Childhood dystonia.
Childhood dystonias are a heterogeneous group of disorders with strong inherited basis. This review describes the clinical characteristics, classification, genetic basis, pathophysiology, biochemistry, pathology, and treatment of dystonias, including the primary dystonias, the dystonia-plus syndromes, secondary dystonias, and heredodegenerative disorders. Conditions discussed in detail include idiopathic torsion dystonia, dopa-responsive dystonia, Wilson's disease, myoclonus dystonia, rapid-onset dystonia parkinsonism, neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome), mitochondrial dystonias, Niemann-Pick type C, and neuroacanthocytosis.
Uc
Ergun Y
EY
Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, IA 52246, USA.
Rodnitzky
Robert L
RL
D002648:Child; D004421:Dystonia / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D020022:Genetic Predisposition to Disease; D006801:Humans