Younger D S, Rowland L P, Latov N, Sherman W, Pesce M, Lange D J, Trojaborg W, Miller J R, Lovelace R E, Hays A P
Department of Neurology, Presbyterian Hospital, Columbia University, College of Physicians and Surgeons, New York, NY.
Neurology. 1990 Apr;40(4):595-9. doi: 10.1212/wnl.40.4.595.
From 1984 to 1988, 11 of 120 patients (9%) with motor neuron disease (MND) had paraproteins detected by serum immunofixation electrophoresis (IFE), compared with 4 (3%) by cellulose acetate gels: 1 patient had progressive spinal muscular atrophy, 5 patients had amyotrophic lateral sclerosis (ALS), and 5 patients had ALS with probable upper motor neuron signs. Four of 5 patients (80%) with cerebrospinal fluid (CSF) protein content above 75 mg/dl had paraproteins, as did 6 of 30 with values above 50 mg/dl. Four of 14 patients with cerebrospinal oligoclonal bands (OCB) also had paraproteins. Two patients with ALS, CSF protein content above 75 mg/dl, and paraproteinemia had lymphoma. We conclude the following about patients with MND: high CSF protein content (especially above 75 mg/dl) or CSF OCB makes paraproteinemia more likely; some of these patients may have lymphoma; there is an inordinately high occurrence of paraproteinemia in MND; and IFE on agarose is more sensitive than electrophoresis on cellulose acetate in detecting paraproteins. Syndromes of paraproteinemia and high CSF protein are not restricted to the lower motor neuron but qualify as "ALS" with coexisting upper motor neuron signs.
1984年至1988年期间，120例运动神经元病（MND）患者中有11例（9%）通过血清免疫固定电泳（IFE）检测到副蛋白，相比之下，通过醋酸纤维素凝胶检测到4例（3%）：1例患有进行性脊髓性肌萎缩，5例患有肌萎缩侧索硬化症（ALS），5例患有可能伴有上运动神经元体征的ALS。5例脑脊液（CSF）蛋白含量高于75mg/dl的患者中有4例（80%）检测到副蛋白，30例蛋白含量高于50mg/dl的患者中有6例也是如此。14例脑脊液寡克隆带（OCB）患者中有4例也检测到副蛋白。2例患有ALS、脑脊液蛋白含量高于75mg/dl且存在副蛋白血症的患者患有淋巴瘤。我们就MND患者得出以下结论：脑脊液蛋白含量高（尤其是高于75mg/dl）或脑脊液OCB会使副蛋白血症更有可能发生；这些患者中的一些可能患有淋巴瘤；MND中副蛋白血症的发生率极高；在检测副蛋白方面，琼脂糖上的IFE比醋酸纤维素电泳更敏感。副蛋白血症和脑脊液蛋白含量高的综合征不仅限于下运动神经元，在伴有上运动神经元体征时也符合“ALS”的诊断。
