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

A girl of 10-5/12 years is described, who had diabetes mellitus from the age of 5 years on and who developed bilateral ptosis, pigment degeneration of the retina and bilateral impairment of hearing at the age of nine years. A few weeks before death she suffered from an acute gastrointestinal infection which was successfully treated by a hydroxyquinoline derivative. In the days following a severe encephalopathy and signs of cardiac involvement appeared. A month later the girl died of bulbar paralysis and acute heart failure. Histology showed remnants of a granulomatous inflammation in the heart, the kidneys, the pancreas and the skeletal muscles. Furthermore there was a widespread spongiosis in the white substance of the brain, with large astrocytes, and partly also in the basal ganglia, the brain stem and the cerebellum. Foci of sudanophilic tissue necrosis resembling Wernicke's Encephalopathy were found in the medulla oblongata and the spinal cord. The peripheral nerves appeared partially demyelinated and showed axonal lesions. This case is classified as a Juvenile Type of so-called Canavan's Disease. It shows some resemblence to the "Progressive Chronic Ophthalmoplegia with Spongiform Encephalopathy described by Daroff, Kearn and Sayre. The possible neurotoxical effects of the hydroxyquinoline therapy are discussed.
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PMID:[Juvenile spongy dystrophy of CNS with necrosis of the medulla. A. complication of hydroxyquinoline therapy (author's transl)]. 124 13

A retrospective study was performed for all patients diagnosed with haemorrhagic shock and encephalopathy syndrome (HSES) over an 11 year period (1984-94). Soroka University Medical Centre is the only medical facility in the southern Negev region of Israel serving a population of about 400,000 residents, consisting primarily of two ethnic populations, Jews and Bedouins. Twenty patients, 17 Bedouin and three Jews, were diagnosed with HSES. The annual incidence of HSES for infants under the age of 1 year was 5:10,000 for Bedouins and 0.6:10,000 for Jews. Patients ranged in age from 6 to 32 weeks and arrived at the hospital late at night or early morning (2:00 am to 11:00 am), during the winter or early spring (November to April). All were healthy before admission, with short prodromal symptoms of upper respiratory tract or gastrointestinal infection noted in 10 cases. Most infants had markedly high body temperature on arrival. A history of overwrapping and/or excessive heating was obtained in four of 20 infants. Bacteriological and virological cultures were negative in all infants. One infant died and neurological sequelae were observed in all survivors. The high prevalence of hyperpyrexia during sleep in the presence of negative microbiological results with no evidence of excessive heating, and the high incidence of HSES among a closed and culturally isolated society known to have a high incidence of congenital malformations, may support previous assumptions that HSES results from hyperpyrexia, originating in most cases from a 'physiological' heat induced trigger, which starts and peaks during the night in previously healthy infants who are genetically susceptible.
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PMID:Possible aetiology of haemorrhagic shock and encephalopathy syndrome in the Negev area of Israel. 898 22

Progressive encephalopathy with edema, hypsarrhythmia and optic atrophy (PEHO) syndrome is a rare neurodegenerative syndrome first reported in 1991. Most patients described are of Finnish descent and very few patients have been reported in other countries. We report the first Swiss patient who fulfils the criteria of the PEHO syndrome. The course of the epilepsy is less severe than previously reported. Our patient developed a severe dystonic state after a febrile gastrointestinal infection, with a hypotonic state that may have been a dysregulation of brainstem origin. The diagnosis was made because of marked cerebellar atrophy in the repeated MRI. In patients with infantile spasms and severe developmental delay PEHO syndrome should be considered; it is not confined to Finnish heritage. Optic atrophy should be looked for and repeat MRI is indicated.
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PMID:Progressive encephalopathy with edema, hypsarrhythmia and optic atrophy (PEHO) syndrome in a Swiss child. 1554 87

Acute necrotizing encephalopathy of childhood (ANEC) is a disease, characterized by a respiratory or gastrointestinal infection, accompanied with fever, rapid alteration of consciousness, and seizures. The clinical characteristics of ANEC include acute encephalopathy following a viral infection, seizure, altered consciousness, and absence of cerebrospinal fluid (CSF) pleocytosis, with an occasional increase in the level of proteins. This disease is almost exclusively seen in previously healthy infants and children from East Asia. Serial magnetic resonance imaging (MRI) examinations have demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. ANEC has a poor prognosis with high morbidity and mortality rates. Herein, we present three cases of ANEC, who were referred to Bu-Ali Hospital of Ardabil, Iran during two weeks. Report of these three cases promoted the idea of an epidemic. The purpose of this case series was to raise the issue that ANEC may occur as an epidemic.
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PMID:Three Cases of Acute Necrotizing Encephalopathy: Is It an Epidemic or Only Incidental? 3225 27