Síndrome cortico-basal como una presentación clínica de una enfermedad de Creutzfeldt-Jakob esporádica. Corticobasal syndrome as a clinical spectrum of a sporadic Creutzfeldt-Jakob disease.

PALABRAS CLAVE: Degeneracion cortico-basal, demencia, priones, sindrome de Creutzfeldt-Jakob. SUMMARY Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease characterized by rapidly progressive dementia, myoclonus, motor impairment and typical features on complementary tests; however, unusual clinical features might be associated. We report one case of sporadic CJD associated with corticobasal syndrome characterized by asymmetric limb apraxia, cortical sensory impairment, alien limb phenomenon, bradykinesia and rigidity; which is the classic clinical spectrum of the corticobasal degeneration. In addition, findings in electroencephalography (EEG), brain magnetic resonance imaging (MRI) and positive CSF protein 14-3-3 were compatible with probable sporadic CJD. This case suggest that asymmetric neurologic impairment may be associated with sporadic CJD.