A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: Demographic, clinical and neuropathological features [Estudo retrospectivo da doença de Creutzfeldt-Jakob diagnosticada no Norte de Portugal entre 1993-2002: Características demográficas, clínicas e neuropatológicas]

• Type: Article in International Scientific Journal
• Date: 2003

Authors

Silva, AM (Author) Other; Manuel Pires (Author) ICBAS; Bastos-Leite AJ (Author) Other; Honavar, M (Author) Other; Mendes, A (Author) Other; Correia, M (Author) Other; Nora, M (Author) Other; Silva, MR (Author) Other; Costa, M (Author) Other; Guimaraes, A (Author) Other; Monteiro, L (Author) Other

Journal

Title: Arquivos de Neuro-Psiquiatria

Vol. 61 No. 4

Pages: 950-956

ISSN: 0004-282X

Publisher: Associacao Arquivos de Neuro-Psiquiatria Dr. Oswaldo Lange

Indexing

Scopus - 5 Citations

Other information

Authenticus ID: P-007-C3P

Abstract (EN): Objective: Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD). Method: Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002. Results: Eleven patients were identified, 4 females: mean age of onset of symptoms - 64 years, mean duration of disease - 8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive. Conclusion: The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.

Language: Portuguese

Type (Professor's evaluation): Scientific