RT Journal Article
SR Electronic
T1 Hyperekplexia and trismus due to brainstem encephalopathy
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 122
OP 125
DO 10.1136/jnnp.65.1.122
VO 65
IS 1
A1 Mark W Kellett
A1 Peter R D Humphrey
A1 Brian M Tedman
A1 Malcolm J Steiger
YR 1998
UL http://jnnp.bmj.com/content/65/1/122.abstract
AB The brainstem is said to be the generator of pathological startle responses due to reticular reflex myoclonus or hyperekplexia. A patient with facial weakness, nystagmus, and pyramidal tract signs had generalised reflex spasms in response to auditory, visual and tactile stimuli which clinically and neurophysiologically resembled hyperekplexia. The case is unusual because as well as hyperekplexia, the patient’s initial presentation was with an equally rare manifestation of brainstem pathology—brainstem mediated trismus. The causes of brainstem trismus and exaggerated startle responses are discussed with respect to their underlying mechanisms.