Lymphomatous Meningitis in Primary Central Nervous System Lymphoma

Marc C. Chamberlain, M.D.

Disclosures

Neurosurg Focus. 2006;21(5) 

In This Article

Clinical Features of LM

Lymphomatous meningitis classically presents with pleomorphic clinical manifestations encompassing symptoms and signs in the following three domains of neurological function: 1) the cerebral hemispheres; 2) the cranial nerves; and 3) the spinal cord and roots. Signs on examination generally exceed the symptoms reported by the patient.[2,11,36,69,71]

The most common manifestations of cerebral hemisphere dysfunction are headache and mental status changes. Other signs include confusion, dementia, seizures, and hemiparesis. These findings often overlap with signs of parenchymal primary CNS lymphomas and therefore the clinical distinction between parenchymal and CSF compartment disease can be challenging. Diplopia is the most common symptom of cranial nerve dysfunction, with the sixth cranial nerve being the most frequently affected, followed by the third and fourth cranial nerves. Trigeminal sensory or motor loss, cochlear dysfunction, and optic neuropathy are also common findings. Spinal signs and symptoms include weakness (lower extremities more often than upper), dermatomal or segmental sensory loss, and pain in the neck, back, or following radicular patterns. Nuchal rigidity is only present in 15% of cases.[2,11,36,69,71]

A high index of suspicion is required to make the diagnosis of LM. The finding of multifocal neuraxis disease in a patient with primary CNS lymphoma is strongly suggestive of LM, but it is also common for patients with LM to present with isolated syndromes such as symptoms of raised intracranial pressure, cauda equina syndrome, or cranial neuropathy.

New neurological signs and symptoms may represent progression of LM but must be distinguished from the manifestations of parenchymal disease, from side effects of chemotherapy or radiotherapy, and, rarely, from paraneoplastic syndromes.

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