Literature DB >> 10938203

Deafness: an unusual onset of genetic Creutzfeldt-Jakob disease.

M L Cataldi1, O Restivo, E Reggio, D A Restivo, A Reggio.   

Abstract

We describe a case of genetic Creutzfeldt-Jakob disease (CJD) with deafness at the onset. We report clinical features, 14-3-3 protein positivity, electroencephalography and brain stem auditory evoked potential abnormalities, and high signal on magnetic resonance imaging in basal ganglia and temporal cortex. Similarities with CJD Heidenhain variant are discussed.

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Year:  2000        PMID: 10938203     DOI: 10.1007/s100720070119

Source DB:  PubMed          Journal:  Neurol Sci        ISSN: 1590-1874            Impact factor:   3.307


  5 in total

1.  Familial Creutzfeldt-Jakob disease with E200K mutation presenting with neurosensorial hypoacusis.

Authors:  R Reñé; J Campdelacreu; I Ferrer; A Escrig; M Povedano; J Gascón-Bayarri; E Moral
Journal:  J Neurol Neurosurg Psychiatry       Date:  2007-01       Impact factor: 10.154

2.  Familial Creutzfeldt-Jakob disease with E200K mutation presenting with neurosensorial hypoacusis.

Authors:  R Reñé; J Campdelacreu; I Ferrer; A Escrig; M Povedano; J Gascón-Bayarri; E Moral
Journal:  BMJ Case Rep       Date:  2009-07-07

3.  Distinct neuropsychological characteristics in Creutzfeldt-Jakob disease.

Authors:  J S Snowden; D M A Mann; D Neary
Journal:  J Neurol Neurosurg Psychiatry       Date:  2002-12       Impact factor: 10.154

4.  Unusual presentations in patients with E200K familial Creutzfeldt-Jakob disease.

Authors:  O S Cohen; I Kimiagar; A D Korczyn; Z Nitsan; S Appel; C Hoffmann; H Rosenmann; E Kahana; J Chapman
Journal:  Eur J Neurol       Date:  2016-01-25       Impact factor: 6.089

5.  Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies.

Authors:  Anna Ladogana; Pascual Sanchez-Juan; Eva Mitrová; Alison Green; Natividad Cuadrado-Corrales; Raquel Sánchez-Valle; Silvia Koscova; Adriano Aguzzi; Theodoros Sklaviadis; Jerzy Kulczycki; Joanna Gawinecka; Albert Saiz; Miguel Calero; Cornelia M van Duijn; Maurizio Pocchiari; Richard Knight; Inga Zerr
Journal:  J Neurol       Date:  2009-05-15       Impact factor: 4.849

  5 in total

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