Cauda equina syndrome secondary to intravascular lymphoma

Abstract

Intravascular lymphoma (IVL) is an extranodal B-cell lymphoma that involves the lumina of small and medium size vessels.1 Neurologic presentations are varied and include encephalopathy, myelopathy, and polyradiculoneuropathy, among other features.1,2 Cauda equina syndrome (CES) with IVL has been identified late, either at autopsy or after complications such as flaccid paraplegia.2⇓–4 Early diagnosis and treatment before disease dissemination is difficult but increases progression-free survival and remission.1 We describe a patient with isolated CES and subtle non-nodular MRI nerve root enhancement, who was identified to harbor IVL by cauda equina biopsy. A 70-year-old man presented with a 4-week history of perineal, patchy lower limb numbness and urinary retention. Neurologic examination disclosed absent ankle reflexes and sensory alterations in the back of his thighs and perineal region in an S2-S5 distribution. He had an absent anal wink. Normal investigations included complete blood count, electrolytes, creatinine, urea, thyroid-stimulating hormone, B12, hepatic enzymes, serology for hepatitis B and C and HIV 1 and 2, and paraneoplastic antibodies. Lactate dehydrogenase was elevated (460 U/L: normal 100–235). Serum protein electrophoresis identified an immunoglobulin G (IgG) κ monoclonal protein (1.3 g/L). CSF had an …