PT  - JOURNAL ARTICLE
AU  - Aisling Carr
AU  - Zane Jaunmuktane
AU  - Sebastian Brandner
AU  - J Kothari
AU  - Julian Blake
AU  - Shirley D'Sa
AU  - Mike Lunn
TI  - THE NEUROPATHY SPECTRUM IN WALDENSTRöM'S MACROGLOBULINAEMIA
AID  - 10.1136/jnnp-2015-312379.152
DP  - 2015 Nov 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - e4--e4
VI  - 86
IP  - 11
4099  - http://jnnp.bmj.com/content/86/11/e4.60.short
4100  - http://jnnp.bmj.com/content/86/11/e4.60.full
SO  - J Neurol Neurosurg Psychiatry2015 Nov 01; 86
AB  - Background UK WM Guidelines recognise 2 mechanisms of nervous system involvement either direct tumour invasion of the CNS or antibody mediated damage to peripheral nerves. We present 5 cases illustrating an extended diversity of PNS involvement in WM with implications for treatment and outcome.Cases A 73 year-old man (IgMκ WM) developed progressively disabling tremor with stable WM. Neurophysiology was demyelinating with disproportionate DMLs; anti-MAG antibodies were strongly positive. He was treated with DRC with symptom stabilisation.A 64 year-old woman (IgMκ) with typical anti-MAG neuropathy developed severe episodic foot pain and ulceration. Skin biopsy confirmed leucocytoclastic vasculitis. She was treated with DRC but died from sepsis.A 43 year-old man (IgMκ WM) with rapidly progressive sensorimotor neuropathy had lymphplasmacytoid cellular infiltrate in sural nerve. He was successfully treated with IDARAM.A 59 year-old man (IgMλ WM) developed small then large fibre axonal neuropathy with autonomic symptoms within 2 years. A SAP scan showed moderate kidney uptake. MRI neurography directed biopsy and confirmed amyloid. Palliative treatment was offered.A 70 year-old woman (IgMλ WM) developed severe burning pains in her feet and became ataxic 2 months later. Sural nerve biopsy confirmed endoneurial amyloid. DRC was given.Discussion These cases highlight the importance of accurate diagnosis in WM-associated neuropathy. We propose a novel diagnostic algorithm.