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系统性硬化症合并颈椎钙化患者的中央脊髓综合征:病例报告及文献复习

Central cord syndrome in a patient with systemic sclerosis and cervical calcinosis: case report and review of literature.

作者信息

Nguyen Ha Son, Sharma Abhishiek, Doan Ninh, Gelsomino Michael, Shabani Saman, Maiman Dennis

机构信息

Department of Neurosurgery, Medical College of Wisconsin , Milwaukee, WI, USA.

出版信息

Spinal Cord Ser Cases. 2016 Jan 7;2:15029. doi: 10.1038/scsandc.2015.29. eCollection 2016.

Abstract

Systemic sclerosis, or scleroderma, rarely involves the cervical spine. The disorder can induce dystrophic calcinosis that can cause spinal canal stenosis and spinal instability. An association between this pathology with acute traumatic central cord syndrome and dural erosion is rare. This case report highlights such an association and postulates the underlying mechanisms. A 57-year-old female, with a history of chronic obstructive pulmonary disease (COPD), schizophrenia and systemic sclerosis, presented with weakness in all extremities after a minor fall. Imaging demonstrated a calcified soft-tissue mass at C4/5 causing severe cord compression, cord edema spanning C3-C6, calcified soft-tissue masses at right C2/C3 facet joint and bilateral C4/5 facet joints, and significant subluxation at C4/5. Patient underwent C4-C7 laminectomies, and C3-T1 fixation and fusion. The calcinosis appeared to erode through the dura, which necessitated dura repair once the calcionsis was carefully debulked. The patient recovered well, and improved clinically with rehabilitation. Dystrophic calcinosis from underlying systemic sclerosis can cause significant chronic cervical stenosis, which may reduce the threshold for acute traumatic cervical cord syndrome under minor trauma. Moreover, dystrophic calcinosis may exhibit a propensity for dural erosion, possibly through chronic adhesions secondary to chronic inflammation. Aggressive removal of the calcinosis may breach the dura. Consequently, subtotal debulking may be reasonable if the spinal canal is adequately decompressed after laminectomies.

摘要

系统性硬化症,即硬皮病,很少累及颈椎。该疾病可引发营养不良性钙化,进而导致椎管狭窄和脊柱不稳定。这种病理情况与急性创伤性中央脊髓综合征及硬脊膜侵蚀之间的关联较为罕见。本病例报告强调了这种关联并推测了潜在机制。一名57岁女性,有慢性阻塞性肺疾病(COPD)、精神分裂症和系统性硬化症病史,在轻微跌倒后出现四肢无力。影像学检查显示C4/5水平有一钙化软组织肿块,导致严重的脊髓受压,C3 - C6节段脊髓水肿,右侧C2/C3小关节及双侧C4/5小关节处有钙化软组织肿块,且C4/5节段明显半脱位。患者接受了C4 - C7椎板切除术以及C3 - T1固定融合术。钙化似乎侵蚀了硬脊膜,在仔细切除钙化组织后需要进行硬脊膜修复。患者恢复良好,经康复治疗后临床症状改善。潜在系统性硬化症所致的营养不良性钙化可导致严重的慢性颈椎管狭窄,这可能会降低轻微创伤下急性创伤性颈脊髓综合征的发病阈值。此外,营养不良性钙化可能具有侵蚀硬脊膜的倾向,可能是通过慢性炎症继发的慢性粘连。积极切除钙化可能会穿破硬脊膜。因此,如果椎板切除术后椎管得到充分减压,次全切除钙化组织可能是合理的。

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本文引用的文献

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Systemic sclerosis at the crossroad of polyautoimmunity.系统性硬化症处于多种自身免疫性疾病的十字路口。
Autoimmun Rev. 2013 Sep;12(11):1052-7. doi: 10.1016/j.autrev.2013.05.002. Epub 2013 Jun 19.
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Systemic sclerosis: an update in 2008.
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