Gherardi R, Zuber M, Viard J P
Département de Pathologie (Neuropathologie), Hôpital Henri Mondor, Créteil.
Rev Neurol (Paris). 1988;144(6-7):391-408.
Monoclonal gammopathies are frequently associated with peripheral neuropathies of which clinical, electrophysiological, pathological and possibly pathogenetical aspects are heterogeneous. Nevertheless some clinico-biological entities, which account for the majority of cases, have been recently recognized: 1) The IgM neuropathy is a chronic demyelinating sensori-motor polyneuropathy with tremor and ataxia as prominent features. It can be either associated with MGUS or Waldenstrom macroglobulinemia. The light chain of the gammopathy is kappa in a majority of cases. Numerous reports have demonstrated specific antibody activities supported by the M-protein and directed against various peripheral nerve antigens, usually myelin components such as the myelin associated glycoprotein (MAG). The ultrastructural evidence of widely spaced myelin is suggestive of the diagnosis but is not consistent. Treatment directed towards the gammopathy is occasionally associated with improvement of the symptoms. 2) The neuropathy of the osteosclerotic myelomas and solitary plasmacytomas present as a chronic sensori-motor polyradiculoneuropathy with conspicuous demyelination and may be associated with one or more of the systemic clinical features of the Crow-Fukase or POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes). The POEMS syndrome may also be associated with "benign" monoclonal or even polyclonal dysproteinemias. The M-proteins are almost all IgG or IgA with lambda light chains. There are some relations between POEMS syndrome and Castleman's disease. The pathogenesis of both disorders remains obscure. Treatment is most favorable in case of solitary plasmacytomas, which may be completely removed. 3) The neuropathy observed in patients with primary AL amyloidosis or amylosis associated with malignant plasma-cell dyscrasias is rare. Sensory deficit and autonomic dysfunction are related to a prominent involvement of small myelinated and unmyelinated fibers. A clinical and/or electro-physiological carpal tunnel syndrome is frequent. In a majority of cases the light chain of the M-protein is lambda. Amyloid deposits are observed on nerve biopsy. Treatment is inefficient. 4) The neuropathy associated with cryoglobulinemias may be asymmetric, painful, cryosensitive and associated with cutaneous purpura and neuromuscular vasculitis. In fact, in a majority of cases the symptoms are less suggestive raising the problem of an incidental laboratory finding. 5) A motoneuron disease-like syndrome may develop in patients with various types of monoclonal gammopathies.(ABSTRACT TRUNCATED AT 400 WORDS)
单克隆丙种球蛋白病常与周围神经病相关，其临床、电生理、病理以及可能的发病机制方面均具有异质性。然而，最近已识别出一些占大多数病例的临床生物学实体：1）IgM 神经病是一种慢性脱髓鞘感觉运动性多神经病，以震颤和共济失调为突出特征。它可与意义未明的单克隆丙种球蛋白病（MGUS）或华氏巨球蛋白血症相关。在大多数病例中，丙种球蛋白病的轻链为κ链。大量报告显示，M 蛋白支持的针对各种周围神经抗原（通常是髓鞘成分，如髓鞘相关糖蛋白（MAG））的特异性抗体活性。广泛间隔的髓鞘超微结构证据提示诊断，但并不一致。针对丙种球蛋白病的治疗偶尔会使症状改善。2）骨硬化性骨髓瘤和孤立性浆细胞瘤的神经病表现为慢性感觉运动性多神经根神经病，伴有明显的脱髓鞘，可能与 Crow-Fukase 或 POEMS 综合征（多发性神经病、器官肿大、内分泌病、M 蛋白、皮肤改变）的一种或多种全身临床特征相关。POEMS 综合征也可能与“良性”单克隆甚至多克隆性蛋白异常血症相关。M 蛋白几乎均为带有λ轻链的 IgG 或 IgA。POEMS 综合征与 Castleman 病之间存在一些关联。两种疾病的发病机制仍不清楚。对于孤立性浆细胞瘤，治疗最为有利，因为其可能被完全切除。3）原发性 AL 淀粉样变性或与恶性浆细胞增生异常相关的淀粉样变性患者中观察到的神经病较为罕见。感觉缺陷和自主神经功能障碍与有髓和无髓小纤维的显著受累有关。临床和/或电生理腕管综合征很常见。在大多数病例中，M 蛋白的轻链为λ链。神经活检可见淀粉样沉积物。治疗无效。4）与冷球蛋白血症相关的神经病可能不对称、疼痛、对冷敏感，并伴有皮肤紫癜和神经肌肉血管炎。事实上，在大多数病例中，症状提示性较差，这就引发了偶然实验室检查发现的问题。5）在患有各种类型单克隆丙种球蛋白病的患者中可能会出现运动神经元病样综合征。（摘要截选至 400 字）
