[The study and treatment of dystonias in childhood].
OBJECTIVE
Dystonia is after spasticity and tics the most common movement disorder in childhood. The etiology, diagnostic methods and treatment of childhood dystonia is reviewed.


METHODS
Children have generalized dystonia more often than adults. The most frequent secondary dystonia are due to cerebral palsy, and often it is associated with other movement disorders like spasticity. The study of dystonia must consider the diverse heredo-degenerative causes because the genetic counseling, natural evolution and treatment may be specific. Among the primary dystonias, torsion dystonia DYT1 and dopa responsive dystonias (DRD) are the more frequent. DRD can be produced by mutation of GCH1 (DYT5) or other enzymatic defects of the neurotransmitter formation pathway. The clinical features of DYT1, DYT5, DYT11, DYT12 and other primary dystonias presenting in childhood are described. The treatment of dystonias depends on the anatomic distribution. Focal dystonias are best treated with botulinum toxin type A. Most of generalized dystonias are improved by oral drugs (trihexiphenidil, baclofen, clonazepam, tizanidine, tetrabenazine, neuroleptics) in monotherapy or in associations. Intratecal baclofen and deep brain stimulation are the most useful treatments of severe childhood dystonias.


CONCLUSIONS
The study of dystonias in children is complex, there are many disorders to consider as possible causes. Pharmacological and surgical treatments can be time-consuming and expensive but give a significant improvement of symptoms and quality of life to the patients.
Pascual-Pascual
S I
SI
Hospital Universitario La Paz, 28046 Madrid, Espana. ipascualp.hulp@salud.madrid.org
D002648:Child; D004421:Dystonia / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans