RT Journal Article
SR Electronic
T1 PERIPHERAL NERVE BING-NEEL SYNDROME
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP e4
OP e4
DO 10.1136/jnnp-2015-312379.151
VO 86
IS 11
A1 Aisling Carr
A1 Shirley D'Sa
A1 A Arasaretnam
A1 K Boyd
A1 R Johnston
A1 Zane Jaunmuktane
A1 Sebastian Brandner
A1 T Marafioti
A1 Martin Koltzenburg
A1 Mike Lunn
YR 2015
UL http://jnnp.bmj.com/content/86/11/e4.59.abstract
AB 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.