Neuropathic pain correlates with myelinated fibre loss and cytokine profile in POEMS syndrome
Neuropathic pain correlates with myelinated fibre loss and cytokine profile in POEMS syndrome
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DOI：
10.1136/jnnp.2007.135681
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发表时间：
2008-10-01
影响因子：
11
通讯作者：
Sobue, G.
中科院分区：
文献类型：
--
作者：
Koike, H.;Iijima, M.;Sobue, G.
目的：为探讨POEMS综合征的临床病理特征，对22例POEMS综合征患者的临床资料进行分析，并与腓肠神经病理组织学及血清细胞因子水平进行相关性研究。使用完整的横腓肠神经横截面对总神经纤维群进行评估（不包括由于束内水肿导致的神经内膜区域的显著扩大），结果显示有髓纤维，尤其是小的有髓纤维减少，而无髓纤维被保留。未压实的髓鞘板层和节段性脱髓鞘更常见于小的，而不是大的，有髓纤维。痛觉过敏的存在与腓肠神经中感觉神经动作电位的降低电生理学相关（p < 0.05），并且与有髓纤维损失组织病理学相关（p < 0.01）。痛觉过敏患者血清促炎细胞因子（白细胞介素-1b、白细胞介素-6和肿瘤坏死因子-α）水平显著升高，但其可溶性受体水平无显著升高（p < 0.05-0.01）。结论：POEMS综合征患者的痛觉过敏与有髓纤维减少密切相关，但与无髓纤维减少无关。促炎细胞因子的升高也与痛觉过敏相关。POEMS综合征中的疼痛症状可能是由于缺乏抑制性有髓A纤维沿着细胞因子致敏而由保存良好的无髓C纤维产生的。
Objective: To reveal characteristic clinicopathological correlates of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome.Methods: The clinical features of 22 patients with POEMS syndrome were investigated and correlated with the histopathological features of sural nerves and serum cytokine profiles.Results: More than half of the patients complained of pain in the lower extremities, which is closely related to hyperalgesia. Assessment of the total nerve fibre population using complete transverse sural nerve cross-sections, excluding the marked enlargement of endoneurial areas due to intrafascicular oedema, showed that myelinated fibres, especially small myelinated fibres, were reduced, whereas unmyelinated fibres were preserved. Uncompacted myelin lamellae and segmental demyelination were seen more frequently in the small, rather than the large, myelinated fibres. The presence of hyperalgesia was electrophysiologically associated with a reduction of sensory nerve action potentials in the sural nerve (p < 0.05) and histopathologically associated with myelinated fibre loss (p < 0.01). Serum levels of proinflammatory cytokines (interleukin-1b, interleukin-6 and tumour necrosis factor-alpha), but not their soluble receptors, were significantly elevated in patients with hyperalgesia (p < 0.05-0.01). Conclusions: Hyperalgesia seen in patients with POEMS syndrome is closely related with a reduction in the myelinated, but not unmyelinated, fibre population. Elevation of proinflammatory cytokines is also correlated with hyperalgesia. The painful symptoms in POEMS syndrome may be generated by well-preserved unmyelinated C-fibres due to the lack of inhibitory myelinated A-fibres, along with cytokine sensitisation.