中国西南地区散发性肌萎缩侧索硬化症患者的临床特征

Clinical features of sporadic patients with amyotrophic lateral sclerosis in southwest China

目的研究中国西南地区散发性肌萎缩侧索硬化症（amyotrophic lateral sclerosis,ALS）患者的临床特征。方法分析我院2006年5月到2009年1月收治的成年发病的散发性ALS患者临床资料,并进行连续性的随访6个月。结果纳入198例患者,男128例,女70例。平均发病年龄（51.6±11.3）岁,诊断时间（16.9±15.5）个月,病程（36.9±25.2）个月,其中42例患者（21.2%）在随访过程中死亡。本组病例以脊髓症状起病的患者较延髓症状者多（161∶37）,且发病年龄（49.9±11.2岁）明显早于以延髓症状起病患者（59.0±8.6）岁（P〈0.001）。在连续性随访中,患者的症状均逐渐加重,每3个月ALSFRS评分（ALS Functional Rating Scale）逐渐减低（27.7±8.6分;20.4±6.3分,P=0.002）。相关分析发病年龄与病程呈明显负关联（r2=-0.297,P〈0.01）,发病年龄和平均诊断时间呈明显负关联（r2=-0.241,P〈0.01）,病程和诊断时间之间呈明显正相关（r2=0.775,P〈0.01）。48例患者服用利鲁唑治疗,与未服用利鲁唑组患者生存时间无统计学差异。结论 ALS起病隐袭,表现为逐渐进展的肌无力和萎缩。中国西南地区散发的ALS患者以50~60年龄段发病为主,男性多见,以脊髓症状发病患者较多,而且脊髓症状发病者较延髓症状发病者年龄轻。发病年龄越晚,病情发展越快,患者生存时间越短。ALSFRS是评价患者功能状态和病情的进展情况的敏感指标。

Objective This study is to analyze clinical features of sporadic patients with ALS in southwest China.Methods All patients diagnosed with adult-onset sporadic ALS were consecutively followed up,and their clinical characteristics were collected from May 2006 to January 2009.Results One hundred and ninety-eight patients were included in the study.There were one hundred and twenty-eight male and seventy female.The mean age of onset was（51.6±11.3）years.The mean diagnostic delay was 16.9±15.5 months and the mean disease duration was（36.9±25.2）months.Forty-two patients（21.2%）died during the period of follow-up.There are more patients with initial spinal symptoms than those with initial bulbar symptoms（161 vs 37）and the onset age of the spinal onset group（49.9±11.2 years）was significantly earlier than that of the bulbar onset group（59.0±8.6 years）（P0.001）.During the consecutive follow-up,the symptoms of all ALS patients gradually progressed and the ALSFRS significantly decreased in every three months（from 27.7±8.6 to 20.4±6.3,P=0.002）.Time to diagnosis positively correlated with disease duration,whereas disease duration and time to diagnosis negatively correlated with onset ago.There was no significant difference in disease duration between patients with 100 mg/day riluzole treatment（forty-eight patients）and without.Conclusions ALS is characterized by onset slowly and progressive muscular paralysis.The average age of onset of sporadic patients with ALS in southwest China is about fifty to sixty years.The male is predominant and there are more patients whose initial symptoms involved the spinal,while the onset age of the spinal onset group is significantly earlier than that of the bulbar onset group.The older the onset,the more rapid the progression of the disease and shorter the survival time.ALSFRS can be a sensitive tool for evaluation of the functional status and disease progression in ALS patients.