Abstract Details

Title The Clinical Spectrum of Anti-MAG Neuropathies at the National Hospital for Neurology

Topic Peripheral Nerve

Presentation(s) S58 - (-)

Poster/Presentation
Number 006

Objective

Background IgM paraproteins are associated with demyelinating and inflammatory peripheral neuropathies. IgM paraproteins may bind with anti-myelin-associated glycoprotein antibodies (anti-MAG) to cause a neuropathy.

Design/Methods Case notes were analysed for the presentation, progression, treatments given and response. All statistics were paired 2-tail Student's t-test.

Results At 1st consultation (month 0) patients had a MRC score 66/70 ± 1.6 (mean ± SEM) with a non-significant progression over 10 years. A 6-point sensory sum score increased from 5.4/24 ± 0.89 (month 0) to 6.5 ± 1.5 (month 41) but was not significant. 9 patients had tremor and 9 patients developed unsteady gait with no clear overlap between the two. 21 of 22 patients had raised total IgM from which 18 patients had ? 1 IgM ? band. 19 patients had MGUS, 1 patient progressed to chronic lymphocytic leukaemia after several years, and only 1 patient was diagnosed with Waldenstroms Macroglobulinaemia after initial assessment. There was no difference in clinical phenotype of neuropathy between any of the different haematological diagnoses. 12 patients received immunomodulation - 1 patient received steroids and 4 IVIg without effect. 7 patients had rituximab and, of these, 4 had no subjective improvement, 1 had an initial improvement in balance and then deteriorated, 1 had an objective improvement in sensation yet no actual improvement in tremor or balance, and 1 patient achieved stabilisation.

Conclusions This cohort reflects that anti-MAG neuropathies are a slowly-progressive mainly sensory neuropathy that is almost always associated with a MGUS. Patients are usually refractory to common treatments and there is no consistent response to rituximab.

Authors/Disclosures
PRESENTER	No disclosure on file
Douglas R. Jeffery, MD, PhD (Lake Norman Neurology At Piedmont Health Care)	No disclosure on file
Hadi Manji	No disclosure on file
	No disclosure on file
Mary Reilly, MD, FRCP, FRCPI (National Hospital for Neurology and Neurosurgery)	The institution of Mary Reilly, MD, FRCP, FRCPI has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alynlam. Mary Reilly, MD, FRCP, FRCPI has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AKCEA. Mary Reilly, MD, FRCP, FRCPI has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Inflectis. The institution of Mary Reilly, MD, FRCP, FRCPI has received research support from MRC. The institution of Mary Reilly, MD, FRCP, FRCPI has received research support from NIH. The institution of Mary Reilly, MD, FRCP, FRCPI has received research support from MDA.
Michael P. Lunn, MBBS	No disclosure on file

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