Vol. 15 nº 4 - Oct/Nov/Dec de 2021
Case Report Páginas: 548 a 549

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

Authors Janaina Mariana de Araujo Miranda Brito-Marques1; Eduardo Sousa de Melo1,2; Fabíola Lys de Medeiros1; Cristiano Sobral de Carvalho1; Paulo Roberto de Brito-Marques1,3


keywords: dementia, prion disease, hypoacusis.

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.


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