50例散发性肌萎缩侧索硬化临床表现分析及文献复习

目的：探讨散发性肌萎缩侧索硬化（SALS）的临床特征,为早期准确诊断SALS提供依据。方法：回顾性研究近年来作者收治的50例散发性肌萎缩侧索硬化患者的临床表现,对其发病特点、症状、体征及实验室检查进行统计分析。结果：SALS在临床上隐袭起病,缓慢进展,50岁前后发病,平均年龄50.4岁,20岁以前70岁以后发病少见,平均病程3.5年。男性显著多于女性。首发症状为单侧上肢远端肌肉无力和肌肉萎缩最常见,其次为球麻痹、肌束震颤、锥体束征,少数出现肢体麻木/疼痛/发凉等感觉异常。上肢远端为好发病部位,其次为延髓支配肌肉。临床主要症状是肢体无力、肢体和舌肌肌肉萎缩、锥体束征、肢体和舌肌肌束震颤。舌咽/迷走神经运动核最常受累,舌下神经、副神经、面神经等运动核也多见损害,其余颅神经罕见损害。无明显诱因。所有SALS侵害的部位均表现神经源性损害,胸锁乳突肌肌电图检查、头颅/脊髓CT和MRI检查、肌肉活检有鉴别诊断意义。结论：目前SALS的诊断仍然依靠临床表现。肌电图、肌肉活检是SALS诊断和鉴别诊断的重要辅助手段。

The Clinical Features of Sporadic Amyotrophic Lateral Sclerosis

Objective: To provide clinical data for earlier accurate diagnosis of sporadic amyotrophic lateral sclerosis(SALS),and to study and analysis the clinical features of SALS.Methods:The records of the clinical SALS information published in professional journal and that of cases hospitalized in my hospital during recent 17 years were retrospectively reviewed.The clinical characteristics,symptoms,signs,and data of laboratory examination of the patients were used for statistical analysis.Results:The onset of most SALS was a chronic insidious neurodegenerative disease of slowing progressive.Onset age was around 50 years,mean onset age is 50.4 years,onset before 20 years and after 70 years was less.The mean course of disease was 3.5 years.Males were significantly more than females.The commonest initial symptoms were muscle weakness and atrophy in distal terminal of one upper limb,and the second one was bulb paralysis,fasciculation,and pyramidal sign,paraesthesia of limb numbness,pain,and cool feel showed less.The distal terminal of upper limb was the most predilected position,followed by muscle controlled by medullae.Main clinical sign was that limb weakness,atrophy of limb and tongue muscle,pyramidal sign,fasciculation of limb and tongue muscle.The commonest cranial nerves were glossopharyngeal nerve and vagus nerveprincipal.hypoglossal nerve,accessory nerve and facial nerve cause often were damaged too,the other cranial nerves were never damaged.The past disease history was no statistically significance.Electromyogram of the SALS invasive muscle showed neural damage.Sternocleidomastoid Electromyogram,CT,MRI of head and spinal cord,muscle biopsy were important assistant method of SALS diagnosis and differential diagnosis.Conclusion:Now the SALS diagnosis is depend on clinical sign yet,Electromyogram,Muscle biopsy is a useful assistant method of diagnosis and differential diagnosis.