Renault F, Flores-Guevara R, Soupre V, Vazquez M P, Baudon J J
Unité de neurophysiologie clinique, Hôpital d'enfants Armand-Trousseau, Paris, France.
Early Hum Dev. 2000 May;58(2):141-52. doi: 10.1016/s0378-3782(00)00073-6.
Polysomnography, electromyography (EMG) of the face, tongue, and soft palate, blink reflexes (BRs), EMG during bottle-feeding, and brainstem auditory evoked responses (BAERs) were performed in 25 newborn babies with isolated Pierre Robin sequence (PRS) to aid in evaluation and management. Obstructive apneas were found in 23/24 patients (the 25th having undergone tracheotomy). Number and duration of central respiratory pauses were always normal, as well as electroencephalographic and clinical organization of sleep stages. EMG recruitment pattern in facial and lingual muscles, and BRs were normal 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-duration and low amplitude motor unit potentials in 10/25, and showed signs of denervation in 1/25. EMG during bottle-feeding showed sucking-swallowing disorders in 20/25 patients. BAERs showed a bilateral conductive impairment with increased latencies and thresholds in 5/19 patients, but with normal and symmetric I-III and I-V interpeak latencies in 19/19. These neurophysiological findings suggest that in isolated PRS a dysfunction of the lingual and pharyngeal motor organization exists without any structural impairment in brainstem nuclei and pathways.
对25例患有孤立性皮埃尔·罗宾序列征（PRS）的新生儿进行了多导睡眠图、面部、舌头和软腭的肌电图（EMG）、眨眼反射（BRs）、奶瓶喂养期间的EMG以及脑干听觉诱发电位（BAERs）检查，以辅助评估和管理。24例患者中有23例发现阻塞性呼吸暂停（第25例已接受气管切开术）。中枢性呼吸暂停的次数和持续时间、睡眠阶段的脑电图和临床结构始终正常。所有病例的面部和舌肌EMG募集模式以及BRs均正常。25例患者中有14例软腭肌肉的EMG募集模式正常，10例表现为平均振幅降低，运动单位电位持续时间短且振幅低，1例表现为失神经征象。奶瓶喂养期间的EMG显示25例患者中有20例存在吸吮-吞咽障碍。BAERs显示19例患者中有5例双侧传导障碍，潜伏期和阈值增加，但19例患者中有19例I-III和I-V峰间期正常且对称。这些神经生理学结果表明，在孤立性PRS中，存在舌和咽运动组织功能障碍，而脑干核团和通路无任何结构损伤。
