Migrainous headaches and transient ischaemic attacks are amongst the commonest acute neurological presentations seen in the UK. Our case highlights a rare complication of a rare disorder presenting as a mimic for these common neurological conditions.
A 68 year-old woman presented with gradual onset severe frontotemporal headache with vomiting, mild photophobia and transient aphasia. Previously, she had experienced episodes of sudden onset left arm weakness and numbness spreading to the face, associated with complete aphasia, lasting for ten minutes. Examination was unrevealing.
Contrast head MRI showed enhancing leptomeningeal disease. An IgM kappa paraprotein was detected in serum, with a lymphoplasmacytic lymphoma diagnosed on bone marrow aspirate/trephine. CSF was highly cellular containing a population of predominantly CD20+ lymphocytes, in keeping with a B-cell lymphoplasmacytic lymphoma infiltrating the leptomeninges. A diagnosis of Waldenstrom’s macroglobulinaemia (WM) presenting with Bing-Neel syndrome was made. She was started on chemoimmunotherapy with MATRix regimen.
Bing-Neel syndrome is a rare complication of WM that results from infiltration of the central nervous system, and rarely peripheral nervous system, by malignant lymphoplasmacytic cells. Neurological manifestations are diverse and protean. The diagnosis should be considered with unexplained neurological symptoms in the presence of macroglobulinaemia.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.