Diffuse large B-cell lymphoma masquerading as demyelination

Patients with predominantly neurological symptoms and signs as their first manifestation of diffuse large B cell lymphoma (DLBCL) represent a diagnostic challenge. We present the clinical course, imaging findings and postmortem pathological correlates of a patient first presenting to neurology with symptoms relating to DLBCL. He died before confirmation of the diagnosis despite early suspicion of central nervous system (CNS) lymphoma and relevant investigation. The diagnosis was confirmed at postmortem by immunohistochemistry of spinal and brain lesions. MRI showing CNS lesions consistent with demyelination is common and lymphoma is rarely the cause. However, when there is high clinical suspicion, tissue diagnosis should be sought whether by skin, lymph node or brain biopsy.