Clinical View Points on Primary Dystonia and Dystonia-plus Syndrome

J Korean Neurol Assoc > Volume 22(6); 2004 > Article

Journal of the Korean Neurological Association 2004;22(6): 569-576.

임상적 관점에서 본 Primary Dystonia와 Dystonia-plus Syndrome

한양대학교 의과대학 신경과학교실

Clinical View Points on Primary Dystonia and Dystonia-plus Syndrome

Hee-Tae Kim

Department of Neurology, Hanyang University College of Medicine, Seoul, Korea

Abstract

Dystonia is a disorder of movement caused by involuntary, sustained muscle contractions affecting one or more sites of body, frequently causing twisting and repetitive movements, or postures. Dystonic movements and postures can produce a wide range of clinical presentations. Some distinguishing clinical features of dystonia can help the appropriate diagnosis of primary dystonia. The direction of contraction is almost consistent. Action dystonia and occupational dystonia are related to a movement or task-specific movement, respectively. Sensory tricks or gestes antagonistes are usually seen in patients with dystonia. Two types of tremors can be seen in patients with dystonia: a postural and/or action tremor that resembles essential tremor and a rhythmic expression of dystonic tremor. Sometimes dystonic tremor appears to be less regular and can be associated with myoclonus. Onset age of dystonia, body distribution and etiologies are important to correct diagnosis of primary dystonia. It is well known that the age of onset has important prognostic implications. This article highlights general concepts of phenomenology, classification that are relevant for the purpose a clinical diagnosis. Key Words: Primary dystonia, DYT1 dystonia, Dopa-responsive dystonia, Clinical viewpoints