[Creutzfeldt-Jakob disease: case report with spinal cord involvement].
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.
Azevedo
M F
MF
Instituto de Neurologia Deolindo Couto, Universidade Federal do Rio de Janeiro, Brazil. mfaa@openlink.com.br
Nascimento
F
F
Quintella
L
L
Rosso
A L
AL
Maranhão Filho
P A
PA
Corrêa
R B
RB
Chimelli
L
L
Vincent
M
M
Novis
S A
SA
D000328:Adult; D002531:Cerebellum / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000473:pathology*; D003704:Dementia / Q000150:complications; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D011328:Prions / Q000032:analysis; D013118:Spinal Cord Diseases / Q000209:etiology / Q000473:pathology*
D011328:Prions