南方医科大学学报Issue(11)：1688-1692,5.DOI:10.3969/j.issn.1673-4254.2014.11.28
肯尼迪病与肌萎缩侧索硬化的临床及电生理特点比较
Comparison of clinical and physiological characteristics between Kennedy disease and amy-otrophic lateral sclerosis
摘要
Abstract
Objective To study the clinical presentations of Kennedy disease (KD) and compare the neurophysiological features between KD and amyotrophic lateral sclerosis(ALS). Methods Nine patients with KD, 13 patients with ALS and 26 normal control subjects were recruited. The clinical presentations of KD were analyzed, and the results of nerve conduction studies and electromyography were compared among the 3 groups. Results The rates of tongue atrophy and facial fasciculation were 100% and 88.9%, respectively, in the early course and mid-course of KD, sensory damages might be perceived. 2)The sural nerve sensory nerve action potential (SNAP) was not elicited in 56.3% of the patients with KD, and sural nerve SNAP amplitudes were significantly lower in KD (7.9±3.4μV) than in ALS patients (20.0±5.2μV) and normal control subjects (26.1± 16.8 μV) (P<0.05). Conclusion The onset of clinical presentations mimicking motor neuron disease, appearance of tongue atrophy and facial fasciculation in the early and mid-course, and presence of sensory impairment with a decreased sural nerve SNAP amplitude may suggest the diagnosis of KD and should prompt a genetic test for KD.关键词
Kennedy disease/amyotrophic lateral sclerosis/compound muscle action potential/sensory nerve action potentialKey words
Kennedy disease/amyotrophic lateral sclerosis/compound muscle action potential/sensory nerve action potential引用本文复制引用
杨嘉玲,王群,林丽珍,王冬梅,郑卉,管玉青..肯尼迪病与肌萎缩侧索硬化的临床及电生理特点比较[J].南方医科大学学报,2014,(11):1688-1692,5.基金项目
南方医科大学南方医院院长基金(2011B003) （2011B003）
