Objective: To describe a case of oculopalatal tremor with hemifacial involvement due to hypertrophic inferior olivary degeneration after pontine hemorrhage.

Background: Symptomatic palatal tremor develops months after structural lesions that disrupt gabanergic connections from the dentate nucleus to the contralateral inferior olivary nucleus.  Maladaptive linkages to the cerebellar cortex cause pendular ocular oscillations characteristic of oculopalatal tremor. Oculopalatal tremor can occur with other brainstem pathology such as eight-and-a-half syndrome, but the mechanism by which synchronous hemifacial clonic movements would occur is unknown.

Method: Case report.

Results: A 38-year-old Asian woman with Fahr disease, prior diagnosis of hemifacial spasm and hypertensive right pontine intracranial hemorrhage one year prior complicated by quadriplegia and persistent respiratory failure requiring tracheostomy was admitted to the intensive care unit for urosepsis. Neurology was consulted for reevaluation of left “hemifacial spasm” present for several months. Family reported the patient was intermittently able to communicate using head signals, and could move her limbs spontaneously and occasionally to command. Examination revealed a comatose, ventilator-dependent woman with diffuse spasticity and semirhythmic clonic movements involving the left lower facial muscles which were synchronous with torsional left ocular and vertical right ocular myoclonus and palatal myoclonus. No factors that improved or exacerbated the facial twitching were identified, including antiepileptic medications, and prolonged video electroencephalogram was negative for electrographic seizures. Neuroimaging was notable for brainstem atrophy worsened since last imaging, and T2/FLAIR hyperintensity and enlargement of the inferior olivary nucleus bilaterally, more prominently on the right. No other new neuropathology was identified.

Conclusion: We describe a patient who developed involuntary facial movements synchronous with symptomatic oculopalatal tremor in association with hypertrophic olivary degeneration after right pontine hemorrhage. Palatal tremor with facial involvement has been described in two prior case reports and is not commonly recognized, as evidenced by this patient previously diagnosed with hemifacial spasm.[1] This case report was submitted to the American Academy of Neurology 2019 annual meeting.

References: [1] Bolen RD, Balakrishnan MDN. Palatal myoclonus, eight-and-a-half syndrome, and Holmes tremor in a patient from a single brainstem lesion. J Neurol Sci 2014; 347(1-2):411-2.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/hemifacial-involvement-in-a-patient-with-oculopalatal-tremor-due-to-inferior-olivary-hypertrophy/