Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa 920-8640, Japan.
Joint Bone Spine. 2011 May;78(3):316-8. doi: 10.1016/j.jbspin.2010.12.008. Epub 2011 Jan 26.
We describe an 18-year-old male with cervical flexion myelopathy with Hirayama disease-like features who showed apparent long tract signs. He first experienced insidious-onset hand muscle weakness and atrophy at the age of 15. Subsequently, he developed sensory disturbance in his lower limb. Neurological examination revealed atrophy and weakness in the right hand and forearm, pyramidal signs in the right lower extremity, and disturbance of superficial sensation in the lower left half of the body. Cervical magnetic resonance images and computed tomographic myelography revealed anterior displacement with compression of the cervical cord in flexion that was more apparent in the right side. The right side of the cervical cord showed severe atrophy. The mechanisms of myelopathy in our patient appeared to be same as that of "tight dural canal in flexion," which has been reported to be the mechanism of juvenile muscular atrophy of the unilateral upper extremity (Hirayama disease). Patients with Hirayama disease generally show minimal sensory signs and no pyramidal signs. An autopsy case of Hirayama disease revealed confined necrosis of the cervical anterior horn without obvious changes in the white matter. Our patient's disease progression suggests that cervical flexion myelopathy patients with severe cervical cord compression in flexion may develop extensive cervical cord injury beyond the anterior horn.
我们描述了一名 18 岁男性患有颈曲性脊髓病，具有平山病样特征，表现出明显的长束体征。他在 15 岁时首次出现隐匿性手部肌无力和萎缩。随后，他出现下肢感觉障碍。神经学检查显示右手和前臂肌肉萎缩和无力，右侧下肢锥体束征，左下肢下半部浅感觉障碍。颈椎磁共振成像和计算机断层脊髓造影显示颈椎前移位伴屈曲时颈髓受压，右侧更为明显。右侧颈髓严重萎缩。我们患者的脊髓病机制似乎与“屈曲时硬脑膜管紧张”相同，这被认为是青少年单侧上肢肌萎缩（平山病）的机制。平山病患者通常表现出最小的感觉体征，没有锥体束征。平山病的尸检病例显示颈前角局限性坏死，而白质无明显变化。我们患者的疾病进展表明，在屈曲时严重颈髓受压的颈曲性脊髓病患者可能会发生超出前角的广泛颈髓损伤。
