J Neurol. 2010 Jan;257(1):149-51. doi: 10.1007/s00415-009-5329-6.
Two patients with a condition clinically resembling juvenile muscular atrophy of distal upper extremity (Hirayama disease) showed forward protrusive movement of the posterior cervical dura matter during neck flexion. The dura displacement was characteristically limited in an approximately central portion of the posterior dura, which is different from Hirayama disease, which exhibits whole posterior dura displacement. Interestingly, the restricted dura in these patients showed thickening with reduced elastic fibers, indicating that the decreased stretchability of the posterior dura had caused motor dominant cervical myelopathy.
两名表现出类似于远端上肢少年型近端运动神经元病（平山病）临床症状的患者，在颈部弯曲时出现后颈椎硬脑膜向前突出。硬脑膜移位的特征是局限于后硬脑膜的大约中央部分，这与平山病不同，平山病表现为整个后硬脑膜移位。有趣的是，这些患者的受限硬脑膜出现增厚和弹性纤维减少，表明后硬脑膜伸展性降低导致以运动为主的颈脊髓病。
