and gastrointestinal surgery department. Case report A 20-year-old man class III obesity (BMI 50.17 kg/m2) underwent laparoscopic sleeve gastrectomy with uneventful recovery. Five weeks after surgery he was admitted in the Emergency Department because of persistent vomiting and dysphagia to solids. Esophagogastroduodenal transit and upper gastrointestinal endoscopy were requested but no relevant findings were shown. Laboratory analyses showed vitamin B1 12.2 ng/mL and 48 h following admission the patient experienced generalized weakness, sialorrhea and restrictions of actions such as reading a book. Neurological evaluation found confusion, motor ataxia, diplopy and nystagmus. A brain magnetic resonance was normal. According to low level of vitamin B1 and symptoms found in the patient a presumed diagnosis of Wernicke encephalopathy was made and parenteral thiamine 100 mg/day was started. The patient was discharged asymptomatic with oral intake of vitamin B1 600 mg per day. Conclusion Nutritional deficiencies after restrictive procedures are uncommon but easily preventable and can result in life threatening. With the upswing of bariatric surgery, surgeons and emergency physicians should be able to diagnose and treat those complications. Prophylactic thiamine should be administered to patients with predisposing factors. PMID:26826934

  3. Bovine spongiform encephalopathy surveillance in the Republic of Korea.

    PubMed

    Lee, Y H; Kim, M J; Tark, D S; Sohn, H J; Yun, E I; Cho, I S; Choi, Y P; Kim, C L; Lee, J H; Kweon, C H; Joo, Y S; Chung, G S; Lee, J H

    2012-12-01

    National surveillance for bovine spongiform encephalopathy (BSE) began in the Republic of Korea (ROK) in 1996. Surveillance programmes changed overtime to comply with the guidelines of the World Organisation for Animal Health (OIE). Bovine spongiform encephalopathy was designated as a notifiable disease in 1997. From July 2008, the BSE surveillance programme was intensified to test cattle in designated high-risk populations more effectively. New measures included the compulsory testing of all non-ambulatory cattle at abattoirs, and encouraging the testing of all dead cattle examined and recorded under the Mutual Aid Insurance Scheme (fallen stock). In addition, there was a vigorous search for animals suspected of being clinically infected. As a result, a total of 426,919 OIE points were achieved over a period of seven consecutive years to the end of October 2009. This enabled the submission of a successful application to the OIE in 2010 for recognition of the ROK's BSE disease status as being one of controlled risk, in accordance with Chapter 11.5. of the OIE Terrestrial Animal Health Code.

  4. Specific HLA genotypes confer susceptibility to acute necrotizing encephalopathy.

    PubMed

    Hoshino, A; Saitoh, M; Miyagawa, T; Kubota, M; Takanashi, J-I; Miyamoto, A; Tokunaga, K; Oka, A; Mizuguchi, M

    2016-09-01

    Acute necrotizing encephalopathy (ANE) is a rare and severe syndrome of acute encephalopathy triggered by viral infections. Cytokine storm is considered as the main pathogenetic mechanism of ANE. ANE is prevalent in East Asia, suggesting the association of host genetic factors. To elucidate the genetic background of Japanese ANE, we examined genotypes of human leukocyte antigen (HLA)-A, C, B, DRB1, DQB1 and DPB1 in 31 patients. Significant positive association was observed in both the allele frequency and positivity of DRB1*09:01 (P=0.043 and 0.025, respectively), as well as those of DQB1*03:03 (P=0.034 and 0.026, respectively). The carrier frequency of DRB1*09:01 and DQB1*03:03 alleles was higher in the patients (45.16%) than in controls (28.57%). These alleles are more common in East Asian than in European populations, and are reportedly associated with various autoimmune diseases in Japanese patients. Our data provide further evidence that altered immune response based on individual HLA genotypes may contribute to ANE pathogenesis. PMID:27467284

  5. Neuroimaging and spectroscopy in children with epileptic encephalopathies

    PubMed Central

    Parker, A.; Ferrie, C.; Keevil, S.; Newbold, M.; Cox, T.; Maisey, M.; Robinson, R.

    1998-01-01

    AIMS—To investigate the nature of the unifocal cortical abnormalities on FDG positron emission tomography (PET) in children with an epileptic encephalopathy but no focal abnormality on electroencephalogram or standard magnetic resonance imaging (MRI).
METHODS—Repeat FDG PET, surface rendered high resolution MRI, and single voxel magnetic resonance proton spectroscopy of the areas of abnormal metabolism compared to the contralateral side in 11children aged 2 to 12 years. Imaging was repeated after a median of 13months.
RESULTS—Visual analysis of repeat FDG PET revealed similar abnormalities in 10 of 11 children. Semiquantitative analysis revealed similar sited abnormalities in eight children. One child with ictal hypermetabolism initially had an inconsistent second scan. Magnetic resonance spectra in three children showed the N-acetylaspartate/choline ratio was lower in the hypometabolic focus than in the reciprocal area on the opposite side, in two children it was higher, and in one child it was equal. Surface rendered MRI was normal in seven of eight children, and showed temporal lobe asymmetry in one.
CONCLUSION—In children with established epileptic encephalopathies most hypometabolic areas on FDG PET are stable over time. While focal neuronal loss is likely in these areas in some children, microdysplasias or other focal cortical dysplasias are probable in others.

 PMID:9771250

  6. Cell therapy for neonatal hypoxic-ischemic encephalopathy.

    PubMed

    Pimentel-Coelho, Pedro M; Mendez-Otero, Rosalia

    2010-03-01

    Neonatal hypoxic-ischemic encephalopathy (HIE) is a common cause of long-term neurological disability in children. Despite advances in supportive care, no treatments for HIE are available at present. The potential use of stem/progenitor cell therapies for neuroprotection or regeneration of the damaged adult brain has been evaluated in several preclinical studies, and the most promising results are now being tested in clinical trials. In recent years, the use of stem/progenitor cell transplantation in animal models of HIE has also been evaluated in several laboratories. It was shown that human umbilical cord blood mononuclear cells and mesenchymal stem/progenitor cells may have a therapeutic potential through multiple mechanisms acting locally in the central nervous system and possibly in peripheral organs of hypoxic-ischemic animals. Neural stem/progenitor cells (NSCs) have also been transplanted in animal models of HIE, migrating long distances to ischemic brain areas and differentiating into neurons. The results of these studies have raised important questions that must be addressed before these findings can be translated to the bedside. In this review, we give a critical overview of the different studies published up to now, and we discuss the endogenous regenerative potential of NSCs of the newborn brain when challenged by an HIE insult. We also discuss the use of cell therapies for the encephalopathy of prematurity.

  7. Detecting Minimal Hepatic Encephalopathy in an Endemic Country for Hepatitis B: The Role of Psychometrics and Serum IL-6

    PubMed Central

    Tsai, Chia-Fen; Chu, Chi-Jen; Huang, Yi-Hsiang; Wang, Yen-Po; Liu, Pei-Yi; Lin, Han-Chieh; Lee, Fa-Yauh; Lu, Ching-Liang

    2015-01-01

    Background & Aims It remains unknown what the prevalence of minimal hepatic encephalopathy is in Taiwan, a highly endemic country for chronic viral hepatitis infection. It is also unclear whether abnormal serum cytokine levels can be indicative of the presence of minimal hepatic encephalopathy. We aimed to standardize the tests of psychometric hepatic encephalopathy score and predictive value of proinflammatory cytokines in minimal hepatic encephalopathy in Taiwan. Methods 180 healthy subjects and 94 cirrhotic patients without a history of overt hepatic encephalopathy from a tertiary center were invited to participate in this cross-sectional study. Blood sampling for determination of serum levels of interleukin 6 and 18 and tumor necrosis factor-α was performed. Based on the normogram of psychometric hepatic encephalopathy score from healthy volunteers, patients with minimal hepatic encephalopathy were identified from the cirrhotic patients using the criterion of a psychometric hepatic encephalopathy score less than −4. Results In the healthy subjects, age and education were predictors of subtests of psychometric hepatic encephalopathy score. Minimal hepatic encephalopathy was identified in 27 (29%) cirrhotic patients. Serum interleukin 6 level (OR = 6.50, 95% CI = 1.64–25.76, P = 0.008) was predictive of the presence of minimal hepatic encephalopathy after multivariate analysis. Conclusions The psychometric hepatic encephalopathy score can be a useful tool for detecting patients with minimal hepatic encephalopathy in Taiwan and around one third of cirrhotic outpatients fulfill this diagnosis. A high serum interleukin 6 level is predictive of the presence of minimal hepatic encephalopathy. PMID:26039496

  8. Analysis of policy towards improvement of perinatal mortality in the Netherlands (2004-2011).

    PubMed

    Vos, Amber A; van Voorst, Sabine F; Steegers, Eric A P; Denktaş, Semiha

    2016-05-01

    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.

  9. Clinical subtypes of chronic traumatic encephalopathy: literature review and proposed research diagnostic criteria for traumatic encephalopathy syndrome

    PubMed Central

    2014-01-01

    The long-term consequences of repetitive head impacts have been described since the early 20th century. Terms such as punch drunk and dementia pugilistica were first used to describe the clinical syndromes experienced by boxers. A more generic designation, chronic traumatic encephalopathy (CTE), has been employed since the mid-1900s and has been used in recent years to describe a neurodegenerative disease found not just in boxers but in American football players, other contact sport athletes, military veterans, and others with histories of repetitive brain trauma, including concussions and subconcussive trauma. This article reviews the literature of the clinical manifestations of CTE from 202 published cases. The clinical features include impairments in mood (for example, depression and hopelessness), behavior (for example, explosivity and violence), cognition (for example, impaired memory, executive functioning, attention, and dementia), and, less commonly, motor functioning (for example, parkinsonism, ataxia, and dysarthria). We present proposed research criteria for traumatic encephalopathy syndrome (TES) which consist of four variants or subtypes (TES behavioral/mood variant, TES cognitive variant, TES mixed variant, and TES dementia) as well as classifications of ‘probable CTE’ and ‘possible CTE’. These proposed criteria are expected to be modified and updated as new research findings become available. They are not meant to be used for a clinical diagnosis. Rather, they should be viewed as research criteria that can be employed in studies of the underlying causes, risk factors, differential diagnosis, prevention, and treatment of CTE and related disorders. PMID:25580160

  10. Use of murine bioassay to resolve ovine transmissible spongiform encephalopathy cases showing a bovine spongiform encephalopathy molecular profile.

    PubMed

    Beck, Katy E; Sallis, Rosemary E; Lockey, Richard; Vickery, Christopher M; Béringue, Vincent; Laude, Hubert; Holder, Thomas M; Thorne, Leigh; Terry, Linda A; Tout, Anna C; Jayasena, Dhanushka; Griffiths, Peter C; Cawthraw, Saira; Ellis, Richard; Balkema-Buschmann, Anne; Groschup, Martin H; Simmons, Marion M; Spiropoulos, John

    2012-05-01

    Two cases of unusual transmissible spongiform encephalopathy (TSE) were diagnosed on the same farm in ARQ/ARQ PrP sheep showing attributes of both bovine spongiform encephalopathy (BSE) and scrapie. These cases, UK-1 and UK-2, were investigated further by transmissions to wild-type and ovine transgenic mice. Lesion profiles (LP) on primary isolation and subpassage, incubation period (IP) of disease, PrP(Sc) immunohistochemical (IHC) deposition pattern and Western blot profiles were used to characterize the prions causing disease in these sheep. Results showed that both cases were compatible with scrapie. The presence of BSE was contraindicated by the following: LP on primary isolation in RIII and/or MR (modified RIII) mice; IP and LP after serial passage in wild-type mice; PrP(Sc) deposition pattern in wild-type mice; and IP and Western blot data in transgenic mice. Furthermore, immunohistochemistry (IHC) revealed that each case generated two distinct PrP(Sc) deposition patterns in both wild-type and transgenic mice, suggesting that two scrapie strains coexisted in the ovine hosts. Critically, these data confirmed the original differential IHC categorization that these UK-1 and UK-2 cases were not compatible with BSE.

  11. Prominent Bilateral Hand Tremor in Hashimoto’s Encephalopathy: A Video Demonstration

    PubMed Central

    Ramcharan, Kanterpersad; Hosein, Nadeem; Teelucksingh, Joel David; Rampersad, Fidel; Teelucksingh, Surujpal

    2016-01-01

    Background Hashimoto’s encephalopathy often presents with neuropsychiatric manifestations including seizures and movement disorders. Case Report We describe a patient who presented with bilateral hand tremor and mild cognitive defects that fulfilled the criteria for a diagnosis of Hashimoto’s encephalopathy. There was a rapid response to glucocorticoid therapy with relapse following treatment withdrawal. Discussion Recently published clinical criteria for the diagnosis of Hashimoto’s encephalopathy include seizures, myoclonus, hallucinations, or stroke‐like episodes but do not include tremor. Our case had mild cognitive dysfunction and a coarse tremor as the predominant clinical features, which probably represent mild disease. PMID:27790384

  12. Wernicke's Encephalopathy in a Patient with Nasopharyngeal Carcinoma: Magnetic Resonance Imaging Findings.

    PubMed

    Law, Huong Ling; Tan, Suzet; Sedi, Rosleena

    2011-07-01

    We report a case of Wernicke's encephalopathy in a patient with nasopharyngeal carcinoma with a 3-month history of poor oral intake related to nausea and vomiting due to chemotherapy. The patient later developed deep coma while receiving in-patient therapy. Magnetic resonance imaging of the brain revealed typical findings of Wernicke's encephalopathy. The patient was treated with thiamine injections, which resulted in subsequent partial recovery of neurological function. This paper stresses the importance of magnetic resonance imaging for prompt diagnosis of Wernicke's encephalopathy.

  13. A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke’s Encephalopathy

    PubMed Central

    Solmaz, Soner; Gereklioğlu, Çiğdem; Tan, Meliha; Demir, Şenay; Yeral, Mahmut; Korur, Aslı; Boğa, Can; Özdoğu, Hakan

    2015-01-01

    Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke’s encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke’s encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke’s encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature. PMID:25912759

  14. Public health issues related to animal and human spongiform encephalopathies: memorandum from a WHO meeting.

    PubMed Central

    1992-01-01

    The transmissible spongiform encephalopathies (TSE) include bovine spongiform encephalopathy (BSE), which was first described in 1986 in the United Kingdom but has occurred subsequently in several other countries. This Memorandum reviews the existing state of knowledge on all the known spongiform encephalopathies, and evaluates the pathways of transmission and associated hazards. The possible implications of the animal diseases, especially BSE, with regard to the use of animal tissues as animal feed, human food, and in the preparation of medicinal and other products for human use are discussed, with recommendations to national health authorities on appropriate measures to minimize the consequences of BSE to public and animal health. PMID:1600580

  15. The "Plateau" Phenomenon of Stepwise Extracorporeal Albumin Dialysis Bispectral Index Hepatic Encephalopathy Recovery.

    PubMed

    Dahaba, Ashraf A

    2015-12-15

    The Bispectral Index™ monitor, an electroencephalographic-derived parameter, can quantify hepatic encephalopathy cerebral function recovery and differentiate between West Haven grades 1 to 4. I report a very peculiar "plateau" phenomenon of 3 clear distinct plateaus of stepwise albumin dialysis Bispectral Index hepatic encephalopathy recovery during an 8-hour Molecular Adsorbent Recirculating System™ (MARS™) liver-assist extracorporeal detoxification, manifesting in conjunction with 3 West Haven grade recoveries. In the patients, I observed recovery of cerebral function after hepatic encephalopathy as a series of plateaus with abrupt transitions between the plateaus, rather than the gradual recovery I anticipated. PMID:26657702

  16. Wernicke's encephalopathy and anabolic steroid drug abuse. Is there any possible relation?

    PubMed

    Christopoulos, P; Katsanoulas, C; Timplalexi, G; Lathyris, D; Vasiliagkou, S; Antoniadou, E

    2012-10-01

    Wernicke's encephalopathy is a reversible, neurologic disorder due to thiamine deficiency which is mainly related to chronic alcohol abuse. We report a case of a young male patient, who was bodybuilder and anabolic drug user, in whom encephalopathy was diagnosed after a short medical course in the ICU after a major upper gastrointestinal bleeding (Mallory-Weiss syndrome) and hypovolemic shock. His clinical condition was typical for Wernicke's encephalopathy and although neuroimaging tests were not indicative, the patient received thiamine supplement therapy, which resulted in rapid clinical improvement. The diagnosis was based only on clinical sings and anabolic drug abuse was considered as a possible predisposing factor for the manifestation of the syndrome.

  17. Neurocognitive Outcome of Children Exposed to Perinatal Mother-to-Child Chikungunya Virus Infection: The CHIMERE Cohort Study on Reunion Island

    PubMed Central

    Ramful, Duksha; Boumahni, Brahim; Bintner, Marc; Alessandri, Jean-Luc; Carbonnier, Magali; Tiran-Rajaoefera, Isabelle; Beullier, Gilles; Boya, Irénée; Noormahomed, Tahir; Okoï, Jocelyn; Rollot, Olivier; Cotte, Liliane; Jaffar-Bandjee, Marie-Christine; Michault, Alain; Favier, François; Kaminski, Monique; Fourmaintraux, Alain; Fritel, Xavier

    2014-01-01

    Background Little is known about the neurocognitive outcome in children exposed to perinatal mother-to-child Chikungunya virus (p-CHIKV) infection. Methods The CHIMERE ambispective cohort study compared the neurocognitive function of 33 p-CHIKV-infected children (all but one enrolled retrospectively) at around two years of age with 135 uninfected peers (all enrolled prospectively). Psychomotor development was assessed using the revised Brunet-Lezine scale, examiners blinded to infectious status. Development quotients (DQ) with subscores covering movement/posture, coordination, language, sociability skills were calculated. Predictors of global neurodevelopmental delay (GND, DQ≤85), were investigated using multivariate Poisson regression modeling. Neuroradiologic follow-up using magnetic resonance imaging (MRI) scans was proposed for most of the children with severe forms. Results The mean DQ score was 86.3 (95%CI: 81.0–91.5) in infected children compared to 100.2 (95%CI: 98.0–102.5) in uninfected peers (P<0.001). Fifty-one percent (n = 17) of infected children had a GND compared to 15% (n = 21) of uninfected children (P<0.001). Specific neurocognitive delays in p-CHIKV-infected children were as follows: coordination and language (57%), sociability (36%), movement/posture (27%). After adjustment for maternal social situation, small for gestational age, and head circumference, p-CHIKV infection was found associated with GND (incidence rate ratio: 2.79, 95%CI: 1.45–5.34). Further adjustments on gestational age or breastfeeding did not change the independent effect of CHIKV infection on neurocognitive outcome. The mean DQ of p-CHIKV-infected children was lower in severe encephalopathic children than in non-severe children (77.6 versus 91.2, P<0.001). Of the 12 cases of CHIKV neonatal encephalopathy, five developed a microcephaly (head circumference <−2 standard deviations) and four matched the definition of cerebral palsy. MRI scans showed severe

  18. Molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies.

    PubMed

    Priola, Suzette A; Vorberg, Ina

    2004-01-01

    The transmissible spongiform encephalopathy (TSE) diseases are a group of rare, fatal, and transmissible neurodegenerative diseases that include kuru and Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep, transmissible mink encephalopathy (TME), and chronic wasting disease (CWD) in mule deer and elk. Over the last 20 yr, they have gone from a fascinating but relatively obscure group of diseases to one that is a major agricultural and economic problem as well as a threat to human health. The shift in the relative impact of the TSE diseases began in the late 1970s when the United Kingdom altered the process by which animal carcasses were rendered to provide a protein supplement (i.e., meat and bone meal) to sheep, cattle, and other livestock. Several years later a new disease was recognized in the British cattle population. The pathological and immunohistochemical characteristics of the disease clearly placed it among the TSEs. The new disease was named bovine spongiform encephalopathy (BSE) by the scientific community and "mad cow disease" by the less-than-scientific press. At its peak in the UK, several thousand cattle a year were diagnosed with BSE, and millions of cattle were slaughtered. Introduction of the specified offals ban as well as banning the practice of feeding ruminants to other ruminants has led to a drastic decrease in the number of yearly BSE cases in the UK (less than 500 in 2003), and the epidemic is clearly on the wane. However, BSE has now spread throughout the rest of Europe, as well as to Japan, Russia, Canada, and Israel and thus remains a worldwide problem.A primary concern following the identification of BSE in 1985 was that it might cross species barriers to infect humans. Initially, it was thought that transmission of BSE to humans was unlikely, given that humans appeared to be resistant to scrapie, an animal TSE that had been endemic in British sheep for centuries. However, a few years after BSE was first recognized, a

  19. Adult survivors of childhood sexual abuse: suggestions for perinatal caregivers.

    PubMed

    Roussillon, J A

    1998-11-01

    As many as 1 in 4 women are survivors of childhood sexual abuse. This traumatic life event profoundly influences the care that advanced practice nurses provide throughout the life cycle, and particularly the care that is provided during times of physical and emotional stress. Despite the prevalence of sexual abuse, there has been little research on the experiences of survivors during the perinatal period, and few suggestions for interventions. This article reviews the literature on the implications of sexual abuse on a woman's experience of pregnancy, birth, and breastfeeding. It emphasizes the importance of routine screening for abuse, as well as assessment of a survivor's stage in the recovery process. Finally, this article suggests topics for appropriate perinatal anticipatory guidance for women who have a history of sexual abuse.

  20. Suicide during Perinatal Period: Epidemiology, Risk Factors, and Clinical Correlates

    PubMed Central

    Orsolini, Laura; Valchera, Alessandro; Vecchiotti, Roberta; Tomasetti, Carmine; Iasevoli, Felice; Fornaro, Michele; De Berardis, Domenico; Perna, Giampaolo; Pompili, Maurizio; Bellantuono, Cesario

    2016-01-01

    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

  1. Suicide during Perinatal Period: Epidemiology, Risk Factors, and Clinical Correlates.

    PubMed

    Orsolini, Laura; Valchera, Alessandro; Vecchiotti, Roberta; Tomasetti, Carmine; Iasevoli, Felice; Fornaro, Michele; De Berardis, Domenico; Perna, Giampaolo; Pompili, Maurizio; Bellantuono, Cesario

    2016-01-01

    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

  2. Perinatal Depression and Patterns of Attachment: A Critical Risk Factor?

    PubMed

    Meuti, Valentina; Aceti, Franca; Giacchetti, Nicoletta; Carluccio, Giuseppe Mattia; Zaccagni, Michela; Marini, Isabella; Giancola, Orazio; Ciolli, Paola; Biondi, Massimo

    2015-01-01

    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.

  3. High perinatal and neonatal mortality in rural India.

    PubMed

    Bhardwaj, N; Hasan, S B

    1993-04-01

    A prospective study conducted in rural India on pregnant women showed poor utilization of primary health services and very poor maternal care receptivity especially in terms of antenatal care. A very high perinatal mortality rate of 81.3/1000 live births and a neonatal mortality rate of 63.7/1000 live births was observed in the present study. Out of 204 live births, 72.05% of newborn developed complications within 6 weeks of the delivery. Most of the complications were of a minor nature and could be attributed to poor environmental conditions, lack of personal hygiene and ignorance. The study highlights the need for training of grass root level workers for the improvement of perinatal and neonatal care in rural India. PMID:8478893

  4. A community based surveillance system for perinatal and neonatal care.

    PubMed

    Dyal Chand, A; Khale, M

    1989-11-01

    The impact of maternal health services on perinatal and neonatal mortality depends on both the quantitative and qualitative coverage of pregnant women with obstetric services. In rural areas this becomes all the more difficult because of the requirement of a large decentralized infrastructure extending from village based health workers and subcentres to the Primary Health Centre and tertiary levels of referral. An effective introduction of socio-cultural, biomedical and managerial interventions is required to reduce perinatal and neonatal mortality. A community based surveillance and monitoring system is central to and facilitates the introduction of all other interventions. Finally, the system operated by grass-root level workers is a motivational tool for achieving expected levels of performance. PMID:2630471

  5. Building Perinatal Case Manager Capacity Using Quality Improvement

    PubMed Central

    Fitzgerald, Elaine

    2015-01-01

    ABSTRACT 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. PMID:26937160

  6. Perinatal exposure to music protects spatial memory against callosal lesions.

    PubMed

    Amagdei, Anca; Balteş, Felicia Rodica; Avram, Julia; Miu, Andrei C

    2010-02-01

    Several studies have indicated that the exposure of rodents to music modulates brain development and neuroplasticity, by mechanisms that involve facilitated hippocampal neurogenesis, neurotrophin synthesis and glutamatergic signaling. This study focused on the potential protection that the perinatal exposure to music, between postnatal days 2 and 32, could offer against functional deficits induced by neonatal callosotomy in rats. The spontaneous alternation and marble-burying behaviors were longitudinally measured in callosotomized and control rats that had been exposed to music or not. The results indicated that the neonatal callosotomy-induced spontaneous alternation deficits that became apparent only after postnatal day 45, about the time when the rat corpus callosum reaches its maximal levels of myelination. The perinatal exposure to music efficiently protected the spontaneous alternation performance against the deficits induced by callosotomy. The present findings may offer important insights into music-induced neuroplasticity, relevant to brain development and neurorehabilitation.

  7. Clustering of the morphological components of perinatal telencephalic leucoencephalopathy

    PubMed Central

    Leviton, Alan; Gilles, Floyd H.

    1971-01-01

    The data upon which the original report of perinatal telencephalic leucoencephalopathy was based have been examined for clustering of the four criteria of neonatal white matter damage. Three clusters have been identified: (1) acutely damaged glia with amphophilic globules, (2) hypertrophic astrocytes and amphophilic globules but without necrotic foci, and (3) hypertrophic astrocytes and amphophilic globules with necrotic foci. The original report of perinatal telencephalic leucoencephalopathy therefore probably included two entities. One was characterized by acutely damaged glia and amphophilic globules and the other by hypertrophic astrocytes and amphophilic globules. The two entities do not occur together significantly more frequently than would be expected on the basis of the independent occurrence of each. PMID:4330699

  8. Perinatal and Early Childhood Environmental Factors Influencing Allergic Asthma Immunopathogenesis

    PubMed Central

    Gaffin, Jonathan M.; Kanchongkittiphon, Watcharoot; Phipatanakul, Wanda

    2014-01-01

    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

  9. Doing, being, and becoming: a family's journey through perinatal loss.

    PubMed

    Forhan, Mary

    2010-01-01

    Occupational therapists are encouraged to reflect on doing, being, and becoming not only as it relates to the development of their profession but also in their own lives (Wilcock 1999). This article is a description of that process for me and my family in our journey through perinatal loss. This autoethnography uses a personally situated account of perinatal death. This article is a form of self-narrative that places me and my family in social context through the lens of an occupational therapist. This article aims to convey the meanings attached to the experience of grief and loss in the context of participation in everyday occupations. By sharing a perspective on the lived experience and connecting it to the literature on grief and occupation, readers will be able to decide if the connection holds as valid from a theoretical and clinical perspective. PMID:20131574

  10. Clinical characteristics of perinatal psychiatric patients: a chart review study.

    PubMed

    Battle, Cynthia L; Zlotnick, Caron; Miller, Ivan W; Pearlstein, Teri; Howard, Margaret

    2006-05-01

    Although postpartum depression and other perinatal disorders have been the subject of increased research attention, important questions remain regarding women who actively seek psychiatric treatment during pregnancy and the postpartum period. In this study, we examined clinical records of 500 perinatal psychiatric patients who received treatment in a psychiatric day hospital (N = 398) or outpatient behavioral health clinic (N = 102). Patients' presenting diagnoses, psychiatric history, treatment course, and depressive symptoms were recorded. The majority of women had major depression as their primary diagnosis, with an average Edinburgh Postnatal Depression Scale score of over 20. Many depressed patients were diagnosed with comorbid anxiety and substance abuse disorders. Although most women were willing to take psychotropic medications, a sizable minority were not, particularly those who were breast-feeding. For more than a third of the sample, the treatment sought while pregnant or postpartum represented their first contact with the mental health system. Treatment implications are discussed. PMID:16699387

  11. The association of in vitro fertilization and perinatal morbidity.

    PubMed

    Kalra, Suleena Kansal; Molinaro, Thomas A

    2008-09-01

    In recent years, there has been increasing concern regarding the safety of in vitro fertilization (IVF) because of the potential health impact on these infants. Multiple pregnancy contributes the vast majority of morbidity associated with IVF and, initially, many thought that adverse outcomes after IVF were solely attributable to the high incidence of twin pregnancies. More recently, multiple studies have suggested that IVF singleton pregnancies may be at increased risk for preterm birth, low birth weight, congenital anomalies, perinatal mortality, and several other pregnancy-related complications compared with unassisted singleton pregnancies. We have focused this review on the increased risk of adverse outcomes in IVF singleton conceptions compared with that of unassisted conceptions. The available evidence evaluating the association between IVF and low birth weight, preterm delivery, placental abruption, preeclampsia, congenital anomalies, and perinatal mortality in singleton pregnancies is summarized. In addition, data reporting an increased risk of congenital and chromosomal anomalies after IVF are presented.

  12. Doing, being, and becoming: a family's journey through perinatal loss.

    PubMed

    Forhan, Mary

    2010-01-01

    Occupational therapists are encouraged to reflect on doing, being, and becoming not only as it relates to the development of their profession but also in their own lives (Wilcock 1999). This article is a description of that process for me and my family in our journey through perinatal loss. This autoethnography uses a personally situated account of perinatal death. This article is a form of self-narrative that places me and my family in social context through the lens of an occupational therapist. This article aims to convey the meanings attached to the experience of grief and loss in the context of participation in everyday occupations. By sharing a perspective on the lived experience and connecting it to the literature on grief and occupation, readers will be able to decide if the connection holds as valid from a theoretical and clinical perspective.

  13. Labour complications remain the most important risk factors for perinatal mortality in rural Kenya.

    PubMed Central

    Weiner, Renay; Ronsmans, Carine; Dorman, Ed; Jilo, Hilton; Muhoro, Anne; Shulman, Caroline

    2003-01-01

    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

  14. [Pathophysiology and therapy of perinatal asphyxia in a mature newborn].

    PubMed

    Storm, W

    1979-03-01

    Based on modern knowledge about the pathophysiology of perinatal asphyxia a new concept of therapy is discussed. Besides cardiopulmonary resuscitation for the initial shock reaction the therapy of secondary complications according to the multi-system-reaction is emphasized. After the description of the correlation of partial asphyxia and the development of neurological defect syndromes in animal models the necessity of a "cerebral resuscitation" is mentioned.

  15. Childbirth Education for Parents Experiencing Pregnancy after Perinatal Loss

    PubMed Central

    Wright, Patricia Moyle

    2005-01-01

    Expectant parents who have experienced previous perinatal loss have special concerns, which can be partially addressed by modifying prepared childbirth education courses. This article presents a review of current literature, highlighting the unique needs of expectant parents who have experienced previous pregnancy loss. Modifications to traditional childbirth education courses are suggested, which include addressing parents' grief, managing anxiety, and facilitating communication with health-care providers and others. PMID:17273448

  16. Perinatal asphyxia: CNS development and deficits with delayed onset

    PubMed Central

    Herrera-Marschitz, Mario; Neira-Pena, Tanya; Rojas-Mancilla, Edgardo; Espina-Marchant, Pablo; Esmar, Daniela; Perez, Ronald; Muñoz, Valentina; Gutierrez-Hernandez, Manuel; Rivera, Benjamin; Simola, Nicola; Bustamante, Diego; Morales, Paola; Gebicke-Haerter, Peter J.

    2013-01-01

    Perinatal asphyxia constitutes a prototype of obstetric complications occurring when pulmonary oxygenation is delayed or interrupted. The primary insult relates to the duration of the period lacking oxygenation, leading to death if not re-established. Re-oxygenation leads to a secondary insult, related to a cascade of biochemical events required for restoring proper function. Perinatal asphyxia interferes with neonatal development, resulting in long-term deficits associated to mental and neurological diseases with delayed clinical onset, by mechanisms not yet clarified. In the experimental scenario, the effects observed long after perinatal asphyxia have been explained by overexpression of sentinel proteins, such as poly(ADP-ribose) polymerase-1 (PARP-1), competing for NAD+ during re-oxygenation, leading to the idea that sentinel protein inhibition constitutes a suitable therapeutic strategy. Asphyxia induces transcriptional activation of pro-inflammatory factors, in tandem with PARP-1 overactivation, and pharmacologically induced PARP-1 inhibition also down-regulates the expression of proinflammatory cytokines. Nicotinamide has been proposed as a suitable PARP-1 inhibitor. Its effect has been studied in an experimental model of global hypoxia in rats. In that model, the insult is induced by immersing rat fetus into a water bath for various periods of time. Following asphyxia, the pups are delivered, treated, and nursed by surrogate dams, pending further experiments. Nicotinamide rapidly distributes into the brain following systemic administration, reaching steady state concentrations sufficient to inhibit PARP-1 activity for several hours, preventing several of the long-term consequences of perinatal asphyxia, supporting the idea that nicotinamide constitutes a lead for exploring compounds with similar or better pharmacological profiles. PMID:24723845

  17. Useful tests for hepatic encephalopathy in clinical practice.

    PubMed

    Nabi, Eiman; Bajaj, Jasmohan S

    2014-01-01

    Hepatic encephalopathy (HE) is a serious complication of liver disease and portosystemic shunting that represents a continuum of neuropsychiatric changes and altered consciousness. It is classified as overt HE (OHE) when clinically apparent or as covert HE (CHE) in its mildest form. Progression of CHE to OHE and its impact of quality of life make its early diagnosis imperative. Several diagnostic techniques ranging from simple clinical scales to sophisticated computerized tests exist, yet diagnosis remains a challenge, due to the time, cost, and personnel involved. Psychometric tests appear promising due to their high sensitivity and low cost, but results are variable depending on age and education. The pros and cons of current diagnostic methods for OHE and CHE are reviewed, along with strategy for CHE testing. PMID:24357348

  18. Encephalopathy: an uncommon manifestation of workplace arsenic poisoning?

    PubMed

    Morton, W E; Caron, G A

    1989-01-01

    This report describes two cases of chronic encephalopathy associated with occupational exposure to arsenic fumes from hot pressurized impregnation of wood. Both cases displayed symptoms of cognitive impairment with onset 14-18 months after start of occupational exposure to arsenic fumes. Laboratory confirmation was provided by elevated urine arsenic levels. One patient was hospitalized for apparent psychiatric reasons. Neuropsychologic testing of one case showed typical and relatively mild impairments of new learning, recent memory, and concentration in addition to the psychological symptoms. Symptoms in both cases disappeared following cessation of exposure and return of urine arsenic excretion to normal levels. In future studies of workers exposed to arsenic, documentation of mild impairment of cognitive function should be sought using neurobehavioral test batteries.

  19. Encephalopathy in Wilson disease: copper toxicity or liver failure?

    PubMed

    Ferenci, Peter; Litwin, Tomasz; Seniow, Joanna; Czlonkowska, Anna

    2015-03-01

    Hepatic encephalopathy (HE) is a complex syndrome of neurological and psychiatric signs and symptoms that is caused by portosystemic venous shunting with or without liver disease irrespective of its etiology. The most common presentation of Wilson disease (WD) is liver disease and is frequently associated with a wide spectrum of neurological and psychiatric symptoms. The genetic defect in WD leads to copper accumulation in the liver and later in other organs including the brain. In a patient presenting with Wilsonian cirrhosis neuropsychiatric symptoms may be caused either by the metabolic consequences of liver failure or by copper toxicity. Thus, in clinical practice a precise diagnosis is a great challenge. Contrary to HE in neurological WD consciousness, is very rarely disturbed and pyramidal signs, myoclonus dominate. Asterixis and many other clinical symptoms may be present in both disease conditions and are quite similar. However details of neurological assessment as well as additional examinations could help in differential diagnosis.

  20. Posterior reversible encephalopathy syndrome: the importance of early diagnosis.

    PubMed

    Teotónio, Rute; Marmoto, Dina; Januário, Cristina; Bento, Conceição

    2012-09-17

    A 14-year-old boy was submitted to cardiac transplant due to a dilated cardiomyopathy. On the fourth day of immunosuppression (corticosteroids, mycophenolate mofetil and tacrolimus), he developed right focal seizures and drowsiness. Blood pressure was in the normal range and laboratory findings in cerebral spinal fluid and blood were unremarkable, with drugs in non-toxic levels. The EEG showed a slow background rhythm more pronounced on the right and a seizure onset in the right occipital region. MRI revealed a diffuse hyperintense subcortical white-matter lesion on fluid attenuated inversion recovery, with lesser involvement of left temporal-occipital region. There was no enhancement with gadolinium and MRI diffusion-weighted imaging was consistent with vasogenic oedema. Tacrolimus was stopped with regression of MRI abnormalities and clinical recovery. Posterior reversible encephalopathy associated with tacrolimus is a rare but potentially serious complication of solid organ transplants. A prompt diagnosis and correct treatment is essential to avoid irreversible brain damage.