Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1994-12-20
pubmed:abstractText
Three autopsy cases of panencephalopathic type of familial Creutzfeldt-Jakob disease (CJD) were investigated. Cases 1 (51-year-old male) and 3 (54-year-old female) were siblings and Case 2 (68-year-old female) was their aunt. In cases 1 and 3, the age of onset (Case 1:51, Case 3:53), duration of illness (Case 1:9 months, Case 3:8 months) and neuropsychiatric symptoms (pyramidal and extrapyramidal tracts involvements, blindness and dementia in chronological order) were similar, but in Case 2, the onset was later (66 years old), duration was longer (32 months) and the initial symptom was dementia. Myoclonus and apallic state in the terminal stage were common to all 3 cases. Neuropathologically, all 3 cases had characteristics that indicated panencephalopathic type of CJD. Cases 1 and 3 had similar neuropathological findings with characteristic circumscribed necrotic foci in the subcortical white matter. In Case 2 in contrast, diffuse demyelination and fibrillary gliosis in the cerebral white matter were observed without circumscribed necrotic foci. In the cerebellum of Case 3, granular cell loss was very slight. The other lesions in the cerebral cortex and striatum of the 3 cases were common. In conclusion, the clinical symptoms and neuropathological findings of our familial CJD cases were different from one another.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:issn
0722-5091
pubmed:author
pubmed:issnType
Print
pubmed:volume
13
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
185-91
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:articleTitle
Familial Creutzfeldt-Jakob disease: three autopsy cases of the panencephalopathic type.
pubmed:affiliation
Department of Psychiatry, Shimane Medical University, Izumo, Japan.
pubmed:publicationType
Journal Article, Case Reports