Childhood Dystonias

Dystonia is a movement disorder caused by diverse etiologies. Its treatment in children is particularly challenging due to the complexity of the development of the nervous system from birth to young adulthood. The treatment options of childhood dystonia include several oral pharmaceutical agents, botulinum toxin injections, and deep brain stimulation (DBS) therapy. The choice of drug therapy relies on the suspected etiology of the dystonia and the adverse effect profile of the drugs. Dystonic syndromes with known etiologies may require specific interventions, but most dystonias are treated by trying serially a handful of medications starting with those with the best risk/benefit profile. In conjunction to drug therapy, botulinum toxin injections may be used to target a problematic group dystonic muscles. The maximal botulinum toxin dose is limited by the weight of the child, therefore limiting the number of the muscles amenable to such treatment. When drugs and botulinum toxin injections fail to control the child's disabling dystonia, DBS therapy may be offered as a last remedy. Delivering optimal DBS therapy to children with dystonia requires a multidisciplinary team of experienced pediatric neurosurgeons, neurologists, and nurses to select adequate candidates, perform this delicate stereotactic procedure, and optimize DBS delivery. Even in the best hands, the response of childhood dystonia to DBS therapy varies greatly. Future therapy of childhood dystonia will parallel the advancement of knowledge of the pathophysiology of dystonic syndromes and the development of clinical and research tools for their study.