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.
van de Warrenburg
Bart P C
BP
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom.
Cordivari
Carla
C
Brown
Peter
P
Bhatia
Kailash P
KP
D000368:Aged; D001927:Brain Diseases / Q000473:pathology* / Q000503:physiopathology*; D001933:Brain Stem / Q000503:physiopathology*; D004569:Electroencephalography; D004576:Electromyography; D005260:Female; D006801:Humans; D009122:Muscle Hypertonia / Q000503:physiopathology*; D012021:Reflex, Abnormal / Q000502:physiology*; D013216:Reflex, Startle / Q000502:physiology