Objective: To analyze the clinical manifestations and EMG characteristics of patients with amyotrophic lateral sclcrosis( ALS) and provide the data for the early and accurate diagnosis. Methods; The records of the clnical information in 52 patients with ALS during recent 3 years were retrospectively reviewed. The clnical characteristics and data of EMG and laboratory examination were analysed. Results; All cases developed sporadically. , with 36 males and 16 fcmals. The mean onset age was 54. 67 years. The mean course of disease was 13. 69 months. The onset of all cases was chronic and progressive. The common onset initial symptoms were muscle weakness and atrophy in limbs. The symptoms of dista limbs were more serious than those of proximal limbs. Cranial nerve nucleus were commonly involved, most common in the hypoglossal nucleus and nerve nucleus. The main clinical manifestations were that limb and tongue muscle weakness, atrophy and fasciculation, pyramidal sign, dysarthria, choking and difficulty swallowing. No sensory disturbances occurred. EMG showed wide region neurogenic abnormalities. Some patients with cervical spine MR1 showed cervical spondylosis. Conclusion; The ALS diagnosis still depends on clinical manifestation and EMG. EMG,MR1 and Blood tests arc useful for the diagnosis and differential diagnosis fo ALS.%目的:探讨视频脑电图(V-EEG)检查在婴儿痉挛诊断中的作用.方法:对2003年5月至2009年5月新诊断未经治疗的83例婴儿痉挛患儿进行V-EEG监测,分析监测录像中婴儿痉挛发作的临床表现和发作期以及发作间期的脑电图(EEG)特点.结果:83例共监测到78例112次临床发作,主要有3种类型典型发作,发作期EEG共7种形式.发作期EEG:患儿几乎都表现为高波幅慢波或单个高波幅尖慢波或棘慢波,随后出现弥漫性低电压,其它放电形式则少见.发作间期EEG以典型的高度节律失调(高度失律)和变异型的高度失律为主.结论:V-EEG能观察到婴儿痉挛的独特轴性发作形式,有时还能监测到合并其它形式发作,又能对发作期和发作间期EEG进行完整记录.发作间期EEG表现为高度失律失调及其变异型是大多数婴儿痉挛患儿的特征性EEG类型.V-EEG监测可为诊断和鉴别诊断婴儿痉挛症提供重要依据.
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