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Persisting hyperekplexia after idiopathic, self‐limiting brainstem encephalopathy

Symptomatic hyperekplexia is a relatively rare entity, but has been documented in various, mostly brainstem diseases. We report the clinical and neurophysiologic vignette of a patient with a self‐limiting, possibly inflammatory brainstem encephalopathy. Trismus was the presenting feature, but she later developed hyperekplexia. Although most brainstem features resolved spontaneously, the hyperekplexia has persisted. © 2007 Movement Disorder Society