[Immune-mediated neuropathies]

Nervenarzt. 2016 Aug;87(8):887-98. doi: 10.1007/s00115-016-0164-3.
[Article in German]

Abstract

The Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are the most common immune-mediated polyneuropathies, which can show variable clinical and electrophysiological manifestations. Rarer immune-mediated neuropathies encompass paraproteinemic neuropathies (PPN), multifocal motor neuropathy (MMN) and vasculitic neuropathies. The diagnosis usually relies on the history of symptom evolution, distribution of nerve dysfunction and particularly on characteristic features in nerve conduction studies, aided by cerebrospinal fluid (CSF) examination and nerve biopsy findings. The therapeutic toolbox encompasses corticosteroids, immunoglobulins and plasmapheresis often accompanied by long-term immunosuppression. It is important to note that immune-mediated neuropathies selectively respond to treatment and contraindications need to be considered. Despite treatment a considerable number of patients suffer from permanent neurological deficits.

Keywords: Chronic inflammatory demyelinating polyneuropathy; Guillain-Barré syndrome; Immunosuppression; Mononeuritis multiplex; Multifocal motor neuropathy.

Publication types

  • Review

MeSH terms

  • Combined Modality Therapy / methods
  • Diagnostic Techniques, Neurological
  • Evidence-Based Medicine
  • Guillain-Barre Syndrome / diagnosis*
  • Guillain-Barre Syndrome / immunology
  • Guillain-Barre Syndrome / therapy*
  • Humans
  • Immunosuppressive Agents / toxicity*
  • Plasmapheresis / methods*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy*
  • Symptom Assessment / methods
  • Treatment Outcome

Substances

  • Immunosuppressive Agents