Linked Life Data

988913

Source:http://linkedlifedata.com/resource/pubmed/id/988913

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1976-10-2
pubmed:abstractText
A girl with non-contributory family history, at the age of 7 years developed a recurrent ataxic-spastic syndrome followed by disorders of consciousness and seizures. She died in a decerebrate state at the age of 10 9/12 years. Diffuse EEG abnormalities changed in accordance with the clinical course. CSF findings were always negative. Brain biopsy performed at the age of 9 5/12 years was non-diagnostic. No clinical signs of adrenal insufficiency were present. Neuropathological examination disclosed severe demyelination of the cerebral white matter with formation of large periventricular cavities, relative sparing of the subcortical arcuate fibers, and peripheral myelin breakdown of the orthochromatic (neutral fat) type. There was complete absence of inflammatory lesions, comparative preservation of axons and oligodendroglia with moderate astroglial reaction. The optic system and cerebellum were not involved. Additional postanoxic changes were seen in the thalamus and hippocampus. The sporadic case is suggested to represent a cavitating form of neutral fat (simple orthochromatic) leukodystrophy which is to be separated from Schilder's diffuse sclerosis and adrenoleukodystrophy. The etiology of the disorder is unknown.
pubmed:language
ger
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0028-3797
pubmed:author
pubmed:issnType
Print
pubmed:volume
7
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
111-21
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1976
pubmed:articleTitle
[Cavitating neutral fat leukodystrophy with recurrent course].
pubmed:publicationType
Journal Article, English Abstract, Case Reports