A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features

• Tipo: Artigo em Revista Científica Internacional
• Data: 2003

Autores

Silva, AM (Autor) Outra; Pires, MM (Autor) Outra; Bastos-Leite AJ (Autor) Outra; Honavar, M (Autor) Outra; Mendes, A (Autor) Outra; Correia, M (Autor) Outra; Nora, M (Autor) Outra; Silva, MR (Autor) Outra; Costa, M (Autor) Outra; Guimaras, A (Autor) Outra; Monteiro, L (Autor) Outra

Revista

Título: Arquivos de Neuro-Psiquiatria

Vol. 61 Nº 4

Páginas: 950-956

ISSN: 0004-282X

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

Indexação

ISI Web of Knowledge - 4 Citações

Outras Informações

ID Authenticus: P-000-E6H

DOI: 10.1590/s0004-282x2003000600012

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.

Idioma: Português

Tipo (Avaliação Docente): Científica

Nº de páginas: 7