Last revised by Francis Deng on 14 May 2022

Neurolymphomatosis is a rare cause of neuropathy due to infiltration of the peripheral nervous system by a hematologic malignancy, in the great majority a B-cell non-Hodgkin lymphoma.

It should be differentiated from non-tumor neuropathies associated with lymphoma, such as irradiation, chemotherapy, or paraneoplastic effects.

Neurolymphomatosis and CNS lymphoma overlap when there is cranial neuropathy or cauda equina involvement as evidenced by imaging and positive CSF cytology. However, neurolymphomatosis is sometimes strictly limited to cases where there is evidence of extradural infiltration of the affected nerves and/or neural plexi 4, as opposed to merely intradural/leptomeningeal disease affecting the nerve roots.

Peripheral nervous system infiltration by malignant cells in the setting of leukemia has sometimes been grouped into the entity of neurolymphomatosis 4, while others prefer the more specific term neuroleukemiosis.

Neurolymphomatosis seems to be the least common neurologic manifestation of lymphoma 3. The typical age of presentation is in the 50s and 60s 4.

Neurolymphomatosis more often appears as an extranodal site of an existing diagnosis of systemic lymphoma, and less commonly as the first presentation of lymphoma 4.

The neuropathy may be painful (more common) or painless, and affect sensorimotor functions (most often) or be purely sensory or purely motor 4. The affected structures may include peripheral nerves (neuropathy), spinal nerve roots (radiculopathy), neural plexus (brachial or lumbosacral plexopathy), or cranial nerves (cranial neuropathy) 4.

Brain or spinal MRI can spot a nerve or root enlargement and/or enhancement 1,3, sometimes with a nodular thickening.

FDG PET shows radiotracer uptake along the affected nerves.

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Cases and figures

  • Case 1: Neurolymphomatosis
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