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Objective To evaluate denervation changes [fibrillation potentials (fib) and positive sharp waves (psw)] in thoracic paraspinal muscles as diagnostic aids in amyotrophic lateral sclerosis (ALS). Methods The fib and psw of thoracic paraspinal muscles,the motor unit potential of sternocleidomastoid muscle were detected in 50 patients who had been identified as having ALS.Age and sex-matched,30 normal subjects and 30 patients who suffered radicular disease in cervical or lumbosacral region were also detected in thoracic paraspinal muscles for finding fib and psw serving as controls. Results In ALS group,a vast of fib and psw were found in thoracic paraspinal muscles in 41 of 50 ALS patients (82%),they were not found with sternocleidomastoid muscle,but the motor unit potential of this muscle was shown having long-duration and high-amplitude in 48 of 50 ALS patients (96%),which were coincided with the chronic neurogenic changes.6 of 50 patients had denervation changes of tongue muscles,3 of them found fib and psw.2 of 30 patients who suffered from radicular diseases were found with a few of fib and psw changes in thoracic paraspinal muscles.None of these symptoms were found in normal subjects.Conclusion A vast of fib and psw in thoracic paraspinal muscles should represent a sensitive tool for diagnosis of ALS.
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55 ?s ranged from 5 0%～100 0% (mean 60 6%) The ratio of block ranged from 0 0%～90 0% (mean 29 3%) FD ranged from 1 4～4 0 (mean 2 56) There were remarkably increased jitter, block and FD in 51 patients with definite or probable ALS It was also found that there was a negative correlation between extensor digitorum communis strength grades and the ncreased jitter with or without block and FD ( P
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Objective To analyze the clinical characteristics of dopa-responsive dystonia (DRD). Methods The clinical characteristics and the causes of misdiagnosis in 15 patients with DRD were reviewed retrospectively.Results 4 male and 11 female patients were investigated in this study. The age at DRD onset was from 3~25 years old,the average age was 11.7 years old.The courses ranged from 2 to 29 years and the average was 12.1 years. The symptoms in female patients often aggravated during gravidity and delivery. Main clinical features included gait irregularity, postural instability and tremor, with marked diurnal fluctuation. The disease may be misdiagnosed as cerebral palsy, idiopathic torsion dystonia, essential tremor, parkinson's disease, neurosis and so on. All the patients in this study improved obviously in 2 weeks after therapy with low dose of Madopar, and recovered in 2~4 years of follow up.Conclusions Clinical diversity and diurnal fluctuation are the characteristics of DRD. Early trial treatment with low dose of Levodopa should be highly recommended for the generalized dystonia in children and untypical adults.