Lavrnić D, Vidaković A, Miletić V, Trikić R, Marinković Z, Rakocević V, Nikolić J, Wirguin I, Sadiq S A, Apostolski S
Department of Neurology, University Clinical Center, Belgrade, Serbia.
Eur Neurol. 1995;35(2):104-7. doi: 10.1159/000117102.
In a study to determine the prevalence of monoclonal gammopathy (MG) among patients with motor neuron disease (MND), 6 out of 56 (10.7%) were found to have a monoclonal paraprotein. Of these 6 patients, 4 had an IgG and 2 had an IgA paraprotein. The clinical syndromes consisted of amyotrophic lateral sclerosis in 2 patients, lower motor neuron syndrome with preserved reflexes in at least one limb in 3 patients, and motor neuropathy with multifocal conduction block in 1 patient. The presence of gammopathy appears to correlate with the absence of marked upper motor neuron involvement and with elevated CSF protein concentration. An underlying malignant disorder was ruled out in all 6 patients, and they were considered to have MG of undetermined significance (MGUS). In a control group of 121 age-matched patients with other neuroimmunological disorders, 5 patients (4.13%) had MG. Four of these had gammopathy associated with malignant myeloma, and 1 had MGUS. These results support previous reports of increased prevalence of MGUS in patients with MND and suggest that an autoimmune mechanism may play a role in the disease.
在一项确定运动神经元病（MND）患者中单克隆丙种球蛋白病（MG）患病率的研究中，56例患者中有6例（10.7%）被发现存在单克隆副蛋白。在这6例患者中，4例为IgG型，2例为IgA型副蛋白。临床综合征包括2例肌萎缩侧索硬化、3例至少有一个肢体反射保留的下运动神经元综合征以及1例伴有多灶性传导阻滞的运动神经病。丙种球蛋白病的存在似乎与明显的上运动神经元受累缺失以及脑脊液蛋白浓度升高相关。所有6例患者均排除了潜在的恶性疾病，他们被认为患有意义未明的单克隆丙种球蛋白病（MGUS）。在一个由121例年龄匹配的患有其他神经免疫性疾病的患者组成的对照组中，有5例患者（4.13%）患有MG。其中4例丙种球蛋白病与恶性骨髓瘤相关，1例患有MGUS。这些结果支持了之前关于MND患者中MGUS患病率增加的报道，并表明自身免疫机制可能在该疾病中起作用。
