关键词: 强直性肌营养不良, 肌营养不良, 肌电图, 神经传导速度, 周围神经病

Abstract: To study clinical and electrophysiological features in myotonic dystrophy. Methods: Electromyography(EMG)and nerve conduction velocity were performed in twenty-four patients with myotonic dystrophy. Results: In 46% of cases,(motor/or sensory)nerve conduction velocity was abnormal in at least two separate nerves. It was characterized by mildly slowing motor/or sensory nerve conduction and reducing amplitude of compound motor action potential(CMAP)/or sensory nerve action potential(SNAP). The severity of disease was associated with the patient's age. Conclusion: Peripheral neuropathy in myotonic dystrophy is not rare. It is a sensorimotor axonal peripheral neuropathy. The clinical symptoms are worse in older patients.

Key words: myotonic dystrophy|muscular dystrophy|electromyography(EMG)|nerve conduction velocity|peripheral neuropathy