Younger D S, Rowland L P, Latov N, Hays A P, Lange D J, Sherman W, Inghirami G, Pesce M A, Knowles D M, Powers J
Department of Neurology, Eleanor and Lou Gehrig MDA/ALS Center, New York, NY.
Ann Neurol. 1991 Jan;29(1):78-86. doi: 10.1002/ana.410290114.
We studied 9 patients with motor neuron disease and lymphoma. The following several observations have not been recognized in the past: (1) Motor neuron syndromes are associated with either Hodgkin's disease or non-Hodgkin's lymphoma. (2) The syndromes are not restricted to lower motor neuron disorders; 8 of 9 patients had definite or probable upper motor neuron signs as well, qualifying for the diagnosis of amyotrophic lateral sclerosis. Corticospinal tracts were affected in both postmortem examinations. (3) The combination of motor neuron disease and lymphoma is often accompanied by paraproteinemia (3 of 7 patients studied), increased cerebrospinal fluid protein content (6 of 9 patients), and cerebrospinal fluid oligoclonal bands (3 of 9 patients). (4) In 2 patients, asymptomatic non-Hodgkin's lymphoma was found only because the discovery of paraproteinemia gave impetus to examine the bone marrow. (5) Patients with both upper and lower motor neuron signs (amyotrophic lateral sclerosis) may show physiological evidence of conduction block in peripheral nerves or autopsy abnormalities in peripheral nerves. The cause of this syndrome is not known. Both lymphoma and motor neuron disease could have a common cause, possibly a retroviral infection. The frequency of paraproteinemia suggests that an immunological disorder may play a role in the pathogenesis of the neurological disorder.
我们研究了9例运动神经元病合并淋巴瘤的患者。以下几点观察结果在过去未被认识到：（1）运动神经元综合征与霍奇金病或非霍奇金淋巴瘤相关。（2）这些综合征不限于下运动神经元疾病；9例患者中有8例也有明确或可能的上运动神经元体征，符合肌萎缩侧索硬化的诊断标准。在两次尸检中皮质脊髓束均受累。（3）运动神经元病与淋巴瘤的组合常伴有副蛋白血症（7例研究患者中有3例）、脑脊液蛋白含量增加（9例患者中有6例）和脑脊液寡克隆带（9例患者中有3例）。（4）在2例患者中，仅因副蛋白血症的发现促使检查骨髓才发现无症状的非霍奇金淋巴瘤。（5）既有上运动神经元体征又有下运动神经元体征的患者（肌萎缩侧索硬化）可能显示周围神经传导阻滞的生理学证据或周围神经尸检异常。该综合征的病因尚不清楚。淋巴瘤和运动神经元病可能有共同的病因，可能是逆转录病毒感染。副蛋白血症的发生率表明免疫紊乱可能在神经疾病的发病机制中起作用。
