Neurophysiological brainstem investigations in isolated Pierre Robin sequence

Polysomnography, electromyography (EMG) of the face, tongue, and soft palat e, blink reflexes (BRs), EMG during bottle-feeding, and brainstem auditory evoked responses (BAERs) were pet-formed in 25 newborn babies with isolated Pierre Robin sequence (PRS) to aid in evaluation and management. Obstructi ve apneas were found in 23/24 patients (the 25th having undergone tracheoto my). Number and duration of central respiratory pauses were always normal, as well as electroencephalographic and clinical organization of sleep stage s. EMG recruitment pattern in facial and lingual muscles, and BRs were norm al in all cases. EMG recruitment pattern in muscles of the soft palate was normal in 14/25 patients, showed a reduced average amplitude with short-dur ation and low amplitude motor unit potentials in 10/25, and showed signs of denervation in 1/25. EMG during bottle-feeding showed sucking-swallowing d isorders in 20/25 patients. BAERs showed a bilateral conductive impairment with increased latencies and thresholds in 5/19 patients, but with normal a nd symmetric I-III and I-V interpeak latencies in 19/19. These neurophysiol ogical findings suggest that in isolated PRS a dysfunction of the lingual a nd pharyngeal motor organization exists without any structural impairment i n brainstem nuclei and pathways. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.