Chapter 22. Paraproteinaemic Demyelinating Neuropathies

  1. Nils Erik Gilhus MD, PhD Professor17,
  2. Michael P. Barnes MD, FRCP Professor of Neurological Rehabilitation Medical Director18 and
  3. Michael Brainin MD Professor19
  1. R. D. M. Hadden1,
  2. E. Nobile - Orazio2,
  3. C. Sommer3,
  4. A. F. Hahn4,
  5. I. Illa5,
  6. E. Morra6,
  7. J. Pollard7,
  8. M. P. T. Lunn8,
  9. P. Bouche9,
  10. D. R. Cornblath10,
  11. E. Evers11,
  12. C. L. Koski12,
  13. J. - M. Léger13,
  14. P. Van den Bergh14,
  15. P. van Doorn15 and
  16. I. N. van Schaik16

Published Online: 6 SEP 2010

DOI: 10.1002/9781444328394.ch22

European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition

European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition

How to Cite

Gilhus, N. E., Barnes, M. P. and Brainin, M. (2010) Paraproteinaemic Demyelinating Neuropathies, in European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition, Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444328394.ch22

Author Information

  1. 17

    Department of Clinical Medicine, University of Bergen and Department of Neurology, Haukeland University Hospital, Bergen, Norway

  2. 18

    University of Newcastle, Hunters Moor Neurorehabilitation Ltd, Newcastle upon Tyne, UK

  3. 19

    Department of Clinical Medicine and Prevention, and Center for Clinical Neurosciences, Donau-Universität Krems, Department of Neurology, Landesklinikum Donauregion Tulln Tulln, Austria

Author Information

  1. 1

    King's College Hospital, London, UK

  2. 2

    IRCCS Humanitas Clinical Institute, University of Milan, Italy

  3. 3

    University of Würzburg, Germany

  4. 4

    University of Western Ontario, London, Canada

  5. 5

    Hospital Sta. Creu i Sant Pau, Barcelona, Spain

  6. 6

    Niguarda Hospital, Milan, Italy

  7. 7

    University of Sydney, Australia

  8. 8

    National Hospital for Neurology and Neurosurgery, Queen Square, London, UK

  9. 9

    CHU Pitié - Salpêtrière, Paris, France

  10. 10

    Johns Hopkins University, Baltimore, MD, USA

  11. 11

    Guillain - Barré Syndrome Support Group of the UK

  12. 12

    University of Maryland, Baltimore, USA

  13. 13

    Faculté de Médecine Pitié - Salpêtrière, Paris, France

  14. 14

    Cliniques St.-Luc, Université Catholique de Louvain, Brussels, Belgium

  15. 15

    Erasmus Medical Centre, Rotterdam, The Netherlands

  16. 16

    Academic Medical Centre, Amsterdam, The Netherlands

Publication History

  1. Published Online: 6 SEP 2010
  2. Published Print: 15 OCT 2010

ISBN Information

Print ISBN: 9781405185332

Online ISBN: 9781444328394



  • paraproteinaemic demyelinating neuropathies;
  • clinically useful guidelines - for patients with demyelinating neuropathy and a paraprotein (paraproteinaemic demyelinating neuropathy, PDN);
  • chronic inflammatory demyelinating polyradiculoneuropathy (CIDP);
  • standard serum protein electrophoresis (SPEP) and serum immunoelectrophoresis (SIEP) and serum immunofixation electrophoresis (SIFE);
  • typical syndromes of paraproteinaemic demyelinating neuropathy (PDN);
  • IgM paraproteinaemic demyelinating neuropathy;
  • patients with IgM PDN, meeting definite electrophysiological criteria for CIDP;
  • antibodies to myelin-associated glycoprotein (MAG) and other neural antigens;
  • IgG or IgA paraproteinaemic demyelinating neuropathy;
  • Waldenström's macroglobulinaemia - defined by presence of IgM (usually kappa) paraprotein


This chapter contains sections titled:

  • Objectives

  • Background

  • Search strategy

  • Methods for reaching consensus

  • Results

  • Investigation and classification of the paraprotein

  • Typical syndromes of paraproteinaemic demyelinating neuropathy (PDN)

  • Other neuropathy syndromes associated with paraproteinaemia

  • Is the paraprotein causing the neuropathy?

  • Cerebrospinal fluid and nerve biopsy

  • Treatment of paraproteinaemic demyelinating neuropathies

  • References