Sporadic creutzfeldt-Jakob disease. A clinical-neuropathological analysis of nine definite cases
Arq. neuropsiquiatr
;
56(3A): 356-65, set. 1998. ilus, tab
Article
in English
| LILACS
| ID: lil-215291
ABSTRACT
The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100 per cent of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56 per cent of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100 per cent of the patients. In 5 (56 per cent) of these 9 cases, prion protein (PrP) amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Creutzfeldt-Jakob Syndrome
Limits:
Female
/
Humans
Language:
English
Journal:
Arq. neuropsiquiatr
Journal subject:
Neurology
/
Psychiatry
Year:
1998
Type:
Article
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