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Bilateral Vestibular Failure as an Early Sign in Creutzfeldt‐Jakob Disease
Author(s) -
Jahn Klaus,
Arbusow Viktor,
Zingler Vera C.,
Strupp Michael,
Kretzschmar Hans A.,
Brandt Thomas
Publication year - 2009
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.2008.03741.x
Subject(s) - oscillopsia , vestibular system , medicine , audiology , vestibulo–ocular reflex , nystagmus , disease , reflex , amyotrophic lateral sclerosis , pathology , anesthesia
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.