Objective: We hereby report a case of oromandibular dystonia with onset in late 70s and symptoms resistant to symptomatic therapy including onabotulinum toxin A.

Background: Oromandibular dystonia is a focal dystonia characterized by abnormal involuntary contractions of muscles of tongue, face, and mastication, thereby resulting in abnormal jaw opening, jaw closing, dysrthria or dysphagia. Its usual age of onset is in mid-50s and management includes dopaminergic depleters and onabotulinum toxin A.

Method: An 80 year old gentleman, with 2 year history of difficulty in swallowing and abnormal jaw opening dystonic movements, with preserved cognition, was evaluated for his symptoms. He was diagnosed as having Idiopathic Oromandibular Dystonia with dysphagia, on the basis of clinical presentation and EMG findings.

Results: Patient was initially treated with tetrabenazine, and trihexyphenydyl, but did not show any improvement in his symptoms. He was then managed with onabotulinum toxin A, with a total dose of 25 units. Due to non- responsiveness, he was treated with EMG guided 50 units of onabotulinum toxin A, to which he responded partially.

Conclusion: Due to rarity of this condition, rational management options are limited to a few case reports or small review articles. Our case highlights the unmet need for further research in the management of oromandibular dystonia, including dosing of onabotulinum toxin A and option of deep brain stimulation.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/late-onset-treatment-resistant-isolated-oromandibular-dystonia-a-case-report/