Oculofaciopalatal and Lingual Myoclonus with Two Separate Generators Following Brainstem Stroke (P07.146)

Abstract

Objective: To describe a unique neurophysiological study in a patient with oculofaciopalatal and lingual myoclonus following brainstem stroke. Background Oculofaciopalatal myoclonus is a syndrome characterized by rhythmic pendular vertical eye movements with synchronous contraction of the soft palate. It is often seen in the context of brainstem infarction or hemorrhage causing damage to the triangle of Guillian-Mollaret, and may causes oscillopsia. We report a case of oculopalatofacial myoclonus secondary to a brainstem stroke, who was noted to also have asynchronous and independent lingual myoclonus. Design/Methods: Case report. Results: A 52 year old man suffered brainstem infarction. On examination, he had a one and a half syndrome and left sided lower motor nerve facial palsy, as well as quadriplegia,and sensory loss in the right hemibody and left face. Rhythmic, synchronous upward contraction of the eyes, palate and face were noted, as well independent rhythmic tongue contractions independent and asynchronous to the oculopalatofacial myoclonus. Polymography of multiple cranial and limb muscles was performed to characterize these movements. Synchronous, rhythmic myoclonic bursts were seen at a frequency of 1.5-2 Hz in the orbicularis oculi, mentalis, palatal muscles and sternocleidomastoid. However, myoclonic bursts in the lingual muscles were noted to occur asynchronously with these other muscles, at the same frequency but with variable latency. Conclusions: Lingual myoclonus has been described in the literature to occur in an idiopathic fashion, and in isolated case reports, with Arnold-Chiari malformations, brainstem tumors and as epileptic phenomena. We believe that in our patient with oculopalatofacial and independent, asynchronous lingual myoclonus, the neurophysiological evaluation demonstrates the presence of a second generator causing rhythmic tongue movements, completely independent and asynchronous from the classic generator of oculofaciopalatal myoclonus. This is a novel description in our patient. Disclosure: Dr. Lysenko has nothing to disclose. Dr. Bhat has nothing to disclose. Dr. Rosenberg has nothing to disclose. Dr. Chokroverty has received personal compensation for activities with Elsevier and Cephalon. Dr. Chokroverty has received personal compensation in an editorial capacity for Sleep Medicine Journal.