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Neurology 2001;57:1499-1502
© 2001 American Academy of Neurology
Brief Communications

An autosomal dominant disorder with episodic ataxia, vertigo, and tinnitus

J. L. Steckley, MSc, G. C. Ebers, MD FRCP(C);, M. Z. Cader, MRCP(UK), MBChB(Hons), BSc(Hons) and R. S. McLachlan, MD FRCP(C)

From the Wellcome Trust Center for Human Genetics (J.L. Steckley), Oxford; Department of Clinical Neurology (Drs. Ebers and Cader), Radcliffe Infirmary, Oxford, UK; and the Department of Medicine (Dr. McLachlan), Shaikh Khalifa Medical Center, Abu Dhabi, United Arab Emirates.

Address correspondence and reprint requests to Professor George C. Ebers, Department of Clinical Neurology, Radcliffe Infirmary, Woodstock Road, Oxford, UK, OX2 6HG; e-mail: george.ebers{at}clneuro.ox.ac.uk

The authors report an autosomal dominant episodic ataxia that is clinically distinct from the other episodic ataxias. Vestibular ataxia, vertigo, tinnitus, and interictal myokymia are prominent; attacks are diminished by acetazolamide. Linkage analyses of markers flanking the EA1 and EA2 loci demonstrate genetic exclusion from the other autosomal dominant episodic ataxias. The authors suggest EA3 for periodic vestibulocerebellar ataxia and EA4 for the disorder described here.




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