S Lagarde1, E Tabouret2, M Matta2, J Franques3, S Attarian3, J Pouget3, A Maues De Paula4, D Figarella-Branger4, P Dory-Lautrec5, O Chinot2, M Barrié2. 1. Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France. Electronic address: stanislas.lagarde@ap-hm.fr. 2. Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France. 3. Centre de Référence des Maladies Neuromusculaires et de la SLA, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France. 4. Service d'anatomie pathologique et de neuropathologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France. 5. Service de neuroradiologie diagnostique et interventionnelle, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
Abstract
BACKGROUND: To discuss the therapeutic approach for primary neurolymphomatosis. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND: To discuss the therapeutic approach for primary neurolymphomatosis. METHODS: 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. RESULTS: 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. CONCLUSIONS: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.
Authors: Arushi Khurana; Mattia Novo; Grzegorz S Nowakowski; Kay M Ristow; Robert J Spinner; Christopher H Hunt; Rebecca L King; Daniel H Lachance; Thomas M Habermann; Ivana N Micallef; Patrick B Johnston Journal: Blood Adv Date: 2021-03-09
Authors: Hyung Jun Park; Ha Young Shin; Se Hoon Kim; Ha Neul Jeong; Young Chul Choi; Bum Chun Suh; Kee Duk Park; Seung Min Kim Journal: J Clin Neurol Date: 2018-01 Impact factor: 3.077