广泛神经源性损害患者的临床分析

The clinical analysis of patients with generalized distribution of neurogenic involvement

目的进一步研究肌萎缩性侧索硬化(ALS)的诊断及预后。方法2008年2月～2010年1月间因缓慢起病、逐渐进展的肌肉无力、萎缩、痉挛、束颤就诊,且针肌电图呈广泛神经源性损害者均纳入登记建档;记录首发起病部位、修订肌萎缩侧索硬化功能评分(ALSFRS-R)以及病程,并分析临床特点。结果(1)广泛神经源性损害患者共80例,19例(23.75%、19/80)为下运动神经元综合征(LMNS)患者,61例(76.25%、61/80)为不同诊断级别的ALS(Awaji-shi ma标准);其中临床肯定型24例(30%、24/80),临床拟诊型22例(27.5%、22/80),临床可能型15例(18.75%、15/80)(χ2=0.067,P=0.381);(2)在不同诊断级别的ALS中,首发起病部位在延髓者,临床肯定型的例数多于拟诊型和可能型(P<0.05);(3)ALSFRS-R平均35.35±6.17,平均病程(27.7±22.2)年;与临床拟诊型和可能型患者组比较,肯定型组ALSFRS-R分值(31.00)更小,病程(1.64年)更短。结论广泛神经源性损害的患者中虽然不同诊断级别的ALS患者占大部分,但临床肯定型仅1/3;预后不良的因素为延髓起病、评分低和病程短。

Objective To analysis the diagnosis and prognosis of patients with amyotrophic lateral sclerosis（ALS）.Methods The patients referred to our hospital between February 2008 and January 2010,who manifested clinically with muscle weakness/atrophy/cramps / fasciculation of slow onset and progression and showed with generalized distribution of neurogenic involvement on needle electromyography,were enrolled for registration.The onset lesion site,revised amyotrophic lateral sclerosis functional rating score （ALSFRS-R） and course of illness were documented,and the relevance of clinical data was analyzed.Results （1） There were totally 80 patients with generalized distribution of neurogenic involvement,among which 19 cases（23.75%,19/80） were diagnosed as lower motor neuron syndrome（LMNS）,61（76.25%、61/80） as ALS with different levels of diagnostic certainty （Awaji-shima consensus recommendations）.There were 24 cases consistent with clinical definite ALS（30%,24/80）,22 clinical probable （27.5%,22/80）,15 clinical possible（18.75%、15/80）,respectively（χ^2=0.067,P=0.381,v.s.LMNS）.（2） In ALS patient with different levels of diagnostic certainty,the number of those with bulbar onset in clinical definite subgroup was significantly more than that in clinical probable and possible subgroups（P〈0.05）.（3） The average ALSFRS-R was （35.35±6.17） and the course of illness was（27.7±22.2） years in all of 80 patients.Compared with clinical probable and possible subgroups,the average score of ALSFRS-R（31.00） was smaller and the course（1.64 years） was shorter significantly in clinical definite subgroup.Conclusions ALS with different levels of diagnostic certainty accounts for the majority in the patients with generalized distribution of neurogenic involvement,but the number of clinical definite ALS was only one third;the poor prognostic indicators are bulbar onset,low ALSFRS-R and short course of illness.