先天性多发性颅神经病变：婴儿口面部肌电图的相关性

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

作者信息

Renault Francis, Flores-Guevara Roberto, Baudon Jean-Jacques, Vazquez Marie-Paule

机构信息

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.

出版信息

Muscle Nerve. 2015 Nov;52(5):754-8. doi: 10.1002/mus.24636. Epub 2015 Sep 7.

DOI:10.1002/mus.24636

PMID:25736713

Abstract

INTRODUCTION

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).

CONCLUSION

EMG highlights multiple cranial neuropathies that can be clinically silent in infants with malformation syndromes or encephalopathies.

摘要

引言

本研究旨在评估使用口面部肌电图（EMG）识别出患有两种或更多种颅神经病变的婴儿的诊断情况和预后。

方法

这项回顾性研究涉及90名患者。诊断综合考虑了临床、放射学和遗传学数据。EMG检查了眼轮匝肌、颏舌肌和腭帆提肌，并检测了眨眼反应。为评估预后，记录了神经功能障碍、呼吸并发症和喂养困难情况。

结果

患者患有畸形综合征（59例）、脑病（29例）或无潜在疾病（2例）。平均在4块肌肉中检测到神经源性EMG体征，反映平均有3条神经受累。与仅通过临床检查相比，EMG识别出的神经病变数量更多（面部神经病变：82例对31例；咽神经病变：56例对2例；舌下神经病变：25例对3例）。当EMG识别出≥4条受累神经时，不良预后和死亡更为常见（P = 0.02）。