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Case Reports
. 2021 Oct-Dec;15(4):548-549.
doi: 10.1590/1980-57642021dn15-040016.

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

Janaina Mariana de Araujo Miranda Brito-Marques  1 , Eduardo Sousa de Melo  1   2 , Fabíola Lys de Medeiros  1 , Cristiano Sobral de Carvalho  1 , Paulo Roberto de Brito-Marques  1   3
Affiliations
Case Reports

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

Janaina Mariana de Araujo Miranda Brito-Marques et al. Dement Neuropsychol. 2021 Oct-Dec.

Abstract
in English, Portuguese

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.

Relatamos o caso de um paciente do sexo masculino, 61 anos, com anacusia, síndrome cerebelar, mioclonia e sinais frontais. A ressonância magnética cerebral mostrou hiperintensidade estriada bilateral do fluid-attenuated inversion recovery (FLAIR) e difusão restrita no diffusion-weighted imaging (DWI) e áreas hipointensas correspondendo ao coeficiente de difusão aparente no córtex cerebral. A autópsia revelou imuno-histoquímica positiva para a proteína PrPSc. A doença de Creutzfeldt-Jakob que se apresenta com perda auditiva é incomum.

Keywords: dementia; hypoacusis; prion disease.

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Conflict of interest statement

Disclosure: The authors report no conflicts of interest.

Figures

Figure 1.
Figure 1.. Brain MRI. Panel A show hyperintensity in bilateral striatum in the FLAIR sequence. Panels B and C shows water restriction in cortex with a predominance in the right hemisphere (DWi/ADC map).
Figure 2.
Figure 2.. Electroencephalogram with periodic activity, biphasic or triphasic morphology, of 0.8–1.3 seconds, with diffuse distribution, lateralized to the right cerebral hemisphere, and predominantly posterior.
See this image and copyright information in PMC

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