NR AAWD
AU Azevedo,M.F.; Nascimento,F.; Quintella,L.; Rosso,A.L.; Maranhao Filho,P.A.; Correa,R.B.; Chimelli,L.; Vincent,M.; Novis,S.A.
TI [Creutzfeldt-Jakob disease: case report with spinal cord involvement]
OT Doenca de Creutzfeldt-Jakob: a proposito de um caso com comprometimento medular.
QU Arquivos de Neuro-Psiquiatria 2001 Dec; 59(4): 964-7
IA http://www.scielo.br/pdf/anp/v59n4/a24v59n4.pdf
PT journal article
AB Creutzfeldt-Jakob disease (CJD) is the most common subacute transmissible spongiform encephalopathy. Approximately 85% of the cases are sporadic. The remaining 15% consist of genetic and iatrogenic forms. We report a sporadic form of CJD with spinal cord involvement and a clinical manifestation characterized by dementia and cerebellar syndrome, myofasciculation with absent reflexes and seizures. The two last manifestations are rare. The clinical hypothesis was probable CJD which was confirmed with autopsy and immunohistochemistry. We conclude that CJD should always be suspected when rapidly progressive dementia occurs and the absence of pyramidal or extrapyramidal signs suggest a spinal cord and/or peripheral nerve involvement.
MH Adult; Cerebellum/pathology; Cerebral Cortex/pathology; Creutzfeldt-Jakob Syndrome/complications/*pathology; Dementia/complications; Disease Progression; English Abstract; Fatal Outcome; Female; Humans; Immunohistochemistry; Prions/analysis; Spinal Cord Diseases/etiology/*pathology
AD Instituto de Neurologia Deolindo Couto, Universidade Federal do Rio de Janeiro, Brazil. mfaa@openlink.com.br
SP portugisisch
PO Brasilien