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Pathogenesis of cranial neuropathies in Moebius syndrome: Electrodiagnostic orofacial studies
Author(s) -
Renault Francis,
FloresGuevara Roberto,
Sergent Bernard,
Baudon Jean Jacques,
Aouizerate Jessie,
Vazquez MariePaule,
Gitiaux Cyril
Publication year - 2018
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/mus.26095
Subject(s) - electromyography , medicine , facial muscles , facial nerve , cranial nerves , abducens nerve , facial electromyography , corneal reflex , facial paralysis , anatomy , anesthesia , surgery , physical medicine and rehabilitation , reflex , palsy , pathology , alternative medicine
: We designed a retrospective study of 59 patients with congenital sporadic nonprogressive bilateral facial and abducens palsies. Methods : Examinations included needle electromyography (EMG) of facial and oral muscles, facial nerve motor latency and conduction velocity (FNCV), and blink responses (BR). Results : Neurogenic EMG changes were found in 1 or more muscles in 55 of 59 patients, with no abnormal spontaneous activity. EMG changes were homogeneously neurogenic in 17 patients, homogeneously myopathic in 1 patient, and heterogeneous in 41 of 59 patients. Motor latency was increased according to recordings from 52 of 137 facial muscles. An increase of motor latency was not associated with neurogenic EMG (Fischer's test: right, P = 1; left, P = 0.76). FNCV was slowed in 19 of 36 patients. BR was absent bilaterally in 35 of 58 patients; when present, R1 and R2 latencies were normal. Discussion : Our results support the hypothesis of an early developmental defect localized in motor cranial nerves with spared V–VII internuclear pathways. Muscle Nerve 58 : 79–83, 2018