Hyper-Endorphin Syndrome in a Child with Necrotizing Encephalomyelopathy

SUBACUTE necrotizing encephalomyelopathy, or Leigh's syndrome, 1 is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebell...

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Veröffentlicht in:The New England journal of medicine 1980-10, Vol.303 (16), p.914-916
Hauptverfasser: Brandt, Niels Jacob, Brandt, Sven, Terenius, Lars, Jacobsen, Bendt Brock, Klinken, Leif, Nordius, Åsa, Blegvad, Karin, Yssing, Minna
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container_end_page 916
container_issue 16
container_start_page 914
container_title The New England journal of medicine
container_volume 303
creator Brandt, Niels Jacob
Brandt, Sven
Terenius, Lars
Jacobsen, Bendt Brock
Klinken, Leif
Nordius, Åsa
Blegvad, Karin
Yssing, Minna
description SUBACUTE necrotizing encephalomyelopathy, or Leigh's syndrome, 1 is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebellar cortex, and dentate nucleus, and they consist of bilateral symmetrical areas of rarefaction, loss of myelin, and cell proliferation in the capillaries and glia. This disorder occurs in infancy and early childhood and is inherited as an autosomal-recessive trait, but the basic biochemical defect is unknown. We report on a syndrome in a boy suffering from attacks mimicking acute morphine poisoning with prolonged apnea . . .
doi_str_mv 10.1056/NEJM198010163031604
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The histopathologic changes are found in the brain stem, basal ganglia, cerebellar cortex, and dentate nucleus, and they consist of bilateral symmetrical areas of rarefaction, loss of myelin, and cell proliferation in the capillaries and glia. This disorder occurs in infancy and early childhood and is inherited as an autosomal-recessive trait, but the basic biochemical defect is unknown. 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subjects Ataxia
Ataxia - etiology
Brain - pathology
Brain Chemistry
Brain Diseases - diagnosis
Brain Diseases - pathology
Brain Diseases - physiopathology
Case reports
Cerebrospinal Fluid - analysis
Dehydrogenases
Diagnosis, Differential
Endorphins
Endorphins - analysis
Endorphins - physiology
Enkephalins - analysis
Enkephalins - physiology
Fibroblasts
Humans
Infant
Injections, Intravenous
Intellectual Disability - etiology
Male
Metabolism
Metabolites
Morphine - poisoning
Naloxone - administration & dosage
Naloxone - therapeutic use
Narcotics
Necrosis
Pediatrics
Peptides
Poisoning
Respiration
Spinal Cord Diseases - diagnosis
Spinal Cord Diseases - pathology
Spinal Cord Diseases - physiopathology
Syndrome
Urine
title Hyper-Endorphin Syndrome in a Child with Necrotizing Encephalomyelopathy
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