Neuroimmunology

Standardised CE IVD marked ELISAs
for investigation of all common peripheral neuropathies.

Neuroimmunological disorders are autoimmune diseases that occur when the immune system attacks the nervous system 
(central or peripheral).

Applications

New Guidelines for Diagnosis of CIDP Released

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-mediated neurological disorder. Typically it is characterised by a symmetrical impairment in motor function and sensory disturbance, with proximal and distal muscle weakness.
Diagnosing CIDP can be difficult, and in almost half of cases misdiagnosis is reported. It has several different clinical presentations, giving rise to a number of atypical variants, and misdiagnosis occurs predominantly in patients with these variants.

The guidelines on the diagnosis and treatment of CIDP have recently been reviewed by Van den Bergh et al.

As serological laboratory testing of antibodies is an important element in the diagnostic work-up for CIDP, they advise anti-MAG (Myelin associated glycoprotein) antibody testing in all patients with an IgM paraprotein fulfilling CIDP diagnostic criteria.

Recommended Assay

Determining the presence of anti-MAG antibodies in some CIDP variants is vital as high titres of these antibodies would imply a different diagnosis.The new guidelines recommend the use of the BÜHLMANN anti-MAG Autoantibodies ELISA.

Test for Two Antibodies at Once

Testing for anti-GM1 antibodies may also give additional hints to demyelinating processes in CIDP variants. The BÜHLMANN Gangliocombi™ MAG ELISA combines testing for both anti-MAG and anti-ganglioside antibodies, including the anti-GM1 antibody, at the same time. The kit features all enzyme label-conjugates and allows for an efficient and complete work-up in two steps.

Autoantibodies to Glycoproteins and Glycolipids

Immune-mediated neuropathies represent an important sub-section of peripheral neuropathies, particularly as they are progressive and may be treated successfully. Diagnostic testing for anti-ganglioside and/or anti-MAG antibodies plays a crucial role in the diagnosis and treatment of these diseases. Our neuroimmunology portfolio contains sensitive anti-ganglioside and specific anti-MAG antibody assays.

Guillain-Barré Syndrome (GBS) and related disease patterns constitute a group of demyelinating or axonal motor/sensory neuropathies that are treatable. The demyelination of neurons by autoimmune processes directed against peripheral nerves can be triggered by infection of the upper air or gastrointestinal tracts (Campylobacter jejuni).

Miller-Fisher Syndrome (MFS) is a variant of GBS with ophthalmoplegia, ataxia and areflexia. Similar to GBS, MFS may occur upon infection. More than 90% of patients have antibodies against ganglioside Q1b (GQ1b).

Multifocal Motor Neuropathy (MMN) affects numerous, localised areas within a patient and is a progressive and chronic disease. In up to 80% of patients, serologic polyclonal antibodies against ganglioside M1 (GM1) may be found. These antibodies are known to be of pathologic relevance. The polyclonal neuropathies are distinguished from the monoclonal gammopathies, which originate from demyelinating neuropathies. As many as 10% of patients with a neuropathy of unknown etiology can be assigned to the group of monoclonal autoimmune processes. In as many as 50% of these patients, IgM antibodies against Myelin Associated Glycoprotein (MAG), a major component of the myelin sheath may be detected.

Not only is the determination of anti-MAG antibodies of diagnostic significance, but it can also be important in the context of therapy (e.g. Rituximab).

Polyneuropathies Disease Isotype Associated Autoantibodies
Polyneuropathies Disease Isotype Associated Autoantibodies Acute GBS > IgG GM1
Polyneuropathies Disease Isotype Associated Autoantibodies AMAN (1) > IgG GM1, GD1a
Polyneuropathies Disease Isotype Associated Autoantibodies AIDP (2) > IgG GM1
Polyneuropathies Disease Isotype Associated Autoantibodies AMSAN (3) > IgG GM1
Polyneuropathies Disease Isotype Associated Autoantibodies MFS and variants > IgG GQ1b, GD1b
Polyneuropathies Disease Isotype Associated Autoantibodies Chronic MAG neuropathy (4) > IgM MAG (5)
Polyneuropathies Disease Isotype Associated Autoantibodies CANOMAD (6) > IgM GQ1b, GD1b
Polyneuropathies Disease Isotype Associated Autoantibodies Mononeuropathies MMN > IgM GM1
Polyneuropathies Disease Isotype Associated Autoantibodies MMN > IgM GA1, GM1
Polyneuropathies Disease Isotype Associated Autoantibodies MMN > IgM GM1, GM2

Key to table:
(1) AMAN = acute motor axonal neuropathy
(2) AIDP = acute inflammatory demyelinating polyneuropathy
(3) AMSAN = acute motor sensory axonal neuropathy
(4) MAG = Myelin-associated Glycoprotein
(5) MAG negative patients may be anti-SGPG positive and vice versa
(6) CANOMAD = chronic ataxic neuropathy ophthalmoplegia IgM agglutinins disyalosyl antibodies (such as GD1b and GQ1b)

Neuroimmunology ELISAs

The BÜHLMANN Anti-MAG & Anti-Ganglioside Autoantibody ELISAs offer the most sensitive and efficient screening and monitoring of peripheral neuropathies.

GanglioCombi™ MAG ELISA

GanglioCombi™ MAG ELISA offers the possibility for the investigation of anti-Ganglioside antibodies as well as anti-MAG (myelin-associated glycoproteins) antibodies in the same approach. The Kit contains all 3 Enzyme labels:

IgG/IgM-Mix, IgG and IgM that allows cost-effective algorithms including testing in 2 steps: (1st) Initial screening for antibodies with the IgM/IgG-mix followed by (2nd) separate re-testing of positive samples with IgG- and IgM-enzyme label to identify the underlying pathology.

GanglioCombi™ Light ELISA

GanglioCombi™ Light ELISA combines on one microplate the three most relevant and frequent gangliosides, GD1b, GQ1b and GM1. The kit contains 3 enzyme labels: IgG/IgM-Mix, IgG and IgM.

Anti-MAG ELISA

The anti-MAG antibody ELISA test by BÜHLMANN is the acknowledged Gold standard by many Neurologists and Laboratories to reliably quantify anti-MAG antibodies in immune-mediated demyelinating neuropathies.

Based on highly purified MAG from human brain, the assay is characterized by outstanding sensitivity and specificity. Anti-MAG antibody ELISA may be used as stand-alone test or in combination with BÜHLMANN GanglioCombi™ MAG ELISA to confirm auto-antibodies against MAG.

Anti-SGPG ELISA

In addition to the anti-MAG ELISA, the BÜHLMANN anti-SGPG ELISA enhances the chance to detect all IgM-related neuropathies.

Anti-SGPG antibodies have a wider spectrum than anti-MAG antibodies – some patients which are anti-SGPG positive are negative for anti-MAG antibodies. Therefore, patients should be tested for both antibodies.

Anti-GM1 ELISA

Anti-GM1 ELISA allows individual testing of anti-GM1 antibodies of the IgG and/or the IgM isotype.

While IgG antibodies are related to Guillain-Barré Syndrome (GBS), IgM antibodies are found in multifocal neuropathy (MMN) and lower motor neuron disease (LMND).