Primary neurolymphomatosis diagnosis and treatment: A retrospective study

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...

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Bibliographic Details
Published in:Journal of the neurological sciences 2014-07, Vol.342 (1), p.178-181
Main Authors: 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
Format: Article
Language:English
Subjects:
Aged
Alkylating Agents - therapeutic use
Antimetabolites, Antineoplastic - therapeutic use
Cancer
Chemotherapy
Cognitive science
Disease-Free Survival
Electromyography
Electroneuromyography
Fatal Outcome
Female
Humans
Life Sciences
Lymphoma
Lymphoma, B-Cell - diagnosis
Lymphoma, B-Cell - drug therapy
Lymphoma, B-Cell - pathology
Magnetic Resonance Imaging
Male
Methotrexate - therapeutic use
Middle Aged
MRI
Neuroimaging
Neurology
Neurolymphomatosis
Neuroscience
Peripheral nervous system
Peripheral Nervous System Neoplasms - diagnosis
Peripheral Nervous System Neoplasms - drug therapy
Peripheral Nervous System Neoplasms - pathology
Retrospective Studies
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Summary: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.
ISSN:0022-510X
1878-5883
DOI:10.1016/j.jns.2014.04.030