Rahmani M, Birouk N, Amarti A, Loukili Idrissi A, Marnissi F, Belaidi H, El Alaoui Faris M, Benchekroun S, Ouazzani R
Service de Neurologie A et de Neuropsychologie, hôpital des spécialités, Rabat, Maroc.
Rev Neurol (Paris). 2007 Apr;163(4):462-70. doi: 10.1016/s0035-3787(07)90422-1.
Lymphoma occasionally affects the peripheral nervous system. Neuropathy usually appears in patients with known lymphoma but rarely represents the initial manifestation of underlying malignancy. We report a case in which mononeuritis multiplex (MM) was the dominant feature in the clinical presentation of a peripheral T-cell non-Hodgkin lymphoma (NHL).
A 32-year-old man suffered from an asymmetric progressive sensory-motor peripheral neuropathy. The left peroneal nerve was affected first, then the left median nerve after one month, followed by the left trigeminal nerve ten months later. The electrophysiological study confirmed the diagnosis of axonal sensory-motor MM. Mediastinal adenopathies, splenomegaly, pancytopenia and inflammatory syndrome were also found. An osteo-medullary biopsy showed a T-cell NHL. Nerve biopsy study found an inflammatory lymphoid infiltration without malignant cell supporting the hypothesis of an inflammatory pathogenic process. Chemotherapy including cyclophosphamide, hydralazine, vincristine and prednisone were administered monthly during 8 months. No improvement was obtained.
It must be emphasised that this case is an uncommon one. On the one hand, NHL is rarely associated with MM and on the other hand, it can exceptionally be revealed by a MM. We were able to find 30 reported cases of distal neuropathy revealing a NHL including, 8 mononeuritis simplex, 9 MM and 13 polyneuropathies. Polyradiculoneuritis cases were excluded from this study because the neuropathy is usually caused by a meningeal infiltration. The neuropathy was in the majority of the cases chronic and axonal. The lymphoma was more often B-cell than T-cell. The B-cell lymphoma was frequently associated with a poor prognosis. All mechanisms were present with a predominance of neurolymphomatosis.
淋巴瘤偶尔会累及周围神经系统。神经病变通常出现在已知患有淋巴瘤的患者中，但很少作为潜在恶性肿瘤的初始表现。我们报告一例以多发性单神经炎（MM）为主要特征的外周T细胞非霍奇金淋巴瘤（NHL）临床病例。
一名32岁男性患有不对称性进行性感觉运动性周围神经病变。左侧腓总神经首先受累，一个月后左侧正中神经受累，十个月后左侧三叉神经受累。电生理研究证实为轴索性感觉运动性MM。还发现纵隔淋巴结肿大、脾肿大、全血细胞减少和炎症综合征。骨髓活检显示为T细胞NHL。神经活检研究发现炎症性淋巴细胞浸润，但无恶性细胞，支持炎症致病过程的假说。在8个月期间每月给予包括环磷酰胺、肼苯哒嗪、长春新碱和泼尼松在内的化疗。未取得改善。
必须强调的是，该病例并不常见。一方面，NHL很少与MM相关，另一方面，它极少由MM首发。我们能够找到30例报告的以NHL首发的远端神经病变病例，包括8例单神经炎、9例MM和13例多发性神经病。本研究排除了多神经根神经炎病例，因为该神经病变通常由脑膜浸润引起。在大多数病例中，神经病变为慢性且呈轴索性。淋巴瘤更多为B细胞型而非T细胞型。B细胞淋巴瘤常与预后不良相关。所有机制均存在，以神经淋巴瘤病为主。
