A Unique Case of Intravascular Lymphoma Mimicking Encephalomyeloradiculoneuropathy

Angiotropic lymphoma is a rare large B-cell lymphoma involving the intravascular compartment without local tissue or vessel wall infiltration.

A 48-year-old male presented with 8 months of progressive paraparesis and bowel and bladder incontinence. Initial cerebrospinal fluid analysis showed high protein, lymphocytic pleocytosis, high IgG synthesis, but no oligoclonal bands. Brain imaging at presentation was unrevealing. Electromyography revealed evidence of bilateral lumbosacral radiculoplexopathy [R>L]. A trial of intravenous solumedrol resulted in significant improvement in weakness. He had multiple hospital admissions for worsening lower extremity weakness, altered mental status, and new-onset upper extremity weakness. Magnetic resonance imaging of the spine revealed abnormal hyperintense T2 signal at T7-T8 level. Visual-evoked potentials revealed significant slowing of conduction in both optic nerves. Repeat electromyography showed a moderate to severe motor axonal polyneuropathy with secondary demyelination in the upper and lower extremities. A working diagnosis of encephalomyeloradiculoneuritis was made because of signs of polyradiculopathy, peripheral neuropathy and myelopathy. Patient had 3 trials of intravenous solumedrol with transient improvement in symptoms. The magnetic resonance imaging brain on his sixth hospital admission revealed multiple areas of restricted diffusion throughout the brain parenchyma. The patient underwent a right frontal lobe biopsy, which showed large CD-20 neoplastic lymphocytes within small arteries, veins, and capillaries with no extension to surrounding brain parenchyma. The findings were consistent with intravascular large B-cell lymphoma.

Angiotropic lymphoma is a rare disease with frequent involvement of central nervous system and skin that can present with neurological involvement of both the peripheral and central nervous system.