Congenital multiple cranial neuropathies: Relevance of orofacial electromyography in infants.
BACKGROUND
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).


CONCLUSIONS
EMG highlights multiple cranial neuropathies that can be clinically silent in infants with malformation syndromes or encephalopathies.
Clinical Neurophysiology Unit, Hôpital Armand-Trousseau, Assistance Publique-Hôpitaux de Paris, 28 avenue Arnold-Netter, 75571 Paris 12, France.
Francis
F
Renault
Clinical Neurophysiology Unit, Hôpital Armand-Trousseau, Assistance Publique-Hôpitaux de Paris, 28 avenue Arnold-Netter, 75571 Paris 12, France.|Facultad de Medicina, Universidad Nacional Mayor de San Marcos, Lima, Peru.
Roberto
R
Flores-Guevara
Faculté de Médecine René-Descartes, Université Paris 5, Paris, France.
Jean-Jacques
JJ
Baudon
Faculté de Médecine René-Descartes, Université Paris 5, Paris, France.|Department of Maxillofacial Surgery, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France.
Marie-Paule
MP
Vazquez
D002675:Child, Preschool; D003389:Cranial Nerve Diseases / Q000175:diagnosis* / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods*; D005152:Facial Muscles / Q000002:abnormalities* / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies
blink responses; brainstem; child; cranial nerves; electromyography