平山病的诊治进展

平山病为临床非常少见的一种良性自限性疾病,是一种与曲颈运动有关的颈髓病变。典型表现为：局限性上肢远端、手指及腕无力,伴远端肌肉萎缩、寒冷麻痹和手指伸展时束颤,肌电图表现为神经源性损害。在临床上与运动神经元病肌萎缩侧索硬化及脊髓进行性肌萎缩表现相似而预后截然不同,主要是男性青少年发病。

Diagnosis and treatment of hirayama disease

Objective Hirayama disease is a benign self-limited disease that is rare in clinic,which is the cervical cord lesion related to cervical flexion.Its characteristic manifestation is myasthenia of limited distal of limb,finger and wrist accompanied with distal amyotrophy,cold anaesthesia and fasciculation when finger or wrist extending.The manifestation of electromyogram is harmfulness derived from nerve.It has a prefer good prognosis,and often occurs in adolescent boys.It is completely different from motor neuron disease with amyortrophyr and side cable cirrhosis,and also different from progressing amyortrophy of cervical spinal cord disease.