Primary neurolymphomatosis diagnosis and treatment: A retrospective study


  • Lagarde Stanislas
  • Tabouret E.
  • Matta M.
  • Franques J.
  • Attarian S.
  • Pouget J.
  • Maues de Paula A.
  • Figarella-Branger D.
  • Dory-Lautrec P.
  • Chinot O.
  • Barrié M.


  • MRI
  • Electroneuromyography
  • Peripheral nervous system
  • Chemotherapy
  • Lymphoma
  • Neurolymphomatosis

document type



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.

more information