Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
J Neurosurg Spine. 2013 Mar;18(3):303-9. doi: 10.3171/2012.11.SPINE12860. Epub 2013 Jan 4.
Neuropathic arthropathy (Charcot joint) caused by syringomyelia is rare and commonly misdiagnosed. Few cases have been reported by neurosurgeons. The aims of this study were to analyze the clinical and imaging presentations of neuropathic arthropathy and to discuss the effect of surgical management of the primary neurological deficits on neuropathic arthropathy.
The authors retrospectively reviewed clinical and imaging data of 12 patients with neuropathic arthropathy caused by syringomyelia who were referred to the department of neurosurgery between January 2003 and September 2012. Radiographs revealed destruction, dislocation, disorganization, and increased density or debris in the joints. Magnetic resonance imaging showed a syrinx of the spinal cord in all patients, with Chiari malformation in 11 patients and tethered spinal cord in 1 patient. Neurosurgical operations were performed in 5 of 12 patients, including posterior fossa decompression in 4 patients and syrinx-subarachnoidal shunt placement in 1 patient. Surgical management of the neuropathic joints was not performed in any of the patients. All patients were followed up, with a mean duration of 39 months.
Sixteen joints were involved, including 10 elbows, 3 shoulders, 2 interphalangeal joints, and 1 wrist. The side of the syrinx on cervical axial MRI was consistent with the side of the affected limb in every patient. Five patients who underwent neurosurgical treatments stated improvement in neurological dysfunctions and no deterioration in symptoms related to neuropathic arthropathy. In the 7 patients without neurosurgical treatments, 5 reported aggravation of neuropathic arthropathy manifestations, with deterioration of neurological symptoms in 4 of the 5 patients. The condition of the other 2 patients remained stable.
The elbow is the most frequently involved joint in neuropathic arthropathy caused by syringomyelia, followed by the shoulder. The authors speculate that the side of the syrinx determines the side of the neuropathic arthropathy. A detailed medical history and a careful physical examination are crucial for differentiating neuropathic arthropathy from other joint lesions. This study suggests that early management of the primary neurological condition may play an important role in preventing the development of neuropathic arthropathy and avoiding disease progression.
由脊髓空洞症引起的神经关节病（夏科关节）罕见且常被误诊。神经外科医生报告的病例很少。本研究旨在分析神经关节病的临床和影像学表现，并探讨对原发性神经功能缺损进行手术治疗对神经关节病的影响。
作者回顾性分析了 2003 年 1 月至 2012 年 9 月期间到神经外科就诊的 12 例由脊髓空洞症引起的神经关节病患者的临床和影像学资料。X 线片显示关节破坏、脱位、紊乱、密度增加或有碎片。磁共振成像显示所有患者均存在脊髓空洞，11 例存在 Chiari 畸形，1 例存在脊髓栓系。对 12 例患者中的 5 例行神经外科手术，其中 4 例行后颅窝减压术，1 例行脊髓空洞-蛛网膜下腔分流术。未对任何患者进行神经关节病的手术治疗。所有患者均获得随访，平均随访时间为 39 个月。
共涉及 16 个关节，包括 10 个肘部、3 个肩部、2 个指间关节和 1 个腕部。每个患者颈椎轴位磁共振成像上的脊髓空洞侧与患侧肢体一致。5 例接受神经外科治疗的患者表示神经功能障碍改善，神经关节病相关症状无恶化。在未接受神经外科治疗的 7 例患者中，5 例报告神经关节病表现加重，其中 4 例患者神经症状恶化。另外 2 例患者病情稳定。
由脊髓空洞症引起的神经关节病最常累及肘部，其次是肩部。作者推测脊髓空洞的位置决定了神经关节病的位置。详细的病史和仔细的体格检查对于鉴别神经关节病与其他关节病变至关重要。本研究表明，早期治疗原发性神经状况可能对预防神经关节病的发展和避免疾病进展起到重要作用。
