title: Facial, lingual, and pharyngeal electromyography in infants with Pierre Robin sequence.
abstract: BACKGROUND
  We evaluated the role of electromyography (EMG) in assessing orofacial neurological dysfunction in 81 infants with Pierre Robin sequence (PRS).
  
  
  METHODS
  Needle EMG of muscles of the face, tongue, and soft palate, and blink responses were recorded. A two-channel EMG recorded sucking and swallowing during bottle feeding.
  
  
  RESULTS
  Neurogenic EMG signs were detected in facial or oral muscles in 17 of 24 associated PRS and 1 of 57 isolated PRS cases (P < 0.0001). Soft palate muscles showed low-amplitude traces in 41.4% of patients who required two surgical steps for cleft palate repair and 18.5% of those who required only one step. Regarding EMG study during bottle feeding, patients with moderate or severe abnormalities of oral/pharyngeal coordination required more prolonged enteral feeding than patients with mild abnormalities or normal coordination (P = 0.002).
  
  
  CONCLUSIONS
  Combined EMG methods were useful in the treatment of infants with PRS. EMG detection of cranial nerve involvement strongly suggests an associated form of PRS.
authors.affiliation: Clinical Neurophysiology Unit, Hôpital Armand-Trousseau, AP-HP, 28 avenue Arnold-Netter, 75571 Paris 12, France. francis.renault@trs.aphp.fr
authors.forename: Francis
authors.initials: F
authors.lastname: Renault
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Baudon
authors.forename: Eva
authors.initials: E
authors.lastname: Galliani
authors.forename: Roberto
authors.initials: R
authors.lastname: Flores-Guevara
authors.forename: Sandrine
authors.initials: S
authors.lastname: Marlin
authors.forename: Erea Noel
authors.initials: EN
authors.lastname: Garabedian
authors.forename: Marie-Paule
authors.initials: MP
authors.lastname: Vazquez
mesh_terms: D004576:Electromyography / Q000379:methods*; D005152:Facial Muscles / Q000294:innervation / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008410:Masticatory Muscles / Q000294:innervation / Q000503:physiopathology; D010609:Pharyngeal Muscles / Q000294:innervation / Q000503:physiopathology*; D010855:Pierre Robin Syndrome / Q000175:diagnosis / Q000503:physiopathology*; D014059:Tongue / Q000294:innervation / Q000503:physiopathology*