Sporadic Creutzfeldt-Jakob disease. A clinico-neuropathological analysis of nine definite cases

• Published in: Arquivos de neuro-psiquiatria Vol. 56; no. 3A; pp. 356 - 365
• Main Authors: Costa, C M, Brucher, J M, Laterre, C
• Format: Journal Article
• Language: English
• Published: Brazil Academia Brasileira de Neurologia - ABNEURO 01.09.1998; Academia Brasileira de Neurologia (ABNEURO)
• Subjects: Aged; Cerebellum - ultrastructure; Cerebellum - virology; Creutzfeldt-Jakob Syndrome - pathology; Creutzfeldt-Jakob Syndrome - virology; dementia; Female; Gliosis; Humans; Middle Aged; neuropathology; NEUROSCIENCES; periodic EEG; Plaque, Amyloid - pathology; prion disease; prion protein (PrP) amyloid plaque; Prions - isolation & purification; PSYCHIATRY; sporadic Creutzfeldt-Jakob disease; prion disease; placa amilóide de proteína prion (PrP); neuropatologia; doença por prion; EEG periódico; dementia; prion protein (PrP) amyloid plaque; demência; periodic EEG; sporadic Creutzfeldt-Jakob disease; doença de Creutzfeldt-Jakob do tipo esporádico; neuropathology
• ISSN: 0004-282X; 1678-4227; 0004-282X; 1678-4227
• DOI: 10.1590/s0004-282x1998000300003

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.