Sporadic creutzfeldt-Jakob disease. A clinical-neuropathological analysis of nine definite cases
Arq. neuropsiquiatr
; 56(3A): 356-65, set. 1998. ilus, tab
Artigo
em Inglês
| LILACS
| ID: lil-215291
Biblioteca responsável:
BR1.1
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.
Texto completo:
Disponível
Coleções:
Bases de dados internacionais
Base de dados:
LILACS
Assunto principal:
Síndrome de Creutzfeldt-Jakob
Limite:
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Arq. neuropsiquiatr
Assunto da revista:
Neurologia
/
Psiquiatria
Ano de publicação:
1998
Tipo de documento:
Artigo