Schold S C, Cho E S, Somasundaram M, Posner J B
Ann Neurol. 1979 Mar;5(3):271-87. doi: 10.1002/ana.410050310.
Ten patients developed a subacute lower motor neuron syndrome as a remote effect of Hodgkin's disease or other lymphoma. The illness usually followed a benign course independent of the activity of the underlying neoplasm. Seven of the patients improved spontaneously, and 3 became neurologically normal. Two patients died of intercurrent infections related to immunosuppression. Neuropathological examination of these 2 patients and 3 previously reported cases showed prominent neuronal degeneration restricted to the anterior horns of the spinal cord and mild posterior column demyelination. Demyelination was also present in the anterior roots of our autopsied patients and was accompanied by large, hyperchromatic Schwann cells. The cause of the illness is obscure, but both radiation therapy and opportunistic infection may be contributing factors. Attempts at virus isolation have been unsuccessful. The syndrome should be distinguished from the more common direct effects of lymphoma on the nervous system, since its identification spares the patient additional, potentially harmful therapy.
10名患者出现亚急性下运动神经元综合征,这是霍奇金病或其他淋巴瘤的远期效应。该疾病通常呈良性病程,与潜在肿瘤的活动无关。7名患者自发改善,3名患者神经功能恢复正常。2名患者死于与免疫抑制相关的并发感染。对这2名患者以及3例先前报道病例的神经病理学检查显示,显著的神经元变性局限于脊髓前角,伴有轻度后柱脱髓鞘。在我们尸检患者的前根中也存在脱髓鞘,并且伴有大的、染色质过多的施万细胞。该疾病的病因尚不清楚,但放射治疗和机会性感染可能都是促成因素。病毒分离的尝试未成功。该综合征应与淋巴瘤对神经系统更常见的直接效应相区分,因为识别该综合征可使患者避免接受额外的、可能有害的治疗。