Encephalomyeloradiculoneuropathy Revealing a Rare Case of Intravascular Large B-Cell Lymphoma

Intravascular large B cell lymphoma (IVLBCL) is a rare form of extranodal non-Hodgkin's lymphoma, usually of B-cell lineage. Several organs are affected, most commonly the skin and the nervous system. We report a case of a 52-year-old man, with no medical history admitted with a five-month history of back pain with lower extremity numbness and tingling evolved to weakness associated with urinary retention, constipation and abdominal pain. Spinal magnetic resonance imaging (MRI) showed a gadolinium-enhancing lesion in the conus medullaris (CM). Electromyography (EMG) and nerve conduction velocity (NCV) test was consistent with demyelinating polyradiculoneuropathy in lower limbs. Slight clinical improvement with corticosteroids was observed. Three months after discharge, he presented a generalized tonic-clonic seizure. Cerebral MRI showed patchy lesions in the subcortical white matter with infiltration of the internal table of the skull with elevated serum lactate dehydrogenase (LDH). Calvarial biopsy revealed an intravascular large B-cell lymphoma. Treatment with cyclophosphamide and high-dose corticosteroids was initiated but the patient developed impaired consciousness and died of respiratory and circulatory failure six weeks after his readmission. Intravascular large B cell lymphoma should be considered in patients with a rapidly progressive severe encephalomyeloradiculoneuropathy. A biopsy of involved organs including the brain should not be delayed when IVLBCL is suspected, to initiate prompt systemic therapy.