NR ACWX
AU Costa,C.M.; Brucher,J.M.; Laterre,C.
TI Sporadic Creutzfeldt-Jakob disease. A clinico-neuropathological analysis of nine definite cases.
QU Arquivos de Neuro-Psiquiatria 1998 Sep; 56(3A): 356-65
PT journal article
AB 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% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% 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% of the patients. In 5 (56%) 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.
MH Aged; Case Report; Cerebellum/ultrastructure/virology; Creutzfeldt-Jakob Syndrome/*pathology/virology; Female; Gliosis; Human; Middle Age; Prions/isolation & purification; Senile Plaques/pathology
AD Service of Neurology (Hospital University)/Laboratory of Experimental Neurology (Department of Physiology and Pharmacology), Federal University of Ceara (UFC), Brazil.
SP englisch
PO Brasilien