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

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.

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Main Authors: Brito-Marques,Janaina Mariana de Araujo Miranda, Melo,Eduardo Sousa de, Medeiros,Fabíola Lys de, Carvalho,Cristiano Sobral de, Brito-Marques,Paulo Roberto de
Format: Digital revista
Language:English
Published: Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento 2021
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642021000400548
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spelling oai:scielo:S1980-576420210004005482021-12-01Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob diseaseBrito-Marques,Janaina Mariana de Araujo MirandaMelo,Eduardo Sousa deMedeiros,Fabíola Lys deCarvalho,Cristiano Sobral deBrito-Marques,Paulo Roberto de dementia prion disease hypoacusis 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.info:eu-repo/semantics/openAccessAcademia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e EnvelhecimentoDementia & Neuropsychologia v.15 n.4 20212021-12-01info:eu-repo/semantics/reporttext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642021000400548en10.1590/1980-57642021dn15-040016
institution SCIELO
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libraryname SciELO
language English
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author Brito-Marques,Janaina Mariana de Araujo Miranda
Melo,Eduardo Sousa de
Medeiros,Fabíola Lys de
Carvalho,Cristiano Sobral de
Brito-Marques,Paulo Roberto de
spellingShingle Brito-Marques,Janaina Mariana de Araujo Miranda
Melo,Eduardo Sousa de
Medeiros,Fabíola Lys de
Carvalho,Cristiano Sobral de
Brito-Marques,Paulo Roberto de
Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
author_facet Brito-Marques,Janaina Mariana de Araujo Miranda
Melo,Eduardo Sousa de
Medeiros,Fabíola Lys de
Carvalho,Cristiano Sobral de
Brito-Marques,Paulo Roberto de
author_sort Brito-Marques,Janaina Mariana de Araujo Miranda
title Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
title_short Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
title_full Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
title_fullStr Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
title_full_unstemmed Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
title_sort bilateral hearing loss as an initial presentation of creutzfeldt-jakob disease
description 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.
publisher Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento
publishDate 2021
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642021000400548
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