运动神经元病165例临床特征及危险因素分析

Clinical features and related risk factors of motor neuron disease : an analysis of 165 cases

目的探讨运动神经元病（MND）的临床特征及相关危险因素，为临床治疗提供依据。方法回顾性分析165例MND患者的临床资料，包括一般资料、首发症状部位、神经系统体征、影像学检查、伴随疾病、误诊情况、治疗情况、职业分布等。结果165例MND患者中，男女比例为1．84：1，平均发病年龄（52．2±10．4）岁；单侧上肢起病66例（40．0％），单侧下肢起病46例（27．9％），双上肢起病5例（3．0％），双下肢起病3例（1．8％），四肢起病12例（7．3％），延髓起病33例（20．0％）；70例患者曾被误诊，误诊率为42．4％；发病患者中，农林工作者76例（46．1％），有毒物质、金属、煤炭等接触者48例（29．1％），吸烟者51例（30．9％）；多数给予口服维生素B1、维生素B12等营养神经治疗，未见明显疗效。结论MND患者多以单侧肢体远端肌无力或肌萎缩起病，逐渐累及四肢。发病高峰集中在50～59岁，男性发病率高于女性；误诊率高；尚无有效治疗手段；职业暴露、手术外伤、毒物／药物接触史、吸烟等可能是MND的危险因素。

Objective To investigate the clinical characteristics and related risk factors of motor neuron disease （MND）. Methods The clinical data of 165 MND patients including general information, initial symptom, signs of nervous system, imaging examination, concominant diseases, misdiagnosis condition, therapies and professional distribution were analyzed retrospectively. Results In 165 MND patients, the ratio of male to female was 1.84： 1, and the average onset age was （52.2 ± 10.4） years. The onset sites of the disease accounted for 40.0% in unilateral upper limb, 27.9% in unilateral lower limb, 3.0% in bilateral upper extremities, 1.8% in bilateral lower extremities, 7.3% in four limbs, 20.0% in medullary bulb, respectively. Of 165 MND patients, 70 were ever misdiagnosed with a misdiagnosis rate of 42.4%. Of 165 MND patients, 76 （46.1% ） were agriculture and forestry workers, 48 （29.1%） were exposured to toxic substances or metal material, 51 （30.9%） were smoker. Most pa- tients were administered by oral vitamin Bl, vitamin B12 and other neurotrophic drugs, but no apparent effect was found. Conclusions In most MND patients, the MND is characterized by the onset of weakness and atrophy from unilateral distal limb, and then the four limbs are gradually affected. The onset peak is concentrated in the ages of 50 to 59 years. The prevalence of male is higher than that of female. The misdiagnosis rate is higher. MND is still lack of effective treatment. The risk factors of MND may be associated with oceupational exposures, surgical trauma, poison/drug contact history and smoking.