先天性眼球运动不能综合征中颅神经病变的发病机制：电诊断性口面部研究

Pathogenesis of cranial neuropathies in Moebius syndrome: Electrodiagnostic orofacial studies.

作者信息

Renault Francis, Flores-Guevara Roberto, Sergent Bernard, Baudon Jean Jacques, Aouizerate Jessie, Vazquez Marie-Paule, Gitiaux Cyril

机构信息

Centre de référence des malformations rares de la face et de la cavité buccale MAFACE, Hôpital Necker-Enfants-Malades, Paris, France.

Facultad de Medicina, Universidad Nacional Mayor de San Marcos, Lima, Peru.

出版信息

Muscle Nerve. 2018 Feb 9. doi: 10.1002/mus.26095.

DOI:10.1002/mus.26095

PMID:29424937

Abstract

INTRODUCTION

We designed a retrospective study of 59 patients with congenital sporadic nonprogressive bilateral facial and abducens palsies.

METHODS

Examinations included needle electromyography (EMG) of facial and oral muscles, facial nerve motor latency and conduction velocity (FNCV), and blink responses (BR).

RESULTS

Neurogenic EMG changes were found in 1 or more muscles in 55 of 59 patients, with no abnormal spontaneous activity. EMG changes were homogeneously neurogenic in 17 patients, homogeneously myopathic in 1 patient, and heterogeneous in 41 of 59 patients. Motor latency was increased according to recordings from 52 of 137 facial muscles. An increase of motor latency was not associated with neurogenic EMG (Fischer's test: right, P = 1; left, P = 0.76). FNCV was slowed in 19 of 36 patients. BR was absent bilaterally in 35 of 58 patients; when present, R1 and R2 latencies were normal.

DISCUSSION

Our results support the hypothesis of an early developmental defect localized in motor cranial nerves with spared V-VII internuclear pathways. Muscle Nerve, 2018.

摘要

引言

我们对59例先天性散发性双侧面部及展神经麻痹患者进行了一项回顾性研究。

方法

检查包括面部及口腔肌肉的针极肌电图（EMG）、面神经运动潜伏期及传导速度（FNCV）以及瞬目反射（BR）。

结果

59例患者中有55例在1块或更多肌肉中发现神经源性肌电图改变，无异常自发电活动。17例患者的肌电图改变均为神经源性，1例为均一性肌病性改变，59例中有41例为异质性改变。根据137块面部肌肉中的52块记录，运动潜伏期延长。运动潜伏期延长与神经源性肌电图无关（费舍尔检验：右侧，P = 1；左侧，P = 0.76）。36例患者中有19例FNCV减慢。58例患者中有35例双侧BR消失；若存在BR，R1和R2潜伏期正常。