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强直性肌营养不良症的神经肌肉损害特征为肌强直、进行性肌无力和肌萎缩,其电生理呈现惯常的肌病型式及典型的高频放电。中枢神经系统也可受累,脑电图检查θ、δ波过多并有局灶性尖波发放,有脑萎缩、局灶性白质病变和脑室系统弥漫性扩大。为了阐明中枢神经受累是否反映出中枢运动通路的传导异常,作者采用磁刺激头皮和颅骨,为5个家系中15例有典型临床表现的强直性肌营养不良症患者作了检查。中枢传导时间(CCT)以ms记,并根据以下公式计算:
Neuromuscular damage in myotonic dystrophy is characterized by myotonic rigidity, progressive myasthenia and muscle atrophy, and electrophysiological presentation of myopathy and typical high-frequency discharges. Central nervous system can also be involved, EEG check θ, δ wave too much and there are focal spikes distribution, brain atrophy, focal white matter lesions and ventricular system diffuse expansion. To clarify whether CNS involvement reflects conduction anomalies in the central motor pathway, the authors used magnetic stimulation of the scalp and skull to examine 15 patients with myotonic dystrophy typical of clinical manifestations in 5 pedigrees. Central conduction time (CCT) in milliliters, and according to the following formula: