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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Sprache:eng
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Zusammenfassung: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 . . .
ISSN:0028-4793
1533-4406
DOI:10.1056/NEJM198010163031604