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Congenital multiple cranial neuropathies: Relevance of orofacial electromyography in infants
Author(s) -
Renault Francis,
FloresGuevara Roberto,
Baudon JeanJacques,
Vazquez MariePaule
Publication year - 2015
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.24636
Subject(s) - medicine , electromyography , cranial nerves , corneal reflex , facial muscles , surgery , anesthesia , physical medicine and rehabilitation , anatomy , reflex
: The aim of this study was to assess diagnoses and outcomes of infants with 2 or more cranial neuropathies identified using orofacial electromyography (EMG). Methods : This retrospective study involved 90 patients. Diagnoses took into account clinical, radiological, and genetic data. EMG examined the orbicularis oculi, genioglossus, and levator veli palatini muscles, and blink responses. To evaluate outcome, neurological disability, respiratory complications, and feeding difficulties were recorded. Results : The patients had malformation syndromes (59), encephalopathies (29), or no underlying disorders (2). Neurogenic EMG signs were detected in a mean of 4 muscles, reflecting a mean of 3 affected nerves. EMG identified a higher number of neuropathies than clinical examination alone (82 vs. 31, facial; 56 vs. 2, pharyngeal; 25 vs. 3, hypoglossal). Poor outcome and death were more frequent when EMG identified ≥4 affected nerves ( P = 0.02). Conclusion : EMG highlights multiple cranial neuropathies that can be clinically silent in infants with malformation syndromes or encephalopathies. Muscle Nerve 52 : 754–758, 2015