Bilateral vestibular failure as an early sign in Creutzfeldt-Jakob disease.
Bilateral vestibular failure causes dysfunction of the vestibulo-ocular reflex with consecutive symptoms like apparent movements of the visual environment during head movements (oscillopsia) and unsteady gait in darkness or on uneven ground. A definite case of Creutzfeldt-Jakob disease in which bilateral vestibular loss was one of the first clinical signs is reported. Further, in a series of 9 consecutive patients with Creutzfeldt-Jakob disease (6 probable, 3 definite), 3 had bilateral vestibular loss at initial presentation. The vestibular nuclei are known to be severely affected in animal spongiform encephalopathies. They might also be a vulnerable target in human prion diseases.
Jahn
Klaus
K
Departments of Neurology, of Neurology, Klinikum Grosshadern Marchioninistrasse 15, 81377 Munich, Germany. klaus.jahn@med.uni-muenchen.de
Arbusow
Viktor
V
Zingler
Vera C
VC
Strupp
Michael
M
Kretzschmar
Hans A
HA
Brandt
Thomas
T
D015153:Blotting, Western; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D012027:Reflex, Vestibulo-Ocular; D015837:Vestibular Diseases / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology