Deafness: an unusual onset of genetic Crewtzfeldt-Jakob disease

Authors
Cataldi, ML Restivo, O Reggio, E Restivo, DA Reggio, A
Citation
Ml. Cataldi et al., Deafness: an unusual onset of genetic Crewtzfeldt-Jakob disease, NEUROL SCI, 21(1), 2000, pp. 53-55
Citations number
20
Categorie Soggetti
Neurology
Journal title
NEUROLOGICAL SCIENCES
ISSN journal
15901874 → ACNP
Volume
21
Issue
1
Year of publication
2000
Pages
53 - 55
Database
ISI
SICI code
1590-1874(200002)21:1<53:DAUOOG>2.0.ZU;2-T
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, elec troencephalography 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.