Lagarde S, Tabouret E, Matta M, Franques J, Attarian S, Pouget J, Maues De Paula A, Figarella-Branger D, Dory-Lautrec P, Chinot O, Barrié M
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
J Neurol Sci. 2014 Jul 15;342(1-2):178-81. doi: 10.1016/j.jns.2014.04.030. Epub 2014 May 1.
To discuss the therapeutic approach for primary neurolymphomatosis.
We report all primary neurolymphomatosis cases referred to our institution, with descriptions of clinical, radiological, electrophysiological, histological features and long-term follow-up. We treated all patients with a combination of high-dose methotrexate and alkylating agents.
Five patients were diagnosed with histologically confirmed primary neurolymphomatosis. The majority of them presented with painful asymmetric sensory-motor neuropathy. Magnetic resonance imaging was abnormal in 4 of 5 patients, as shown with gadolinium enhancements. Electroneuromyography revealed denervation in all 4 cases with contributive examinations. All our patients received a chemotherapy combination of high-dose methotrexate and alkylating agent. Median progression-free survival was 8 months (2 complete responses and 2 partial responses), and overall survival was 24 months.
Primary neurolymphomatosis is rare and polymorphic; it represents a difficult diagnosis of neuropathy. In our cohort, treatment with a chemotherapy combination with high-dose methotrexate showed encouraging results.
探讨原发性神经淋巴瘤的治疗方法。
我们报告了所有转诊至我院的原发性神经淋巴瘤病例，描述了其临床、放射学、电生理、组织学特征及长期随访情况。我们采用大剂量甲氨蝶呤和烷化剂联合治疗所有患者。
5例患者经组织学确诊为原发性神经淋巴瘤。大多数患者表现为疼痛性不对称感觉运动性神经病变。5例患者中有4例磁共振成像异常，钆增强显示。4例进行相关检查的患者神经电生理检查均显示失神经改变。所有患者均接受了大剂量甲氨蝶呤和烷化剂联合化疗。无进展生存期的中位数为8个月（2例完全缓解，2例部分缓解），总生存期为24个月。
原发性神经淋巴瘤罕见且具有多态性；它是一种难以诊断的神经病变。在我们的队列中，大剂量甲氨蝶呤联合化疗显示出令人鼓舞的结果。
