NR ACII
AU Cataldi,M.L.; Restivo,O.; Reggio,E.; Restivo,D.A.; Reggio,A.
TI Deafness: an unusual onset of genetic Creutzfeldt-Jakob disease.
QU Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2000 Feb; 21(1): 53-5
ER Neurol Sci 2000 Jun;21(3):172
PT journal article
AB 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.
MH Basal Ganglia/pathology; Case Report; Creutzfeldt-Jakob Syndrome/diagnosis/*genetics/metabolism; Deafness/*genetics; Electroencephalography; Evoked Potentials, Auditory, Brain Stem; Female; Human; Magnetic Resonance Imaging; Middle Age; Temporal Lobe/pathology; Tyrosine 3-Monooxygenase/metabolism
AD Department of Neurological Sciences, University of Catania, Italy.
SP englisch
PO Italien