Kwast O, Ignatowicz R
EMG Laboratory, Rehabilitation Department, Child Health Centre, Warsaw, Poland.
Dev Med Child Neurol. 1990 Sep;32(9):800-7. doi: 10.1111/j.1469-8749.1990.tb08485.x.
Electrophysiological examinations were performed on 32 children aged three to 17 years who had typical clinical manifestations of ataxia-telangiectasia (AT). EMG findings demonstrated neurogenic lesions, more pronounced in the distal leg muscles of older children where they resembled the picture characteristic of motor neuron disease. Electrophysiological and nerve conduction results showed that generalised, progressive, sensory nervous system degeneration, with neurogenic amyotrophy affecting the distal part of the lower limbs, is an established feature of this disease and can be considered one of the diagnostic characteristics of AT. This allows the syndrome to be classified as an hereditary spinocerebellar degeneration.
对32名年龄在3至17岁之间、有典型共济失调毛细血管扩张症（AT）临床表现的儿童进行了电生理检查。肌电图检查结果显示为神经源性损害，在大龄儿童的小腿远端肌肉中更为明显，类似于运动神经元病的典型表现。电生理和神经传导结果表明，全身性、进行性感觉神经系统退化，伴有影响下肢远端的神经源性肌萎缩，是该疾病的既定特征，可被视为AT的诊断特征之一。这使得该综合征可被归类为遗传性脊髓小脑变性。
