Autoimmune Peripheral Neuropathies and Contribution of Antiganglioside/Sulphatide Autoantibody Testing

Dirk Roggenbuck; Emilien Delmont; Dirk Reinhold; Peter Schierack; Karsten Conrad; Joseph Boucraut

doi:10.31138/mjr.31.1.10

Autoimmune Peripheral Neuropathies and Contribution of Antiganglioside/Sulphatide Autoantibody Testing

Mediterr J Rheumatol. 2020 Mar 31;31(1):10-18. doi: 10.31138/mjr.31.1.10. eCollection 2020 Mar.

Authors

Dirk Roggenbuck^{1

2}, Emilien Delmont³, Dirk Reinhold⁴, Peter Schierack^{1

2}, Karsten Conrad⁵, Joseph Boucraut^{6

7}

Affiliations

¹ Faculty of Health Sciences, Joint Faculty of the Brandenburg University of Technology Cottbus - Senftenberg, the Brandenburg Medical School Theodor Fontane and the University of Potsdam, Germany.
² Institute of Biotechnology, Faculty Environment and Natural Sciences, Brandenburg University of Technology Cottbus Senftenberg, Senftenberg, Germany.
³ Referral Center for Neuromuscular Diseases and ALS, La Timone Hospital, AP-HM, Marseille France.
⁴ Institute of Molecular and Clinical Immunology, Otto-von-Guericke University Magdeburg, Magdeburg, Germany.
⁵ Institute of Immunology, Medical Faculty of the Technical University Dresden, Dresden, Germany.
⁶ Aix Marseille Université, Institut de Neurosciences de la Timone, Medicine Faculty, Marseille, France.
⁷ Immunology laboratory, Conception Hospital, AP-HM, Marseille, France.

Abstract

Peripheral immune-mediated polyneuropathies (IMPN) are a diverse group of rare neurological illnesses characterized by nerve damage. Leading morphological features are mostly nerve fibre demyelination or combination of axonal damage and demyelination. There has been remarkable progress in the clinical and electrophysiological categorization of acute (fulminant, life-threatening) and chronic (progressive/remitting-relapsing) immune-mediated neuropathies recently. Besides electrophysiological and morphological makers, autoantibodies against glycolipids or paranodal/nodal molecules have been recommended as candidate markers for IMPN. The progress in testing for autoantibodies (autoAbs) to glycolipids such as gangliosides and sulfatide may have significant implications on the stratification of patients and their treatment response. Thus, this topic was reviewed in a presentation held during the 1st Panhellenic Congress of Autoimmune Diseases, Rheumatology and Clinical Immunology in Portaria, Pelion, Greece. For acute IMPN, often referred to as Guillain-Barré syndrome and its variants, several serological markers including autoAbs to gangliosides and sulphatide have been employed successfully in clinical routine. However, the evolution of serological diagnosis of chronic variants, such as chronic inflammatory demyelinating polyneuropathy or multifocal motor neuropathy, is less satisfactory. Serological diagnostic markers could, therefore, help in the differential diagnosis due to their assumed pathogenic role. Additionally, stratification of patients to improve their response to treatment may be possible. In general, a majority of patients respond well to causal therapy that includes intravenous immunoglobulins and plasmapheresis. As second line therapy options, biologicals (e.g., rituximab) and immunosuppressant or immunomodulatory drugs may be considered when patients do not respond adequately.

Keywords: autoantibody; ganglioside; immune-mediated polyneuropathy; sulphatide.

Publication types

Review