Dystonias

The natural history of dystonias varies depending on the cause.

• For idiopathic focal dystonias, the disease may be insidious in onset and worsen in severity over a few months to a year, and then typically remains stable.

• Some forms of dystonia, such as dystonia associated with TOR1A (also known as DYT1) mutations, may start focally but then usually spread and become generalized; TOR1A-associated dystonia is variable in severity.

• Patients with dopa-responsive dystonia respond dramatically to dopaminergic agents such as levodopa or dopamine agonists, and also respond to anticholinergic agents. The response persists indefinitely, and (unlike patients with Parkinson disease), these patients do not develop dyskinesias following dopaminergic treatment.