Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease

Dementia & Neuropsychologia

关键词: dementia;    prion disease;    hypoacusis;   

DOI  :  10.1590/1980-57642021dn15-040016

来源: DOAJ

【 摘 要 】

ABSTRACT. We reported a case of a 61-year-old male patient with anacusis, cerebellar syndrome, myoclonus, and frontal signs. The brain magnetic resonance imaging showed bilateral striated hyperintensity of the fluid-attenuated inversion recovery and restricted diffusion in the diffusion-weighted imaging and hypointense areas corresponding to the apparent diffusion coefficient in the cerebral cortex. The autopsy revealed positive immunohistochemistry for the PrPSc protein. Creutzfeldt–Jakob disease presenting with hearing loss is unusual.

【 授权许可】

Unknown