돌발성 이상운동증의 임상양상 분석 및 실용적 분류법 |
김지수, 전범석, 임주혁*, 이근호**, 이한보***, 박성호***, 이명종*, 명호진 |
서울대학교 의과대학 신경과학교실, 울산대학교 의과대학 신경과학교실*, 단국대학교 의과대학 신경과학교실**, 보라매병원 신경과*** |
Clinical Characteristics and Practical Classification of Paroxysmal Dyskinesia |
J. S. Kim, M.D., Beom S. Jeon, M.D., J.H. Im, M.D.*, G.H. Lee, M.D.**, H.B. Lee, M.D. ***, S.H. Park, M.D.***, M.J. Lee, M.D.*, H.J. Myung, M.D. |
Department of Neurology, College of Medicine, Seoul National University
Ulsan University*, DanKook University**, Boramae Hospital*** |
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Abstract |
Background and Objectives : Paroxysmal dyskinesia is a heterogenous group of disorders with diverse clinical features. A number of different terms have been used to describe patients, and there have been varied classifications mainly based on the etiology, duration of attacks and precipitating factors. We examined the clinical characteristics of 25 patients with paroxysmal dyskinesia to find the practical classification criteria. Methods : Paroxysmal dyskinesias were classified as paroxysmal kinesigenic dyskinesia (PKD) , paroxysmal nonkinesigenic dyskinesia(PNKD), paroxysmal exertion-induced dyskinesia(PED), paroxysmal hypnogenic dyskinesia(PHD). Movement pattern, duration, frequency, other clinical features and treatment response were examined, Results : Eighteen out of 25 patients(72.0%) had PKD, and 7(28.0%) had PNKD. One patient had both PKD and PED. There was no patient with PHD. Seventeen of 18 PKD patients (94.4%) were male. In PNKD, 2 were male and 5, female. The mean age of onset in PKB was 13.1 ± 2.6 years, which was younger than that in PNKD (30.3 ± 9.6 years). Eleven PKD patients (61.1%) had family history of PlID. In PNKD, only one patient with periodic ataxia had family history. In PKD, neurologic examination, EEG and brain imagings were normal except in one patient who had complex partial seizure. Most common causes of PNKD were psychogenic (in 4) and multiple sclerosis(in 2) . The duration of attacks in PKDs was less than 5 minutes in all patients. Most common movement patterns were chorea and dystonia. PKD affected both sides in 14(77.8%) , however 4(22.2%) had pure unilateral Involvement. The most common aggravating factors of PKD were stress and fatigue. Twelve PKD patients (66.7%) had abnormal sensation or void feeling in the affected body parts just before the attacks. All PKD patients showed dramatic responses to the anticonvulsants. In one patient with PKD and PED, the choreic movements developed for several seconds to 1 minute after 5 to 10 minutes of running. He also responded to the anticonvulsant. The patterns of dyskinesia were diverse in PNKD, psychogenic tremor in 3, painful dystonic spasm in 2 with MS, periodic ataxia in 1 and chorea associated with dystonia and tremor in 1. The durations of PNKD attacks were varied from several seconds to hours. Paroxysmal psychogenic dyskinesia responded to psychotherapy. One periodic ataxia patient showed good response to acetazolamide. Painful tonic spasms were relieved by carbamazepine. Conclusion : The response to treatment was determined by precipitating factors. Duration of attacks did not influence the response. Classification based on the precipitating factors is more appropriate. |
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