Neurolymphomatosis

Neurolymphomatosis (NL) is a rare condition in which malignant lymphocytes directly infiltrate the peripheral nervous system, including cranial nerves, spinal nerve roots, peripheral nerve trunks, and plexuses. It most commonly occurs as a manifestation of non-Hodgkin lymphoma (particularly diffuse large B-cell lymphoma) or acute lymphoblastic leukemia, though it can also arise in the context of other hematologic malignancies. Neurolymphomatosis may present as the initial manifestation of lymphoma or as a relapse of previously treated disease. The condition primarily affects the peripheral nervous system, and clinical features depend on which nerves are involved. Key symptoms include painful peripheral neuropathy (often progressive and asymmetric), cranial nerve palsies, radiculopathy, plexopathy (particularly brachial or lumbosacral), and progressive weakness or sensory loss in affected limbs. Pain is a particularly prominent and early feature, often preceding other neurological deficits. Some patients develop cauda equina syndrome. Because symptoms can mimic other neuropathies, diagnosis is frequently delayed. Diagnosis relies on a combination of clinical suspicion, nerve biopsy (when feasible), cerebrospinal fluid analysis, and advanced imaging. PET-CT and MRI with gadolinium enhancement of affected nerves are important diagnostic tools, with FDG-PET showing increased uptake along involved nerves. Treatment typically involves systemic chemotherapy directed at the underlying lymphoma, often combined with intrathecal chemotherapy and/or radiation therapy to affected nerve segments. Despite treatment, the prognosis is generally poor, with median survival often reported as less than 10 months, though outcomes vary depending on the underlying malignancy and response to therapy. Early diagnosis and prompt initiation of treatment may improve outcomes.

Common questions about Neurolymphomatosis