Objective: To describe a case of proven Hashimoto Encephalopathy presenting with oromandibular dystonia (OMD) and orofacial dyskinesias (OFD)

Background: Neurologic manifestations of Hashimoto’s Encephalopathy (HE) have been increasingly recognized to be diverse, both with respect to clinical presentation and therapeutic response, since the first description. ¹ With respect to movement disorders, myoclonus is the most common, followed by dystonia, ataxia, tremor, chorea, tics usually described in association with encephalopathy.²

Method: A 33 year-old lady presented with abnormal involuntary movements in the perioral region characterized by intermittent spontaneous mouth opening and pouting resulting in lateral tongue bites suggestive of oro-mandibular dystonia and dyskinesias (OMD) (Video 1)* with emotional lability, memory impairment and insomnia for six months. She had been tried with oral medications and botulinum toxin with no benefit. Additionally, her higher function examination demonstrated a MMSE of 28/30 (point lost in recall). Medical history was unremarkable except for well controlled hypothyroidism on 50 microgram of thyroxine supplementation daily. She denied any past or concurrent drug intake or family history of similar illness.

Results: Complete blood count, liver function, renal and thyroid function tests, syphilis serology, serum autoimmune and paraneoplastic profiles were negative. Her anti-TPO antibodies returned at 2230 IU/L (Reference range: 100 IU/L) with normal thyroid functions tests. EEG, MRI brain and CSF were normal. She was treated with intravenous methylprednisolone 500 mg for five days followed by oral prednisolone of 50 mg with complete response at three months of follow-up. The anti TPO titres at six months of follow-up were 12 IU/L. Orolingual dyskinesia is typified by focal partially rhythmic jerky and dystonic movements of the mouth and tongue have been reported in a case of thyrotoxic HE earlier, ² however, this case was unique in the patient being biochemically euthyroid, necessitating steroid therapy alone.

Conclusion: To the best of our knowledge, OMD with OFD have not been described as the initial presentation in a case of euthyroid HE.

References: 1. Singh AS, Sidhu AS. Orthostatic Myoclonic Jerks in a Case of Hashimoto’s Encephalopathy. J Neurosci Rural Pract 2022;13:561-562.
2. Keshavaraj A, Anpalagan J. Reversible Orolingual Dyskinesia: An Unknown Presentation of Hashimoto’s Toxic Encephalopathy. Journal of Case Reports. 2018;8:169-171.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/oromandibular-dystonia-in-hashimoto-encephalopathy-an-unusual-association-not-to-miss/