Despite having had normal speech until age 21 when he contracted varicella encephalitis, this 53-year-old man had always considered himself a stutterer. Neurologic examination revealed lightning-like facial and neck contractions during speech, which were absent at rest (video). Brain and cervical spine MRI had normal results. Surface EMG confirmed intermittent high-amplitude, short-duration bursts suggestive of cortical myoclonus (figure and video).
Figure Surface EMG during speech and during grimacing
Surface EMG of the sternocleidomastoid and pharyngeal muscles during speech revealed short-duration, high-amplitude bursts (<100 msec) suggestive of cortical myoclonus (A). This is in contrast to the relatively prolonged bursts (>250 msec) of voluntary muscle contraction, as demonstrated when the patient voluntarily grimaced as quickly as possible (B).
Speech-activated myoclonus is an unusual variant of segmental action myoclonus.1,2 The etiology in this patient was likely postinfectious, and treatment with valproic acid led to a moderate symptomatic improvement. The myoclonus occurred with speech in both English and his native Ukrainian.
Footnotes
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Supplemental data at www.neurology.org.
Disclosure: Dr. Graham Alec Glass has received personal compensation for speaking and consulting on behalf of Allergan, Inc. UCSF Medical Center holds both an unrestricted educational grant and a research grant from Allergan, Inc.
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