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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty newborn pigs within 3 days of life were randomly divided into different groups according to the time of hypoxic-ischemic encephalopathy. The results showed that the brain damage and pathologic changes were most obvious in the 60 min hypoxia-ischemic group. Brain edema was observed in every group, especially in the group where hypoxia-ischemic lasted for more than 30 min. Cerebromalacia and hemorrhage in ventricles of brain and in arachnoid were observed, and these were particularly noticed in the group where hypoxia-ischemia lasted for 60 min.
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PMID:[Observation on the animal model of hypoxic-ischemic encephalopathy and the change of pathology in newborn pigs]. 858 4

The prenatal histories, clinical courses, and neuroradiographic studies of 8 infants who had survived the in utero demise of a homozygous co-twin were reviewed. Three distinct modes of clinical presentation were found: (1) severe neonatal encephalopathy with seizures; (2) a more benign neonatal course with onset of seizures and profound developmental disabilities within the first 6 months of age; (3) late infantile presentation with seizures. Only the third group had milder outcomes. Neuroradiographic studies demonstrated two pathologic patterns: varying degrees of periventricular white matter infarction with migrational abnormalities observed with earlier demise of the co-twin, and multicystic encephalomalacia observed when demise occurred at or near term. Pathophysiology is uncertain and most likely multifactorial. Exsanguination injury to the survivor can occur acutely following co-twin demise, so urgent delivery may be appropriate at or near term.
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PMID:Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins. 877 Nov 62

Fetal hypoxic-ischemic encephalopathy can be diagnosed at birth by means of cerebral ultrasound scanning. The morphological appearance of the lesions depends on the time elapsed between the insult and examination of the brain. We report a case of a neonate affected by multicystic encephalomalacia and corpus callosum atrophy attributable to an episode of maternal anaphylactic shock which occurred at 27 weeks of gestation following intravenous iron injection. The diagnosis was made by means of a cerebral ultrasound scan performed at birth and confirmed by magnetic resonance. This case demonstrates that maternal severe acute hypotension during pregnancy can cause fetal cerebral damage similar to the hypoxicischemic injuries occurring in the perinatal period.
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PMID:Fetal encephalopathy after maternal anaphylaxis. Case report. 909 98

In order to identify the brain lesions of symptomatic/cryptogenic partial epilepsies (S/CPEs) in infants and children, magnetic resonance imaging (MRI) studies, thorough encephalographic (EEGic) studies, and detailed clinical and neurologic evaluations were obtained in 300 infants and children who were diagnosed to have S/CPEs with onset before the age of 13 years during the past 7 years. The overall detection rate of brain lesions by MRI was 41.7% (125/300). Congenital malformations (18 cases), vascular malformations (9 cases), neurocutaneous syndromes (13 cases), and space-taking lesions (20 cases) constitute a large percentage of SPEs in infants and children. A variety of insults such as infection, ischemia, hemorrhage, trauma and metabolic disorders can result in destructive parenchymal loss lesions including porencephaly, focal atrophy, hemiatrophy, and diffuse brain atrophy (20 cases). Major etiologic factors leading to infarction, encephalomalacia, leukomalacia, included trauma, hvpoxicischemic encephalopathy (HIE), systemic lupus erythematosus (SLE), encephalitis, vasculitis, venous thrombosis, vasculopathies, and heart problems (22 cases). Mesial temporal sclerosis (MTS) could be evidenced in around 20% (18/95) of cases with temporal lobe epilepsy (TLE), which was strongly associated with past histories of febrile seizures and encephalitis complicated by status epileptics. However, cases with porencephaly, global atrophy or delayed myelination of unilateral temporal lobe on MRI were more related to HIE. With the advent of neuroimaging techniques, particularly MRI, a wide variety of underlying pathology can be detected as a cause of symptomatic partial epilepsies in pediatric patients. The occurrence of S/CPE indicates the presence of localized brain dysfunction, and many of the causes are potentially treatable. An orderly and thorough clinical and laboratory investigations, as well as neuroimaging studies should be made to diagnose and treat any underlying conditions.
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PMID:Magnetic resonance imaging in symptomatic/cryptogenic partial epilepsies of infants and children. 915 66

A 1-year-old female presented with neuroleptic malignant syndrome probably caused by methylphenidate. She had defects in the supratentorial brain including the basal ganglia and the striatum (multicystic encephalomalacia) due to severe perinatal hypoxic-ischemic encephalopathy, which was considered to be a possible predisposing factor causing neuroleptic malignant syndrome. A dopaminergic blockade mechanism generally is accepted as the pathogenesis of this syndrome. However, methylphenidate is a dopamine agonist via the inhibition of uptake of dopamine, and therefore dopaminergic systems in the brainstem (mainly the midbrain) and the spinal cord were unlikely to participate in the onset of this syndrome. A relative gamma-aminobutyric acid-ergic deficiency might occur because diazepam, a gamma-aminobutyric acid-mimetic agent, was strikingly effective. This is the first reported patient with neuroleptic malignant syndrome probably caused by methylphenidate.
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PMID:Neuroleptic malignant syndrome and methylphenidate. 983 Oct 2

Kasba (Chuzan) virus (an orbivirus), strain K-47, produced encephalopathy with severe necrosis in suckling mice inoculated intracerebrally. On day 3 after inoculation with 10(3)TCID50, the mice showed severe focal encephalomalacia and meningitis. On day 4, necrosis had spread to the midbrain, cerebellum and spinal cord. From one day after inoculation, virus was recovered from the brain and the titre rose over the next 3 days. Immunohistochemical examination demonstrated viral antigens in the cytoplasm of both degenerate and intact neurons, and ependymal cells in or around necrotic lesions. The study indicated that the virus has an affinity for immature nerve cells in the brains of suckling mice and causes primary encephalomalacia. Since the lesions resembled those of the hydranencephaly-cerebellar hypoplasia syndrome in calves (Chuzan disease), the system described should prove useful in studies on pathogenesis.
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PMID:Encephalopathy in suckling mice infected with Kasba (Chuzan) virus. 1021 69

Carbamyl phosphate synthetase I is a urea cycle enzyme. Severe deficiency of carbamyl phosphate synthetase I presents in the neonatal period as hyperammonemic encephalopathy with altered consciousness and occasional seizures after feeding begins. Episodes of altered consciousness with or without seizures and focal neurologic deficits are seen later with patients of partial carbamyl phosphate synthetase I deficiency. Fatal cerebral edema with brain herniation may develop on occasion. Three patients presenting with carbamyl phosphate synthetase I deficiency are reported with neuroimaging and pathologic findings illustrating the destructive encephalopathy with acute cerebral edema, followed by diffuse cerebral atrophy and occasional cystic encephalomalacia. The deterioration in carbamyl phosphate synthetase I deficiency occurs during the hyperammonemic crises. This deficiency may be difficult to treat despite the current advances in treatment strategies, especially in neonatal-onset patients with low carbamyl phosphate synthetase I activity.
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PMID:Carbamyl phosphate synthetase 1 deficiency: a destructive encephalopathy. 1130 Dec 19

We report three infants with the diagnosis of molybdenum cofactor deficiency. The key findings leading to diagnosis were neonatal seizures unresponsive to treatment, craniofacial dysmorphic features, hyperexcitability, low blood uric acid levels, and neuroimaging findings. The parents were consanguineous in two of these patients. The diagnosis was established by the presence of low blood uric acid levels, positive urine sulfite reaction, quantitative aminoacid analysis, and high-voltage electrophoresis of the urine sample showing a typical increase of S-sulfo-L-cysteine. Skin fibroblast cultures confirmed the diagnosis. Magnetic resonance imaging findings were suggestive of encephalomalacia with cystic changes due to hypoxic-ischemic encephalopathy. We conclude that molybdenum cofactor deficiency must be included in the differential diagnosis of patients presenting with intractable seizures in the newborn period who have computed tomography and magnetic resonance imaging findings reminiscent of those of hypoxic-ischemic encephalopathy, and the urine sulfite dipstick test can be a part of the evaluation of these infants in neonatal intensive care units.
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PMID:Molybdenum cofactor deficiency: report of three cases presenting as hypoxic-ischemic encephalopathy. 1133 61

Twenty-four patients with hypoxic-ischemic encephalopathy were examined with serial magnetic resonance imaging up to 4 years of age. Magnetic resonance imaging studies were performed in the neonatal period, at the fourth month and the fourth year of age, and the findings were compared with the patients' neurodevelopmental outcome at the fourth year of age. Periventricular signal alterations and deep gray matter involvement were usually evident in the initial magnetic resonance imaging studies, and encephalomalacia, periventricular leukomalacia, and atrophy were the common findings on follow-up magnetic resonance imaging studies. In the patients with hypoxic-ischemic encephalopathy, some correlation between magnetic resonance imaging findings and neurodevelopmental outcome was recognized. The patients with deep gray matter involvement on the initial magnetic resonance imaging had a poor prognosis, and the ones with normal magnetic resonance imaging findings had a favorable neurodevelopmental outcome. On the follow-up magnetic resonance imaging findings, encephalomalacia and periventricular leukomalacia were associated with poor neurodevelopmental outcome. In predicting the neurologic outcome at 4 years of age, magnetic resonance imaging findings of the neonatal period had the highest negative predictive value, whereas magnetic resonance imaging findings at 4 months of age and 4 years of age had the highest positive predictive value.
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PMID:Hypoxic-ischemic encephalopathy: correlation of serial MRI and outcome. 1546 39

A frequent polymorphism in the gene coding for 5,10-methylenetetrahydrofolate reductase is the substitution 677C > T which produces a thermolabile and inefficient enzyme. Homozygosity for the 677C > T allele is the most important determinant of hyperhomocys-teinemia, when folic acid intake is reduced. Most studies on the relationship between the 677C > T variant in the mother and defects in the offspring have focused on neural-tube defects. This study is a retrospective case-control investigation of hypoxic-ischemic encephalopathy of the newborn (HIEN) with reference to the 677C > T polymorphism as a genetic risk for this condition. The prevalence of the 677C > T allele was studied in 11 children with HIEN, their respective mothers, and 85 healthy individuals. Plasma homocysteine levels after fasting and methionine loading were determined in both mothers and controls. Ten of 11 patients were evaluated using magnetic resonance (MR) imaging, and all showed multicystic encephalomalacia and severe brain vasculopathy. Seven mothers were homozygous and four heterozygous for the 677C > T allele. Five of the children were homozygous and six heterozygous for this polymorphism. The variant allele frequency was higher in the group of mothers with affected children than in the controls and was associated with an increase in plasma homocysteine after methionine loading, in the group of mothers than in controls. The 677C > T mutation in mothers, either in a homozygous or heterozygous state, together with poor nutritional status (probable folate deficiency) may represent a risk factor for irreversible HIEN in the offspring.
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PMID:Maternal mutation 677C > T in the methylenetetrahydrofolate reductase gene associated with severe brain injury in offspring. 1561 51


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