|Grimm S, Chamberlain MC|
Peripheral Nerve Dysfunction Secondary to Lymphomatous Infiltration of the Nervous System by Non-Hodgkin's Lymphoma
European Association of NeuroOncology Magazine 2014; 4 (2): 65-70
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Keywords: intravascular lymphomatosis, leptomeningeal lymphoma, lymphoma, lymphomatous meningitis, neurolymphomatosis
Lymphomatous meningitis (metastasis of lymphoma cells into the cerebrospinal-fluid spaces [CSF]) and neurolymphomatosis (lymphomatous infiltration of a peripheral nerve or root) are neurologic complications of non-Hodgkin’s lymphoma (NHL) that frequently result in significant neurologic dysfunction. Leptomeningeal metastases most commonly present as cerebral dysfunction (hydrocephalus causing headache or apraxia of gait, encephalopathy, or seizures), cranial neuropathy (diplospia, facial weakness, vertigo, hearing loss, and tongue weakness), and spinal-nerve root dysfunction (incomplete cauda equina syndrome – asymmetric lower- extremity weakness, sensory loss, or incontinence). Diagnosis is made by finding leptomeningeal enhancement on magnetic resonance imaging (MRI) of the brain or spine or demonstration of lymphomatous cells by CSF cytology or flow cytometry. Treatment consists of focal radiotherapy for areas of bulky disease followed by intra-CSF chemotherapy or systemic chemotherapy. Neurolymphomatosis typically presents as a painful, sensorimotor peripheral neuropathy affecting multiple limbs in an asymmetric fashion with rapid evolution although variability in presentation can occur. Diagnosis is made by demonstration of enhancement of nerve roots on MRI of the brachial or lumbosacral plexus or peripheral nerves or by increased hyper-metabolic activity following the course of affected nerves on fluordeoxyglucose positron emission tomography (FDG-PET). Treatment of neurolymphomatosis consists of focal radiotherapy (if significant neurologic dysfunction is present) and high-dose intravenous methotrexate therapy. Standard systemic chemotherapy agents are not effective since they do not penetrate the physiologic “nerve-blood barrier”. Other disorders that must be differentiated from these entities include peripheral-nerve or nerve root compression and paraneoplastic neuropathy.