Management of oromandibular dystonia can be broadly divided into four domains: 1.) sensory tricks, 2.) medical management, 3.) chemodenervation using botulinum neurotoxin (BoNT), and 4.) surgical management. In this article, we explain the first two domains.
Sensory Tricks
Almost pathognomonic for dystonia in the orofacial region is that many patients can partially control or suppress the movement with the use of tactile stimulation, such as touching the chin in the case of orofacial dystonia or holding an object in their mouth.
This suppressive effect has been called geste antagonistique. [1] These tactile maneuvers may mislead physicians to the erroneous diagnosis of malingering or hysteria.
Other examples of sensory tricks include placing a hand on the side of the face, the chin, or the back of the head or touching these areas with one or more fingers which at times will reduce neck contractions associated with cervical dystonia.
The use of distractors, such as wearing a latex glove to improve hand movements, and occlusal splints for oral dystonias seems a feasible approach when other therapies have been unsatisfactory. [2] [3]
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Medical Management
Medical management of oral movement disorders involves the use of various centrally-acting medications ranging from anticholinergics to dopaminergics. This is considered to be the least invasive approach in the management of oromandibular dystonia and involves administration of a medication or withdrawal of an offending agent, providing significantly improved patient’s quality of life and level of disability. [4] [5]
1. Anticholinergic Medications
The first and most common class of mediation used for dystonias are anticholinergic medications. Trihexyphenidyl has the strongest evidence for children with cerebral palsy, especially when the therapy is initiated early. [6]
Biperiden has an additional peripheral choline and ganglion blocking effect, and has been used to treat cervical dystonia. [7]
Both drugs are approved by the FDA for the management of extrapyramidal reactions in Parkinson’s disease. They are also used as off-label medications for managing orofacial dystonias and are only effective in some patients. Anticholinergic drugs have not shown effectivity for patients with antipsychotic-induce movement disorders. [8]
When an anticholinergic medication is prescribed, it has to be started as a very low dose and gradually increased to achieve the desired pharmacologic effect. This slow titration increases patient tolerability and decreases the incidence of side effects such as dry mouth, blurred vision, urinary retention, and confusion. [9]
2. GABA-ergic Medications
The second class of medications used for suppression of dystonia are the GABA-ergic medications such as Baclofen (Lioresal).
Baclofen is used either orally or intrathecally for patients with dystonia and other spastic motor disorders. [10] Intrathecal administration of Baclofen is far more effective than oral administration and involves the use of an implantable infusion pump. [11] [12]
The intrathecal catheters are placed under fluoroscopic guidance in the mid-cervical region for dystonia. The catheter, pump, and surgical wound are subject to numerous complications both at the time of implantation and throughout the life of the implanted system. These complications can be reduced with careful surgical technique and postoperative follow-up. [13]
Baclofen, when administered orally, is started at a low dose of 10 mg at bedtime and increased to a maximum dose of 30 mg three to four times a day. The main side effects include drowsiness, confusion, dizziness, and weakness. An overdose of Baclofen can lead to life-threatening clinical symptoms, including acute respiratory failure requiring mechanical ventilation. [14]
3. Benzodiazepines
The third class of medication, which also affect the GABAergic system, are the benzodiazepines. These drugs include clonazepam, diazepam, and lorazepam and are often used as adjuncts for patients who fail to respond satisfactorily to anticholinergics.
Clonazepam (Klonopin) is the most popular benzodiazepine and is titrated from a dose of 0.25 mg once daily at bedtime to a maximum of 1 mg four times daily. The side effects include drowsiness, confusion, trouble concentrating and dizziness. Paradoxically, benzodiazepines and thiobenzodiazepine derivatives, upon prolonged administration, have been reported to cause blepharospasm. [15]
When a patient exhibits a defect in dopamine synthesis, a logical approach is the use of medications with action in dopamine receptors, neurotransmitter depleting, and dopaminergic drugs.
Dopamine has a dopamine receptor blocking effect, and even though it has shown effectivity for segmental, generalized and tardive dystonia, the side effects limit the potential for this medication (mostly sedation and agranulocytosis). [16]
The use of levo/carbidopa is helpful with Parkinson disease but have very little therapeutic effect for dystonia except for the dopa-responsive dystonia (DRD) that occurs in children. DRD constitutes approximately 5% of childhood dystonias and is often misdiagnosed as cerebral palsy.
The absolute and comparative efficacy and tolerability of drugs in dystonia, including anticholinergic and antidopaminergic drugs, is poorly documented and no evidence-based recommendations can be made to guide prescribing medications for these patients. [17] [18]
When the dystonia is produced by a medication, the reduction on the dose or even the discontinuation of the drug might be the therapeutic approach. Drug-induced dystonia is commonly associated with antipsychotic drugs, antidepressants, antiemetics, and other medications. [19] [20]
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References
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