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背景:脊髓性肌萎缩症是运动神经元疾病中病变仅影响下运动神经元的一组疾病。成人型少见,目前对其研究较少。目的:总结成人型脊髓性肌萎缩症骨骼肌病理学特征。设计:以诊断为依据的回顾性研究。地点和对象:收集1998-02/2002-02在解放军第八一医院南京医学院第二附属医院和南京军区总医院经肌肉活检确诊的、有完整临床资料的门诊和住院患者共46例。方法:结合临床特征及病理学改变进行分析。主要观察指标:病史、家族史、完整体格检查、相关血液及血生化、肌电图和肌肉活检。结果:临床表现为进行性对称性肢体近端肌萎缩,肌无力,实验室检查血肌酸磷酸肌酶12例中轻度升高,肌电图检查2例正常,3例呈轻度肌源性损害,余37例呈神经元性损害,肌活检主要为小群性肌萎缩,腺苷三磷酸酶染色见同型肌群化及肌纤维代偿性肥大。结论:肌活检对成人型脊髓性肌萎缩症具有诊断和鉴别诊断意义。适当、持久的康复锻炼可能对维持患者的运动功能有帮助。
BACKGROUND: Spinal muscular atrophy is a group of diseases in which motor neurons are affected by lesions in motor neuron disease. Rare adults, the current research on its less. Objective: To summarize the pathological features of skeletal muscle in adults with spinal muscular atrophy. Design: A retrospective study based on diagnosis. Location and Subjects: A total of 46 outpatients and inpatients with complete clinical data, confirmed by muscle biopsy at the Second Affiliated Hospital of Nanjing Medical College and the Nanjing Military Region General Hospital of the 8th Hospital of PLA from February 1998 to February 2002 were collected. Methods: Combined with clinical features and pathological changes were analyzed. MAIN OUTCOME MEASURES: Medical history, family history, complete physical examination, related blood and blood biochemistry, electromyography, and muscle biopsy. Results: The clinical manifestations of progressive symmetry of proximal limb muscle atrophy, muscle weakness, laboratory tests of serum creatine phosphokinase slightly elevated in 12 cases, electromyography 2 cases were normal, 3 cases were mild myogenic Damage, more than 37 cases of neuronal damage, muscle biopsy mainly for small groups of muscle atrophy, adenosine triphosphatase staining see the same type of muscle and compensatory hypertrophy of muscle fibers. Conclusion: Muscle biopsy has diagnostic and differential diagnostic significance for adult spinal muscular atrophy. Proper, long-term rehabilitation exercise may be helpful in maintaining the patient’s motor function.