95例运动神经元病的临床特征

Clinical features of motor neuron diseases：an analysis of 95 cases

摘要:

目的 了解运动神经元病的临床特点。方法 回顾性分析95例运动神经元病患者的发病特点、病情进展模式及相关检查，对其进行总结分析以提高早期诊断识别率。结果 95例患者中ALS 73例（76.8%），PLS 1例（1.1%），PMA 13例（13.7%），PBP 8例（8.4%）；平均发病年龄为48.85±11.02岁。上肢无力或肌萎缩首发者44例（46.3%），下肢无力或肌萎缩首发者24例（25.3%），以四肢无力或肌萎缩首发者10例（10.5%），以球麻痹症状首发者17例（17.9%）。男女比例为1.26：1。女性患者较男性更易出现球麻痹或以其作为首发症状（P<0.05）。结论 MND发病男性多于女性，起病以颈段最多，ALS最常见，电生理检测对本病诊断意义重大，需按照诊断标准进行规范的鉴别诊断以排除其他疾病。

Abstract:

Objective To investigate the clinical features of motor neuron diseases (MND).Methods A retrospective analysis was performed on the clinical data of 95 MND patients to investigate the features of onset, patterns of progression, and related examinations. The obtained data were summarized and analyzed to increase early diagnosis rate.Results Of the 95 patients, 73 (76.8%) had amyotrophic lateral sclerosis (ALS), 1 (1.1%) had primary lateral sclerosis, 13 (13.7%) had progressive muscular atrophy, and 8 (8.4%) had progressive bulbar palsy. The mean age of onset was 48.85±11.02 years. As to the initial symptoms, 44 cases (46.3%) had upper limb weakness or amyotrophia, 24 cases (25.3%) had lower limb weakness or amyotrophia, 10 cases (10.5%) had limb weakness or amyotrophia, and 17 cases (17.9%) had bulbar paralysis. The male-to-female ratio was 1.26：1. Females were significantly more likely to develop bulbar paralysis or have bulbar paralysis as the initial symptom than males (P<0.05).Conclusions MND is more common in men than in women. The onset is most seen in cervical myotomes. ALS is the most common type of MND. Electrophysiological examination is of great significance for the diagnosis of MND and should be carried out according to the diagnostic criteria for differential diagnosis.