Article Text

PDF
151
PERIPHERAL NERVE BING-NEEL SYNDROME
  1. Aisling Carr1,
  2. Shirley D'Sa2,
  3. A Arasaretnam3,
  4. K Boyd3,
  5. R Johnston3,
  6. Zane Jaunmuktane1,
  7. Sebastian Brandner1,
  8. T Marafioti2,
  9. Martin Koltzenburg1,
  10. Mike Lunn1
  1. 1National Hospital for Neurology and Neurosurgery
  2. 2UCLH, London
  3. 3Department of Haematology, Brighton

Abstract

Background Bing-Neel syndrome (BNS) is a rare manifestation of Waldenstrom's Macroglobulinaemia (WM) due to direct infiltration of the central nervous system. Isolated peripheral nerve infiltration has not been described but as the blood-nerve-barrier has similar characteristics to the blood-brain-barrier, CNS-penetrating chemotherapy would be required.

Case A 43 year-old man was diagnosed with WM after presenting with constitutional symptoms; IgMk paraprotein (34.6 g/L) and lymphoplasmacytoid cells in the bone marrow. He received R-CVP (Rituximab, Cyclophosphamide, Vincristine, and Prednisolone) with good partial haematological response and minimal distal sensory vincristine-related neuropathy. Ten months later he developed a rapidly progressive, symmetric sensorimotor deficit in all four limbs with impairment of walking and hand clumsiness, MRC sum score=61/70. Electrophysiology showed a non-length dependant axonal neuropathy with patchy slowing. Sural nerve biopsy revealed a patchy infiltrate WM cells. He tolerated 4 cycles of blood-nerve-barrier penetrating multi-agent chemotherapy, IDARAM (idarubicin, dexamethasone, cytosine arabinoside, methotrexate, cytosine arabinoside plus intrathecal methotrexate). A marked improvement was seen during treatment and by 3 months later MRC score was 70/70. No electrophysiological difference was detectable at this stage.

Discussion Peripheral BNS is not a recognised neurological manifestation of WM but with appropriate treatment a good outcome may be achieved.

Statistics from Altmetric.com

Request permissions

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.