Charcot Disease of the Spine: Diagnosis and Treatment

Yu-po Lee, S. Farhan, P. Kiester, N. Bhatia
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Abstract

or neurogenic spinal arthropathy, is a rare, slowly progressive degeneration of the spine. This phenomenon occurs in the setting of any prior condition leading to the loss of afferent innervation to the spine and central nervous system. The end result is that the normal protective sensation of the joints in the vertebral column is diminished. This then leads to the progressive degeneration of the spinal column. Jean-Martin Charcot first described a causal link between neurologic injury and progressive bone and joint damage in 1868. His theory was based on the destruction of certain peripheral joints that he observed in patients with tertiary syphilis. Charcot described this damage as “ataxic arthropathy.” The first case of Charcot spinal arthropathy was reported in 1884 by Kronig in a patient with tabes dorsalis secondary to tertiary syphilis. Historically, Charcot spinal arthropathy was most commonly reported in the setting of tertiary syphilis. Contemporary Charcot spine cases are more commonly seen in patients who have suffered traumatic spinal cord injuries. Charcot spinal arthropathy may also occur secondary to conditions that disrupt the sensory signaling pathways, such as syringomyelia, meningocele, myelomeningocele, diabetes mellitus, peripheral neuropathies, Parkinson disease, transverse myelitis, and other conditions. Repetitive external forces to the insensate vertebral column cause inflammation in the subchondral bone and articular cartilage. This inflammatory process stimulates facet destruction, intervertebral disc degeneration, and bone destruction. This ultimately leads to progressive deformity and gross spinal instability. Untreated, Charcot spinal arthropathy can result in significant morbidity due to continuous pain, loss of sitting tolerance, ascending neurologic dysfunction, infection, vascular injury, and even death. Early medical and surgical care is required once the diagnosis has been recognized.
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脊柱炭疽病:诊断与治疗
或神经源性脊柱关节病,是一种罕见的、缓慢进行的脊柱退行性变。这种现象发生在任何先前导致脊柱和中枢神经系统传入神经支配丧失的情况下。最终的结果是脊柱关节的正常保护感减弱。这会导致脊柱的进行性退化。Jean-Martin Charcot于1868年首次描述了神经损伤与进行性骨关节损伤之间的因果关系。他的理论是基于他在三期梅毒患者身上观察到的某些外周关节的破坏。Charcot将这种损伤描述为“共济失调性关节病”。Kronig于1884年报道了第一例Charcot脊柱关节病,患者为二级至三级梅毒患者。从历史上看,Charcot脊柱关节病最常见于三期梅毒。当代Charcot脊柱病例更常见于遭受创伤性脊髓损伤的患者。Charcot脊柱关节病也可能继发于破坏感觉信号通路的疾病,如脊髓空洞症、脊膜膨出、脊髓脊膜膨体、糖尿病、周围神经病变、帕金森病、横贯性脊髓炎和其他疾病。对不敏感脊柱的重复外力会导致软骨下骨和关节软骨发炎。这种炎症过程刺激小关节面破坏、椎间盘退变和骨破坏。这最终会导致进行性畸形和脊柱不稳定。未经治疗,Charcot脊柱关节病会因持续疼痛、失去坐位耐力、上行神经功能障碍、感染、血管损伤甚至死亡而导致严重的发病率。一旦诊断得到确认,就需要早期的医疗和外科护理。
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