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首页> 外文期刊>Journal of child neurology >Use of Clinical and Electrical Myotonia to Differentiate Childhood Myopathies
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Use of Clinical and Electrical Myotonia to Differentiate Childhood Myopathies

机译：利用临床和电肌强直来区分儿童肌病

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We retrospectively reviewed 2030 childhood electromyograms performed over an 11-year period (2004-2014). Twenty children (1%) with myotonic discharges were identified and placed into 2 groups. Group A (electrical and clinical myotonia) comprised 9 children (8 with myotonia congenita and 1 with paramyotonia congenita); all of them had diffuse myotonic discharges without clinical weakness or elevated creatine kinase. Group B (electrical myotonia without clinical myotonia) comprised 11 children (4 with inflammatory myopathy; 3, congenital myopathy, 3, muscular dystrophy; and 1, congenital muscular dystrophy). Clinical weakness was demonstrated in all of them and elevated creatine kinase in 6; all had a myopathic electromyogram and scattered myotonic discharges. We conclude that myotonic discharges are a rare but characteristic spontaneous discharge identified during electrodiagnostic studies in children. The presence of electrical and clinical myotonia provides helpful clues to differentiate between various muscle disorders in children.
机译：我们回顾性地回顾了在11年（2004-2014年）内进行的2030例儿童肌电图检查。确定了20例（1％）的强直性放电儿童，并将其分为2组。 A组（电和临床肌强直）包括9名儿童（8名先天性肌强直和1名先天性肌强直）。它们均具有弥散性肌强直放电，无临床无力或肌酸激酶升高。 B组（无临床肌强直性肌强直）包括11名儿童（4名患有炎性肌病; 3名先天性肌病; 3名肌营养不良; 1名先天性肌营养不良）。所有患者均表现出临床无力，肌酸激酶升高6例。全部都有肌病性肌电图和散在的肌强直放电。我们得出的结论是，在儿童的电诊断研究中，强直性放电是一种罕见但特征性的自发放电。电动和临床肌强直的存在为区分儿童各种肌肉疾病提供了有用的线索。

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