Nobile-Orazio E, Casellato C, Di Troia A
Giorgio Spagnol Service of Clinical Neuroimmunology, Department of Neurological Sciences, Dino Ferrari Center, Milan University, Italy.
Rev Neurol (Paris). 2002 Oct;158(10 Pt 1):979-87.
Neuropathy has been frequently reported in patients with monoclonal gammopathy, particularly those with monoclonal gammopathy of undetermined significance (MGUS). While the neuropathy associated with IgM-MGUS is well characterized and is often associated with a reactivity of the monoclonal protein with neural antigens, the relationship between the neuropathy and IgG and IgA MGUS is less clear. We review here the clinical, electrophysiological and pathogenetic features of neuropathies associated with IgG and IgA M-proteins in order to determine whether they represent distinct clinical entities and, most importantly, whether the finding of an IgG or IgA monoclonal gammopathy in a patient with neuropathy should led to different diagnostic or therapeutical approaches. This review will mainly focus on neuropathies associated with MGUS since the disclosure of a malignant monoclonal gammopathy, including multiple or osteosclerotic myeloma, lymphoma or primary amyloidosis, in a patient with neuropathy usually divert the therapeutical decisions to the hematologist for an appropriate therapy of the underlying life threatening condition.
神经病在单克隆丙种球蛋白病患者中经常被报道,尤其是那些意义未明的单克隆丙种球蛋白病(MGUS)患者。虽然与IgM-MGUS相关的神经病特征明确,且通常与单克隆蛋白对神经抗原的反应性有关,但神经病与IgG和IgA MGUS之间的关系尚不清楚。我们在此回顾与IgG和IgA M蛋白相关的神经病的临床、电生理和发病机制特征,以确定它们是否代表不同的临床实体,最重要的是,在患有神经病的患者中发现IgG或IgA单克隆丙种球蛋白病是否应导致不同的诊断或治疗方法。本综述将主要关注与MGUS相关的神经病,因为在患有神经病的患者中发现恶性单克隆丙种球蛋白病,包括多发性或骨硬化性骨髓瘤、淋巴瘤或原发性淀粉样变性,通常会将治疗决策转交给血液科医生,以便对潜在的危及生命的疾病进行适当治疗。