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BACKGROUND: Spinal muscular atrophy (SMA) is a kind of degenerative disease of nervous system. There are 4 types in clinic, especially types Ⅰ, Ⅱ and Ⅲ are common, and the researches on those 3 types are relative mature. Type Ⅳ is a kind of adult spinal muscular atrophy (ASMA), which has low incidence rate and is often misdiagnosed as amyotrophic lateral sclerosis, muscular dystrophy, cervical syndrome, or others. OBJECTIVE: To observe the clinical features of 46 ASMA patients and analyze the relationship between course and activity of daily living. DESIGN: Case analysis. SETTING: Departments of Neurology of the 81 Hospital of Chinese PLA, the Second Affiliated Hospital of Nanjing Medical College and General Hospital of Nanjing Military Area Command of Chinese PLA. PARTICIPANTS: A total of 46 ASMA patients were selected from the Departments of Neurology of the 81 Hospital of Chinese PLA, the Second Affiliated Hospital of Nanjing Medical College and General Hospital of Nanjing Military Area Command of Chinese PLA between April 1998 and January 2002. All patients were consentient. Among 46 cases, there were 37 males and 9 females with the mean age of 42 years. The patients’ courses in all ranged from 6 months to 23 years, concretely, courses of 37 cases were less than or equal to 5 years, and those of 9 cases were more than or equal to 6 years. METHODS: ① All the 46 ASMA patients were asked to check blood sedimentation, anti O, serum creatinine, creatine, blood creatine phosphokinase (CPK) and muscular biopsy as early as possible. ② X-ray was used to measure plain film of cervical vertebra borderline film of cranium and neck at proximal end of upper limb of 25 cases and plain film of abdominal vertebra at proximal end of lower limb of 17 cases. ③ Cerebrospinal fluid of lumbar puncture was checked on 42 cases, for routine examination, biochemical examination, and immunoglobulin examination. Electromyogram (EMG) was also examined to 42 cases. ④ Barthel index was used to evaluate activities of daily living (ADL) of patients with various courses. The index ranged from 1 to 100. The more the index of a ASMA was, the stronger his independence was. ⑤ The Barthel indexes of patients with courses ≤ 5 years and those ≥ 6 years were compared with univariate analysis of variance. MAIN OUTCOME MEASURES: ① Incidences of all patients at the first time; ② values of relative blood and blood biochemistry; ③ results of muscular biopsy; ④ results of EMG and relative X-ray plain film of 42 cases; ⑤ results of cerebrospinal fluid of 42 cases; ⑥ comparisons of Barthel index of patients with various courses. RESULTS: A total of 46 ASMA patients were involved in the final analysis. ① Incidence on the first time: 25 patients had the disease at the proximal end of upper limb, 17 at the proximal end of lower limb, and 4 at the four limbs. ② Value of serum-blood CPK of one fourth patients was increased slightly (3.034-9.735 μkat/L; normal value: 0.400-3.001 μkat/L), and other values of blood and blood biochemical indicator were normal. ③ Results of muscle biopsy of all patients showed that a small group of muscular atrophy could be observed mostly, and muscle group in the same type and compensatory hypertrophy of muscle fibres were also observed with ATP enzyme staining. ④ Results of EMG of 42 cases suggested that 37 patients had mild and moderate nerve-derived injury and 3 had mild muscle-derived injury. Results of all the X-ray plain films in this study were normal. ⑤ Results of routine, biochemical and immunoglobulin examination in cerebrospinal fluid of lumbar puncture in 42 cases were all normal. ⑥ The difference between Barthel indexes of patients with courses ≤ 5 years and those ≥ 6 years was not significant [(64.73±20.38) vs (68.89±21.76) points, P > 0.05]. CONCLUSION: ① Amyasthenia is mainly occurred at the proximal end of the four limbs of ASMA patients. A small group of muscular atrophy is its mostly pathological change, and the progression of the disease is slow. ② Most patients have mild and moderate nerve-derived injury under EMG examination. ③ The duration of a patient suffered from the disease has no obvious effect on his ADL ability.
BACKGROUND: Spinal muscular atrophy (SMA) is a kind of degenerative disease of nervous system. There are 4 types in clinic, especially types I, Ⅱ and Ⅲ are common, and the researches on those 3 types are relatively mature. Type Ⅳ is a kind of adult spinal muscular atrophy (ASMA), which has low incidence rate and is often misdiagnosed as amyotrophic lateral sclerosis, muscular dystrophy, cervical syndrome, or others. OBJECTIVE: To observe the clinical features of 46 ASMA patients and analyze the relationship between course and activity of daily living. DESIGN: Case analysis. SETTING: Departments of Neurology of the 81 Hospital of Chinese PLA, the Second Affiliated Hospital of Nanjing Medical College and General Hospital of Nanjing Military Area Command of Chinese PLA. PARTICIPANTS: A total of 46 ASMA patients were selected from the Departments of Neurology of the 81 Hospital of Chinese PLA, the Second Affiliated Hospital of Nanjing Medical College and General Hospital of Nanjing M ilitary Area Command of Chinese PLA between April 1998 and January 2002. All patients were consentient. Among 46 cases, there were 37 males and 9 females with the mean age of 42 years. The patients’ courses in all ranged from 6 months to 23 years , all the 46 ASMA patients were asked to check blood sedimentation, anti O, serum creatinine , creatine, blood creatine phosphokinase (CPK) and muscular biopsy as early as possible. ② X-ray was used to measure plain film of cervical vertebra borderline film of cranium and neck at proximal end of upper limb of 25 cases and plain film of abdominal ③ Cerebrospinal fluid of lumbar puncture was checked on 42 cases, for routine examination, biochemical examination, and immunoglobulin examination. ④ Electrobiology of EMG was also examined in 42 cases. ④ Barthel i ndex was used toThe activities of daily living (ADL) of patients with various courses. The index ranged from 1 to 100. The more the index of a ASMA was, the stronger his independence was. ⑤ The Barthel indexes of patients with courses ≤ 5 years and those ≥ 6 years were compared with univariate analysis of variance. MAIN OUTCOME MEASURES: ① Incidences of all patients at the first time; ② values of relative blood and blood biochemistry; ③ results of muscular biopsy; ④ results of EMG and relative X-ray plain RESULTS: A total of 46 ASMA patients were involved in the final analysis. ① Incidence on the first time: 25 patients had the disease at the proximal end of upper limb, 17 at the proximal end of lower limb, and 4 at the four limbs. ② Value of serum-blood CPK of one fourth patients was increased slightly (3.034-9.735 μkat / L; normal value : 0.400-3.0 01 μkat / L), and other values of blood and blood biochemical indicator were normal. ③ Results of muscle biopsy of all patients showed that a small group of muscular atrophy could be observed mostly, and muscle group in the same type and compensatory hypertrophy of Results of EMG of 42 cases suggested that 37 patients had mild and moderate nerve-derived injury and 3 had mild muscle-derived injury. Results of all the X-ray plain films in this study were normal. ⑤ Results of routine, biochemical and immunoglobulin examination in cerebrospinal fluid of lumbar puncture in 42 cases were all normal. ⑥ The difference between Barthel indexes of patients with courses ≤ 5 years and those ≥ 6 years was not significant [(64.73 ± 20.38) vs (68.89 ± 21.76) points, P> 0.05]. CONCLUSION: ① Amyasthenia is mostly at the proximal end of the four limbs of ASMA patients. A small group of muscular atrophy is its major patholo gical change, and the progression of thMost patients have mild and moderate nerve-derived injury under EMG examination. ③ The duration of a patient suffered from the disease has no obvious effect on his ADL ability.