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  • Review Article
  • Published:

Diagnosis and treatment of chronic acquired demyelinating polyneuropathies

Nature Reviews Neurology volume 10, pages 435–446 (2014)Cite this article

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This article has been updated

Key Points

  • Identification and correct diagnosis of the chronic acquired demyelinating polyneuropathies is important because they are immune-mediated and, therefore, amenable to treatment

  • Early diagnosis and therapy can prevent progression and irreversible secondary axonal degeneration

  • If no other cause is found, chronic demyelinating neuropathies are diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), whereas chronic axonal neuropathies are considered idiopathic, and have no available treatment

  • Demyelinating neuropathies are usually diagnosed on the basis of electrodiagnostic studies; demyelinating-range conductance abnormalities that are more severe than what could result from axonal degeneration indicate demyelinating neuropathy

  • Pathological examination of motor nerve biopsies can help distinguish between multifocal motor neuropathy and motor neuron disease; sensory nerve biopsy can aid diagnosis of CIDP, anti-MAG neuropathy, or POEMS syndrome

  • Serum immunofixation electrophoresis can identify the presence of a monoclonal gammopathy in patients with anti-MAG neuropathy or POEMS syndrome

Abstract

Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria. Demyelinating neuropathies are further classified as hereditary or acquired—this distinction is important, because the acquired neuropathies are immune-mediated and, thus, amenable to treatment. The acquired chronic demyelinating neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuropathy), multifocal motor neuropathy (MMN), and POEMS syndrome. They have characteristic—though overlapping—clinical presentations, are mediated by distinct immune mechanisms, and respond to different therapies. CIDP is the default diagnosis if the neuropathy is demyelinating and no other cause is found. Anti-MAG neuropathy is diagnosed on the basis of the presence of anti-MAG antibodies, MMN is characterized by multifocal weakness and motor conduction blocks, and POEMS syndrome is associated with IgG or IgA λ-type monoclonal gammopathy and osteosclerotic myeloma. The correct diagnosis, however, can be difficult to make in patients with atypical or overlapping presentations, or nondefinitive laboratory studies. First-line treatments include intravenous immunoglobulin (IVIg), corticosteroids or plasmapheresis for CIDP; IVIg for MMN; rituximab for anti-MAG neuropathy; and irradiation or chemotherapy for POEMS syndrome. A correct diagnosis is required for choosing the appropriate treatment, with the aim of preventing progressive neuropathy.

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Figure 1: A suggested diagnostic pathway for chronic acquired demyelinating polyneuropathies.

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Change history

  • 08 July 2014

    In the version of the 'key points' originally posted online, the word 'demyelinating' was missing from the third point. The third point should read "If no other cause is found, chronic demyelinating neuropathies are diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), whereas chronic axonal neuropathies are considered idiopathic, and have no available treatment." The error has been corrected in the online PDF and HTML versions of the article.

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  1. Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 1305 York Avenue, Suite 217, New York, 10021, NY, USA

    Norman Latov

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The author has consulted for Oncogenex Pharmaceuticals, Novartis Pharma, XenoPort, CSL Behring, Baxter Healthcare, Sumitomo Pharma, Merck, and OncoGenex, owns stock in Therapath LLC, and has received a research grant to Cornell University from Grifols.

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Latov, N. Diagnosis and treatment of chronic acquired demyelinating polyneuropathies. Nat Rev Neurol 10, 435–446 (2014). https://doi.org/10.1038/nrneurol.2014.117

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