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Keywords:

  • hyperekplexia;
  • brainstem encephalopathy;
  • trismus;
  • autoimmune;
  • reflex myoclonus

Abstract

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