Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated neuropathy. The diagnosis of CIDP is based on clinical, electrophysiologic, as well as supportive and/or exclusion data. Next to the classical type of CIDP (often presenting with proximal and distal sensorimotor deficits), there are several CIDP variants and CIDP-mimicking diseases. While diagnosis of CIDP is challenging, misdiagnosis of CIDP has been reported in almost half of the cases.
Recently, the consensus guidelines on the diagnosis and treatment of CIDP were revised by a taskforce in order to update the current guidelines dating 2010 and to include evidence-based recommendations and consensus-based Good Practice Points for clinical practice (Van den Bergh et al., 2021).
Thus, serologic laboratory testing of antibodies has become an important element in the diagnostic work-up of CIDP. Not only antibodies contribute to identify CIDP patients with specific clinical characteristics, but also its presence provides evidence for the treatment of these patients.
The taskforce advise anti-MAG antibody testing by means of BÜHLMANN anti-MAG Autoantibodies ELISA, in fact in all patients with an IgM paraprotein fulfilling CIDP diagnostic criteria. Determination of anti-MAG antibodies in this group of patients is important because high titers strongly imply a diagnosis which is different from CIDP.
Further, testing for anti-GM1 antibodies may help to identify multifocal and focal CIDP variants with a demyelinating pathology.
In relation to the treatment of CIDP, antibody testing not only has low costs but positive results have significant implications for both, diagnosis and treatment.
The BÜHLMANN GanglioCombi™ MAG ELISA, featuring both, MAG and gangliosides, provides a valid solution to the serologic work-up of CIDP as all relevant antibodies (against MAG and gangliosides) can be determined from a given sample at the same time.
Figure 1: Clinical variants of CIDP; motor deficits: red; sensory deficits: green; sensorimotor deficits: brown.
Abbreviations: CIDP: chronic inflammatory demyelinating Polyneuropathy. DADS: Distal acquired demyelinating symmetric neuropathy’; LSS: Lewis-Sumner syndrome; CISP: Chronic immune sensory polyradiculopathy; CANOMAD: chronic ataxic neuropathy associated with ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies.
Adopted from Lehmann, H. et al., J Neurol Neurosurg. Psychiatry, 90: 981–987, 2019.
CIDP Guidelines 2021:
Van den Bergh, P.Y.K, van Doorn, P.A., Hadden, R.D.M., et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision. Eur J Neurol. 2021; 1– 27. https://doi.org/10.1111/ene.14959