连枷臂综合征临床特点分析

Clinical features of flail arm syndrome

摘要:

连枷臂综合征是肌萎缩侧索硬化的一种良性的临床变异型,一般男性多见,起病缓慢,生存期较长,主要临床特征为对称性双上肢近端显著的肌无力、肌萎缩,而双下肢、球部功能受累较轻。神经电生理检查表现为3个以上脊髓节段的广泛神经源性损害。目前缺乏针对连枷臂综合征的药物研究,利鲁唑是唯一批准用于肌萎缩侧索硬化的药物。

Abstract:

Flail arm syndrome (FAS) is a benign clinical variant of amyotrophic lateral sclerosis (ALS). It occurs predominantly in males and has a slow onset and a long survival. The major clinical features of FAS are significant muscle weakness and amyotrophia in the proximal segments of both upper limbs and mild involvement of both lower limbs and bulbar muscles. Nerve electrophysiological examination shows extensive neurogenic damage in more than three segments of the spinal cord. At present, there are no drugs for FAS, and riluzole is the only drug approved for the treatment of ALS.