Hirayama K
Department of Neurology, School of Medicine, Chiba University.
Intern Med. 2000 Apr;39(4):283-90. doi: 10.2169/internalmedicine.39.283.
This disease is characterized by initially progressive muscular weakness and wasting of the distal upper limb(s) in young people predominantly in men, followed by a spontaneous arrest within several years. This disease has been thought to be separate from motor neuron diseases, yet some authors still consider the illness a variant of motor neuron disease. However, the pathological evidence of ischemic changes in the lower cervical anterior horn should facilitate differentiation of the disorder from degenerative motor neuron disease. Recent radiological investigations proved compressive flattening of the lower cervical cord due to forward displacement of the cervical dural sac and spinal cord induced by neck flexion. These findings suggest that sustained or repeated neck flexion may cause ischemic changes in the cervical anterior horn. Application of a cervical collar to minimize neck flexion prevents progressive muscular weakness in an early stage of the disease.
这种疾病的特征是，在年轻人（主要是男性）中，最初表现为进行性肌肉无力和远端上肢萎缩，随后在几年内自然停止进展。这种疾病一直被认为与运动神经元疾病不同，但一些作者仍将其视为运动神经元疾病的一种变体。然而，下颈段前角缺血性改变的病理证据应有助于将该疾病与退行性运动神经元疾病区分开来。最近的放射学研究证实，颈部屈曲会导致颈段硬膜囊和脊髓向前移位，从而使下颈段脊髓受压变扁。这些发现表明，持续或反复的颈部屈曲可能会导致颈段前角缺血性改变。在疾病早期使用颈托以尽量减少颈部屈曲可防止肌肉无力进展。
