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Deafness: an unusual onset of genetic Creutzfeldt-Jakob disease

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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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Authors and Affiliations

  1. Department of Neurological Sciences, University of Catania, Via S. Sofia 78, I-95125 Catania, Italy, , , , , , IT

    M.L. Cataldi, O. Restivo, E. Reggio, D.A. Restivo & A. Reggio

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  1. M.L. Cataldi
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  2. O. Restivo
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  3. E. Reggio
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  4. D.A. Restivo
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  5. A. Reggio
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Received: 27 October 1999 / Accepted in revised form: 30 January 2000

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Cataldi, M., Restivo, O., Reggio, E. et al. Deafness: an unusual onset of genetic Creutzfeldt-Jakob disease. Neurol Sci 21, 53–55 (2000). https://doi.org/10.1007/s100720070119

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  • DOI: https://doi.org/10.1007/s100720070119

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