Picture of Medication - Treating Oromandicular Dystonia with Sensory Tricks and Medical Management

Treating Oromandibular Dystonia: Tricks & Medical Management

Picture of Medication - Treating Oromandicular Dystonia with Sensory Tricks and Medical Management

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

[1] Poisson, A., Krack, P., Thobois, S., Loiraud, C., Serra, G., Vial, E., & Broussolle. (2012). History of the ‘geste antagoniste’ sign in cervical dystonia. Journal of Neurology., 259(8), 1580-1584.

[2] Yoshida, K. (2017). Sensory trick splint as a multimodal therapy for oromandibular dystonia. J Prosthodont Res, J Prosthodont Res, 2017.

[3] Paulig, J., Jabusch, H., Großbach, M., Boullet, L., & Altenmüller, E. (2014). Sensory trick phenomenon improves motor control in pianists with dystonia: Prognostic value of glove-effect. Frontiers in Psychology., 5, 1012.

[4] Picillo, M., & Munhoz, R. (2018). Medical Management of Movement Disorders. Prog Neurol Surg, 33, 41-49.

[5] Pirio Richardson, S., Wegele, A., Skipper, B., Deligtisch, A., & Jinnah, H. (2017). Dystonia treatment: Patterns of medication use in an international cohort. Neurology., 88(6), 543-550.

[6] Lumsden, D., Kaminska, M., Tomlin, S., & Lin, J. (2016). Medication use in childhood dystonia. European Journal of Paediatric Neurology., 20(4), 625-629.

[7] Karabanov, A., & Illarioshkin, S. (2012). [Possibilities of treatment of dystonic syndromes with akineton]. Zh Nevrol Psikhiatr Im S S Korsakova, 112(9), 41-46.

[8] Bergman, H., & Soares-Weiser, K. (2018). Anticholinergic medication for antipsychotic-induced tardive dyskinesia. The Cochrane Database of Systematic Reviews., 1, CD000204.

[9] Jankovic J. Treatment of dystonia. Lancet Neurol 2006;5(10):864-72.

[10] Harvey, A., Reddihough, D., Scheinberg, A., & Williams, K. (2017). Oral medication prescription practices of tertiary-based specialists for dystonia in children with cerebral palsy. Journal of Paediatrics and Child Health, Journal of paediatrics and child health, 2017.

[11] Taira T, Ochiai T, Goto S et al. Fifteen year experience of intrathecal baclofen treatment in Japan. Acta Neurochir Suppl 2006;99:61-3.

[12] Aljuboori, Z., Archer, J., Huff, W., Moreno, A., & Jea, A. (2018). Placement of baclofen pump catheter through a C1-2 puncture: Technical note. Journal of Neurosurgery., 1-6.

[13] Vender JR, Hester S, Waller JL et al. Identification and management of intrathecal baclofen pump complications: a comparison of pediatric and adult patients. J Neurosurg 2006;104(1 Suppl):9-15.

[14] Anand, J., Zając, M., Waldman, W., Wojtyła, A., Biliński, P., & Jaworska-Łuczak, B. (2017). Correlation between the single, high dose of ingested baclofen and clinical symptoms. Annals of Agricultural and Environmental Medicine: AAEM., 24(4), 566-569.

[15] Wakakura M, Tsubouchi T, Inouye J. Etizolam and benzodiazepine induced blepharospasm. J Neurol Neurosurg Psychiatry 2004;75(3):506-7.

[16] Jankovic, J. (2013). Medical treatment of dystonia. Movement Disorders, 28(7), 1001-1012.

[17] Albanese A, Barnes MP, Bhatia KP, Fernandez-Alvarez E, Filippini G, Gasser T, Krauss JK, Newton A, Rektor I, Savoiardo M, Valls-Solè J. A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS/MDS-ES Task Force. Eur. J. Neurol. 2006 May;13(5):433-44.

[18] Fan, X., Donsante, Y., Jinnah, H., & Hess, E. (2018). Dopamine receptor agonist treatment for idiopathic dystonia: A reappraisal in humans and mice. The Journal of Pharmacology and Experimental Therapeutics., The Journal of pharmacology and experimental therapeutics. 2018.

[19] Wasif, Nawal, Wasif, Komal, & Saif, Muhammad W. (2017). Bupropion-Induced Acute Dystonia with Dose Escalation and Use of Naranjo Nomogram. Curēus., 9(4), E1157.

[20] Masiran, R. (2017). Persistent oromandibular dystonia and angioedema secondary to haloperidol. BMJ Case Reports, 2017, BMJ case reports , 2017, Vol. 2017.

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About Dr. Glenn Clark

Glenn Clark, DDS, MS is an expert on sleep apnea, orofacial pain and oral medicine, and Temporomandibular Joint Disorder (TMJ). Dr. Clark serves as the Director for the Advanced Program in Orofacial Pain and Oral Medicine at the Herman Ostrow School of Dentistry of USC.