Familial Periodic Vertigo and Ataxia   
                                        - A Case Report -

J Korean Neurol Assoc > Volume 16(1); 1998 > Article

Journal of the Korean Neurological Association 1998;16(1): 77-82.

가족성 주기성 현훈 및 운동 실조증 1례

김미숙, 김동욱 . 이태희 . 오희종*

울산 동강 병원 신경과 , 대구 효성 카톨릭 대학 신경과학 교실*

Familial Periodic Vertigo and Ataxia - A Case Report -

Mi Suk Kim M.D., Dong Wook Kim, M.D., Tae Hee Lee, M.D., Hee Jong Oh, M.D.*

Department of Neurology, Dong Kang Hospital, Ulsan Department of Neurology, School of Medicine, Catholic University of Taegu-Hyosung*

Abstract

Familial periodic vertigo and ataxia is a rare, disabling condition of autosomal dominant episodes of vertigo and/or ataxia, sometimes associated with other brain stem symptoms such as diplopia, weakness and dysarthria. These attacks typically begin in early childhood or early adulthood, last for hours to days. Attacks may occur daily, or may be separated by longer intervals. We present a family with familial periodic vertigo and ataxia. A 20-year-old woman presented recurrent paroxysmal vertigo, nausea, vomiting and ataxia lasting for 2 to 4 hours since middle school age. Recently daily attacks disabled her from daily living activity. Neurologic examination in the attack-free interval revealed downbeat nystagmus at neutral position and bilateral gaze evoked coarse horizontal nystagmus. Her mother, 47-year-old female also had recurrent vertigo, ataxia and dysarthria lasting for 1 to 2 hours since childhood. Their symptoms are precipitated by fatigue, exertion, emotional stress and alcohol. Both patients showed good response to acetazolamide and flunarizine even though showed persistent objective nystamus.