Childhood Dystonia

Abstract

We studied 54 children with dystonia. Dystonia may be either a symptom of an underlyingdisorder or a spesific disease entity. Dystonia is classified according to etiology: 1) Primary (idiopathic) dystonia, 2) Secondary dystonia. In our series, there were 12 patients in the idiopathic group (3 patients with primary dystonia, 4 patients with dopa responsive dystonia, 5 patients with paroxysmal dystonia) and 42 patients in the secondary group (9 patients with Wilson's disease,13 patients with dystonic CP, 3 patients with meningoencephalitis, 3 patients with metachromatic leukodystrophy, 2 patients with Leigh disease, 3 patients with unknown etiology, 3 patients with focallesions on MRG, I patient with acquired demyelinating encephalomyelitis, 2 patients with fenitoin intoxication, 3 patients with neuroleptic usage) Although therapy for most people with dystonia is symptomatic, for a small minority of patients with dopa-responsive dystonia (DRD) or Wilson's disease spesific therapy is available.