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脊髓空洞症表现中的骨科特征。

Orthopaedic features in the presentation of syringomyelia.

作者信息

Williams B

出版信息

J Bone Joint Surg Br. 1979 Aug;61-B(3):314-23. doi: 10.1302/0301-620X.61B3.158024.

Abstract

The orthopaedic surgeon is often the first consultant to whom a patient with syringomyelia is referred. The disease is not as rare as he may suppose, but its early presenting features are very variable; if he relies solely on such familiar features as pes cavus and scoliosis, he may well miss the diagnosis. The commonest presenting symptom is pain in the head, neck, trunk or limbs; headache or neckache made worse by straining is particularly significant. A history of birth injury also may suggest the possibility of syringomyelia, especially if any spasticity subsequently worsens. Neurological features which may be diagnostic include nystagmus, dissociated sensory loss, muscle wasting, spasticity of the lower limbs or Charcot's joints. Radiographic features include erosion of the bodies of cervical vertebrae and widening of the spinal canal; if, at C5, the size of the canal exceeds that of the body by 6 millimetres in the adult, pathological dilatation is present. The presence of basilar invagination or other abnormalities of the foramen magnum, of spina bifida occulta and of scoliosis are further pointers. Thermography is a useful way of showing asymmetrical sympathetic involvement in early cases. A greater awareness of the prevalence of syringomyelia may lead to earlier diagnosis and to early operation, which appears to hold out the best hope of arresting what is all too commonly a severely disabling and progressive condition.

摘要

骨科医生常常是脊髓空洞症患者转诊的首位会诊医生。这种疾病并不像他可能认为的那样罕见,但其早期表现非常多样;如果他仅仅依赖诸如高弓足和脊柱侧弯等常见特征,很可能会漏诊。最常见的症状是头部、颈部、躯干或四肢疼痛;因用力而加重的头痛或颈部疼痛尤为重要。出生时受伤的病史也可能提示脊髓空洞症的可能性,尤其是如果随后出现任何痉挛加重的情况。可能具有诊断意义的神经学特征包括眼球震颤、分离性感觉丧失、肌肉萎缩、下肢痉挛或夏科关节。影像学特征包括颈椎椎体侵蚀和椎管增宽;在成人中,如果在C5水平,椎管大小超过椎体6毫米,则存在病理性扩张。基底凹陷或枕骨大孔的其他异常、隐性脊柱裂和脊柱侧弯的存在是进一步的线索。热成像术是早期病例中显示不对称交感神经受累的一种有用方法。对脊髓空洞症患病率有更高的认识可能会导致更早的诊断和早期手术,这似乎为阻止这种通常严重致残且进行性发展的疾病带来了最大的希望。

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