Johansen P, Leegaard O F
Clin Neuropathol. 1985 May-Jun;4(3):99-104.
Four of 132 consequtive patients with paraproteinemia and with an M-component presented exclusively with neurological symptoms. A sural nerve biopsy was performed in all patients. In one case direct immunofluorescence revealed deposition of immunoglobulin corresponding to the M-component along the perineurium. No deposition of immunoglobulin was found along the myelin sheaths and the patient's serum revealed no immunoreactivity towards normal peripheral myelin or other components of normal peripheral nerves. In a second case, deposition of immunoglobulin corresponding to the M-component was found along the myelin sheaths, and this patient's paraprotein showed immunoreactivity towards normal peripheral myelin. Two patients showed no in situ bound immunoglobulin in the nerve biopsies and their sera did not react with any component in normal peripheral nerves. The patient with deposition of immunoglobulin along the perineurium had severe demyelination and severe peripheral sensory-motor neuropathy, while the patient with immunoglobulin deposition along the myelin sheaths had a slight sensory neuropathy which had not progressed for 4 years. Based on these findings the pathogenesis of neuropathy in patients with paraproteinemia is discussed, and it is concluded that although there is evidence for immune-mediated neuropathy in the patients, the histologic, immunohistochemical and clinical data are conflicting and the exact pathogenic pathway is still unknown.
132例连续性副蛋白血症且伴有M蛋白成分的患者中，有4例仅表现为神经症状。所有患者均进行了腓肠神经活检。1例患者经直接免疫荧光检查发现，对应M蛋白成分的免疫球蛋白沿神经束膜沉积。髓鞘上未发现免疫球蛋白沉积，且患者血清对正常外周髓鞘或正常外周神经的其他成分无免疫反应性。在第2例患者中，发现对应M蛋白成分的免疫球蛋白沿髓鞘沉积，且该患者的副蛋白对正常外周髓鞘有免疫反应性。2例患者的神经活检未显示原位结合免疫球蛋白，其血清与正常外周神经的任何成分均无反应。免疫球蛋白沿神经束膜沉积的患者出现严重脱髓鞘和严重的外周感觉运动神经病变，而免疫球蛋白沿髓鞘沉积的患者有轻微感觉神经病变，且4年未进展。基于这些发现，讨论了副蛋白血症患者神经病变的发病机制，得出的结论是，虽然有证据表明这些患者存在免疫介导的神经病变，但组织学、免疫组织化学和临床数据相互矛盾，确切的致病途径仍不清楚。
