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[长期血液透析患者颈椎后纵韧带肥厚所致脊髓病]

[Myelopathy caused by hypertrophy of the posterior longitudinal ligament of the cervical spine in a patient with long-term hemodialysis].

作者信息

Mizuno J, Nakagawa H, Hashizume Y

机构信息

Department of Neurological Surgery, Aichi Medical University.

出版信息

No Shinkei Geka. 1996 Jul;24(7):655-9.

PMID:8752880
Abstract

A 48-year-old female was admitted to our hospital because of progressive gait deterioration over a period of 4 months. She had been treated by hemodialysis for 14 years after bilateral nephrectomy. She originally suffered achondroplasia. Otherwise she was a healthy woman. Neurological examination showed weakness and hypesthesia below C5, accelerated deep tendon reflexes and inability in walking. T1 weighted magnetic resonance imaging revealed severe cord compression from C4 to C6 due to an epidural isointensity mass located in the ventral surface of the dura. This mass was enhanced by a contrast medium. Anterior decompression followed by an autogenous vertebral bone graft at C4/5 and C5/6 was performed. The posterior longitudinal ligament was remarkably thickened resulting in severe cord compression. Histologically this thick ligament contained amyloid deposition both in the superficial and deep layers. Ossification was not noticed in the ligament. Diagnosis of hypertrophy of the posterior longitudinal ligament (HPLL) was made. HPLL is known to be frequently associated with herniated intervertebral discs or segmental ossification of the posterior longitudinal ligament (OPLL). Myelopathy in this patient with long-term hemodialysis was aggravated by HPLL without ossification due to imbalance of systemic metabolism such as amyloidosis. The authors report a unique case of HPLL caused by amyloidosis as one of the complications of chronic hemodialysis with special emphasis on its pathophysiology and management.

摘要

一名48岁女性因4个月来进行性步态恶化入院。双侧肾切除术后她接受血液透析治疗14年。她最初患有软骨发育不全。除此之外,她是一名健康女性。神经系统检查显示C5以下肌力减弱、感觉减退、腱反射亢进及行走障碍。T1加权磁共振成像显示硬膜腹侧有一等强度硬膜外肿块,导致C4至C6脊髓严重受压。该肿块在注入造影剂后强化。行C4/5和C5/6前路减压及自体椎体骨移植术。后纵韧带明显增厚,导致脊髓严重受压。组织学检查显示该增厚韧带的浅层和深层均有淀粉样沉积。韧带中未发现骨化。诊断为后纵韧带肥厚(HPLL)。已知HPLL常与椎间盘突出或后纵韧带节段性骨化(OPLL)相关。该长期血液透析患者的脊髓病因HPLL且无骨化而加重,这是由于淀粉样变性等全身代谢失衡所致。作者报告了一例由淀粉样变性引起的HPLL独特病例,作为慢性血液透析的并发症之一,并特别强调其病理生理学和治疗方法。

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