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Mobius syndrome: Electrophysiologic studies in seven cases
Author(s) -
Jaradeh Safwan,
D'Cruz O'Neill,
Howard James F.,
Haberkamp Thomas J.,
Konkol Richard J.
Publication year - 1996
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199609)19:9<1148::aid-mus11>3.0.co;2-r
Subject(s) - facial muscles , corneal reflex , jaw jerk reflex , psychology , medicine , electromyography , physical medicine and rehabilitation , audiology , reflex , neuroscience
Mobius syndrome is characterized by congenital facial diplegia, frequent impairment of gaze, variable involvement of other cranial muscles, and various musculoskeletal anomalies. The site of dysfunction remains debatable. We performed detailed electrophysiologic studies in 5 children and 2 adults with Mobius syndrome to better delineate the pathophysiology of this disorder. Sensory and motor conduction studies were normal in the extremities. Facial compound muscle action potential amplitudes were reduced in all patients. The blink reflex R 1 responses were unobtainable unilaterally in 2 patients and unobtainable bilaterally in 3 patients. Otherwise, R 1 and R 2 latencies were variably prolonged. The jaw jerk and masseter silent periods, tested in 2 patients, were normal. Detailed electromyographic studies of facial muscles revealed multifocal, chronic neurogenic changes. The findings indicate a brain stem process predominantly affecting the facial nuclei and their internuclear connections, rather than a supranuclear or muscular site of involvement. © 1996 Wiley & Sons, Inc.