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Neurolymphomatosis as primary presentation of extra-nodal NK/T-cell lymphoma, nasal type

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Abstract

Neurolymphomatosis (NL) describes an infiltration of cranial and peripheral nerves by lymphoma cells, most frequently in non-Hodgkin B-cell lymphoma. This clinical entity is rare and poses a challenging diagnosis. We describe a case of a 64-year-old female patient with NL associated with extra-nodal NK/T-cell lymphoma (ENKTL), nasal type, presenting as a painful progressive mononeuropathy multiplex with an oral cavity lesion. ENKTL is usually associated with Epstein-Barr virus (EBV) infection and rarely affects the central and peripheral nervous system. Lumbar puncture, magnetic resonance imaging (MRI), nerve biopsy, and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) help to establish the diagnosis. Thereby, NL should be considered in the differential diagnosis of painful progressive multiple neuropathies, even in patients without previous history of cancer.

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Correspondence to Maria Inês Silva.

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Silva, M.I., Santos, P., Viegas, D. et al. Neurolymphomatosis as primary presentation of extra-nodal NK/T-cell lymphoma, nasal type. Neurol Sci (2024). https://doi.org/10.1007/s10072-024-07445-4

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