Defaria C R, De Melo-Souza S E, Pinheiro E D
J Neurol Neurosurg Psychiatry. 1979 Jul;42(7):600-5. doi: 10.1136/jnnp.42.7.600.
Twenty haemophilic patients were examined neurologically and found to have varying degrees of muscle atrophy. The pathogenesis of the atrophy was investigated by electromyography, serum muscle enzyme levels, and muscle biopsies. In most cases the EMG showed such abnormalities as reduced numbers of functioning motor units and "myopathic" motor unit potentials. Serum enzyme levels were frequently raised, and type 2 fibre atrophy was found in the three patients who underwent muscle biopsy. These findings suggest the presence of neuromyopathic involvement in haemophilic patients.
对20名血友病患者进行了神经学检查，发现他们存在不同程度的肌肉萎缩。通过肌电图、血清肌肉酶水平和肌肉活检对萎缩的发病机制进行了研究。在大多数情况下，肌电图显示出诸如功能性运动单位数量减少和“肌病性”运动单位电位等异常。血清酶水平经常升高，在接受肌肉活检的3名患者中发现了2型纤维萎缩。这些发现提示血友病患者存在神经肌肉病变。
