Fast Five Quiz: Movement Disorders

Helmi L. Lutsep, MD

Disclosures

July 08, 2021

The options to medically manage dystonic movements have traditionally been fourfold:

  • Rehabilitative therapies

  • Oral medications

  • Neurochemolytic interventions

  • Surgery

Botulinum toxin injections temporarily reduce the ability of the muscles to contract and are often the treatment of choice for blepharospasm, cervical dystonia, and hemifacial spasm. Botulinum toxins are produced by the gram-positive bacterium Clostridium botulinum and act by inhibiting the presynaptic release of acetylcholine at the neuromuscular junction. The onset of effect takes several days after injection. The local injection of botulinum toxins into the offending muscles (often the sternocleidomastoid, trapezius, and splenius capitis) reduces muscle contraction for approximately 3 months. For management of childhood-onset primary generalized or segmental dystonia, trihexyphenidyl is considered a first-line medication.

Although patients with dystonia were often treated with pallidotomy or thalamotomy in the past, those procedures frequently caused significant, often debilitating adverse effects, including dysarthria. Deep brain stimulation (DBS) has largely replaced lesional surgeries. DBS uses surgically implanted wires placed either unilaterally or bilaterally into target areas such as the thalamus, subthalamic nucleus, or globus pallidus. An implanted neurostimulator then delivers electrical stimulation through these wires to the brain. The best results have been obtained with pallidal stimulation in patients with primary dystonias, such as generalized DYT1 dystonia.

Systemic medications for patients with dystonia include:

  • Cholinergics

  • Benzodiazepams

  • Antiparkinsonism drugs

  • Anticonvulsants

  • Baclofen

  • Carbamazepine

  • Lithium

Successful drug therapy often requires combinations of several medications, with choices generally guided by empirical trials and adverse-effect profiles. Doses should be slowly increased over the course of weeks or months until the therapeutic benefit is optimized or until adverse effects occur. In most patients, discontinuation of the drugs requires tapering to prevent withdrawal symptoms.

Read more on the treatment of dystonias.

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