连枷臂(腿)综合征的临床、电生理和病理特点

Clinical,electrophysiological and pathological features in patients with flail arm syndrome and flail leg syndrome

目的探讨连枷臂综合征(flailarm syndrome,FA)和连枷腿综合征(flailleg syndrome,FL)患者的临床、电生理和骨骼肌病理改变特点。方法收集2007-01-2011-10期间就诊于海军总医院神经内科的FA和FL患者。所有患者均进行了详细的病史询问、体格检查以及电生理检查。2例患者行肌肉病理检查。结果 5例患者中FA 4例、FL 1例,男4例、女1例,发病年龄为18～65岁,平均35岁,病程分别为2年、2.5年、3年、14年和31年。FA表现为双上肢近端无力伴萎缩;FL表现为下肢远端无力伴跟腱反射消失。疾病后期FA向肢体远端发展,FL向肢体近端发展,其他节段轻微受累。肌电图显示部分受累肌肉出现失神经支配,运动和感觉神经传导速度正常。肌肉病理呈典型神经源性骨骼肌损害的病理特点。结论 FA和FL是肌萎缩侧索硬化(ALS)的一种变异型,病情进展呈良性过程。电生理和病理均符合失神经支配骨骼肌损害的特点。

Objective To review the clinical, electrophysiological and pathological change characteristics of skeletal muscle of flail arm syndrome （FA） and flail leg syndrome （FL）. Methods Four FA cases, 1 FL case who visited department of Neurology in Navy General Hospital from 2007-01 to 2011-10 were involved in the study. The physical examination and medical history of all cases and muscle pathological examination of 2 cases were analyzed retrospectively. Results Five patients （male 4, female 1） included 4 FA and 1 FL, the age of onset was between 18 and 65 years old, and the average onset age was 35 years old. The course are 2 years, 2.5 years, 3 years, 14 years and 31 years respectively. The clinical features of FA patients were double upper limbs proximal weakness and atrophy while FL patients were weakness of distal end of lower limb and the disappear of achilles tendon reflex. In later stages body near and far paragraph involved respectively with other segments slight involvement. Electromyography showed that part of the affected muscles denervated, and the speed of motor and sensory nerve conduction was normal. Muscle pathology revealed typical neurogenic impairments. Conclusions FA and FL are both variant of amyotrophic lateral sclerosis （ALS） with benign cilnical course, and its electrophysiological and pathological change are according with the characteristics of denervated skeletal muscle.