Treatment of generalized or segmental dystonias
What are the most effective medications for the treatment of generalized or segmental dystonias?
Levodopa, effective in about 10% of children with dystonia, should be tried in all childhood-onset dystonias.
If there is no significant improvement in 2 months, levodopa is replaced by anticholinergics. The initial dose of trihexyphenidyl (Artane) is 2 mg twice daily. High doses, sometimes up to 100 mg/day, may be necessary.
The benefits may not be appreciated for 3 to 4 months after initiation of therapy. Moderate to dramatic improvement is observed in up to 70% of patients, but efficacy may decrease with chronic use.
The usefulness of these medications, especially in adults, is limited by the occurrence of peripheral (dry mouth and blurred vision) and central (forgetfulness, confusion, hallucinations) side effects.
Other drugs that can be tried are baclofen, benzodiazepines, and tetrabenazine.
Levodopa (in children) and anticholinergics (in adults) are the first options among the systemic drugs.
Clonazepam is occasionally highly effective in blepharospasm, whereas baclofen may be particularly useful in cranial dystonia. Systemic treatment of focal dystonias is disappointing, however.
If oral medications are ineffective, local injections of botulinum toxin should be considered in patients with focal dystonia.
Injections of botulinum toxin into the affected musculature are now considered the first choice of treatment.
- Patel N, Hanfelt J, Marsh L, Jankovic J: Alleviating manoeuvres (sensory tricks) in cervical dystonia. J Neurol Neurosurg Psychiatry 85(8):882-884, 2014.
- Thenganatt MA, Jankovic J: Treatment of dystonia. Neurotherapeutics 11(1):139–152, 2014.
- Jankovic J: Medical treatment of dystonia. Mov Disord 28(7):1001–1012, 2013.
- Alterman RL, Miravite J, Weisz D, Shils JL, Bressman SB, Tagliati M: Sixty hertz pallidal deep brain stimulation for primary torsion dystonia. Neurology 69(7):681-688, 2007.
- Sobstyl M, Kmiec T, Zabek M, Szczaluba K, Mossakowski Z: Long-term outcomes of bilateral pallidal stimulation for primary generalised dystonia. Clin Neurol Neurosurg 126:82-87, 2014.
- Volkmann J, Wolters A, Kupsch A, Müller J, Kühn AA, Schneider GH, et al.: Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 11(12):1029-1038, 2012.
- Panov F, Gologorsky Y, Connors G, Tagliati M, Miravite J, Alterman RL: Deep brain stimulation in DYT1 dystonia: a 10-year experience. Neurosurgery 73(1):86-93, 2013.