g(m1)-ganglioside and Polyradiculoneuropathy--Chronic-Inflammatory-Demyelinating

Topics: Animals; Autoantibodies; Diagnosis, Differential; G(M1) Ganglioside; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Prognosis; Syndrome

Topics: G(M1) Ganglioside; Humans; Optic Neuritis; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Vision Disorders

Antibodies to the ganglioside GD1b have been reported in various forms of immune-mediated neuropathy, but their clinical relevance for diagnosis and prognosis is unknown. We investigated the prevalence of anti-GD1b antibodies in acute and chronic immune-mediated neuropathies, and the clinical presentation and outcome in Guillain-Barré syndrome (GBS) and Miller Fisher-GBS overlap syndrome (MF-GBS). Anti-GD1b, anti-GM1 and anti-GQ1b antibodies were tested in serum of patients with GBS (N = 165), Miller Fisher syndrome (N = 10), MF-GBS (N = 28), monoclonal gammopathy of unknown significance neuropathy (MGUS; N = 101), chronic inflammatory demyelinating polyneuropathy (N = 29), paraneoplastic syndrome with anti-Hu-associated neuropathy (PNS; N = 11), other auto-immune diseases (AID; N = 60), and healthy controls (HC; N = 60). All samples were tested by enzyme-linked immunosorbent assay according to the Inflammatory Neuropathy Cause and Treatment protocol. IgM anti-GD1b antibodies were found in GBS (N = 4; 2.4%), MGUS (N = 3; 3.0%), and PNS patients (N = 1; 9.1%). IgG anti-GD1b antibodies were found in GBS (N = 20; 12.1%) and MF-GBS (N = 4; 14.3%) patients, but not in the AID and HC group. In the combined group of MF-GBS and GBS patients ((MF-)GBS), 14/36 (38.9%) patients with IgG anti-GD1b antibodies also had IgG anti-GM1 antibodies, and IgG anti-GD1b and IgG anti-GQ1b antibodies were found in 3/29 (10.3%) patients. Patients with (MF-)GBS and anti-GD1b without anti-GM1 antibodies did not differ regarding sensory disturbances or disease severity but recovered faster regarding the ability to walk independently compared with patients without anti-GD1b antibodies (P = 0.031) and with patients with both anti-GD1b and anti-GM1 antibodies (P = 0.034). In conclusion, testing for anti-GD1b antibodies may identify a specific group of immune-mediated neuropathies and (MF-)GBS patients with only anti-GD1b antibodies tend to recover faster.

Topics: Adult; Aged; Autoantibodies; Autoantigens; Autoimmune Diseases of the Nervous System; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Lupus Erythematosus, Systemic; Middle Aged; Miller Fisher Syndrome; Monoclonal Gammopathy of Undetermined Significance; Paraneoplastic Polyneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Sjogren's Syndrome

LM1 is the predominant glycolipid in human peripheral nerve myelin and antibodies to LM1 and LM1-containing ganglioside complexes are detected in some patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The clinical features of patients with such antibodies have not yet been investigated.. Serum antibodies to LM1, a mixture of GM1 and LM1 (GM1/LM1), and that of GD1b and LM1 (GD1b/LM1) were examined in 75 consecutive patients with CIDP. The clinical features of the CIDP patients with such antibodies in the present series and those in the previous reports were investigated and compared with those of antibody-negative patients.. Of the 75 patients with CIDP, two had antibodies to LM1, three had anti-GM1/LM1 complex antibody, one had anti-GD1b/LM1 complex antibody and two had antibodies to both the GM1/LM1 and GD1b/LM1 complexes. Patients with the LM1-associated antibodies did not have cranial nerve deficits (p<0.05) and exhibited ataxia more frequently than the antibody-negative patients (p<0.01).. In humans, LM1 is contained more in the dorsal root than in the cranial nerves. The clinical features of CIDP patients with antibodies to LM1 and LM1-containing complexes may be associated with the distribution of the LM1 antigen. LM1-associated antibodies are possible markers for a subclass of CIDP.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Ataxia; Autoantibodies; Child; Cranial Nerve Diseases; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Glycolipids; Humans; Immunoglobulin G; Immunotherapy; Male; Middle Aged; Neural Conduction; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Young Adult

The favorable response to treatment with IV immunoglobulins and the presence of IgM antibodies to the glycolipid GM1 are indications that inflammation underlies multifocal motor neuropathy (MMN) pathogenesis. We investigated the association of MMN with human leukocyte antigen (HLA) class I and II antigens.. HLA class I and II antigens of 74 Dutch patients with MMN and 700 controls were determined in a case-control study. Associations of HLA types with MMN disease characteristics were investigated.. Compared with controls, patients with MMN had higher frequencies of HLA-DRB1*15 (41 vs 24%, p = 0.0017). Disease characteristics were not associated with specific HLA types.. Similar associations were found in patients with multiple sclerosis and women with chronic immune-mediated demyelinating neuropathy, which may suggest that these demyelinating disorders share pathogenic mechanisms.

Topics: Adult; Age Factors; Aged; Brachial Plexus; Case-Control Studies; Chi-Square Distribution; Female; G(M1) Ganglioside; HLA-DQ Antigens; HLA-DQ beta-Chains; HLA-DR Antigens; HLA-DRB1 Chains; Humans; Immunoglobulin M; Magnetic Resonance Imaging; Male; Membrane Glycoproteins; Middle Aged; Multiple Sclerosis; Neural Conduction; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retrospective Studies

Distal acquired demyelinating symmetric (DADS) neuropathy is clinically characterised by distal motor and sensory disturbances. Typically DADS does not respond or responds poorly to intravenous immunoglobulins (IVIg). We report the case of a 58-year-old patient who developed distal paraparesis. Serum electrophoresis demonstrated monoclonal IgM paraproteinemia having an anti-GM1 but no anti-MAG activity. Conduction velocities showed demyelinating pattern. Work-up excluded a lymphoproliferative disorder After IVIg treatment we observed a clinical and neurophysiological improvement. Regarding these peculiar findings, we suggest that DADS needs to be splitted in several forms determined among others by clinical, neurophysiological and antiganglioside profile and therapeutic response. We advocate to perform systematic antiganglioside antibodies assay additionnaly to anti-MAG when DADS is suspected in order to improve dysimmune neuropathies classification.

Topics: Antibodies, Anti-Idiotypic; Demyelinating Diseases; G(M1) Ganglioside; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Neural Conduction; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating

Topics: Autoantibodies; Biomarkers; Enzyme-Linked Immunosorbent Assay; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Specimen Handling

Tumor necrosis factor-alpha (TauNuFalpha) blockers are effective in the treatment of inflammatory arthritis but can induce autoimmune disorders including multiple sclerosis. Described are two patients who developed chronic inflammatory demyelinating polyneuropathy after initiation of anti-TNFalpha treatment.

Topics: Aged; Antibodies, Monoclonal; Antirheumatic Agents; Arthritis, Rheumatoid; Autoantibodies; Etanercept; Female; G(M1) Ganglioside; Humans; Immunoglobulin G; Infliximab; Male; Middle Aged; Muscle Weakness; Neural Conduction; Peripheral Nerves; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Receptors, Tumor Necrosis Factor; Recovery of Function; Sensation Disorders; Spondylitis, Ankylosing; Tumor Necrosis Factor-alpha

This study was performed to determine whether increased ganglioside-specific T cell reactivity can be detected in the peripheral blood of patients with Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). T cell responsiveness to the gangliosides GM1, GM3, GD1a, GD1b, GD3, GT1b, GQ1b and sulphatide was assessed in peripheral blood mononuclear cells from untreated GBS patients (57), CIDP patients (43), patients with other peripheral neuropathies (55) and healthy control subjects (74) in a standard 6-day proliferation assay. Increased T cell reactivity to GM1 occurred in GBS patients compared to healthy controls and patients with other neuropathies. There was increased reactivity to GM3 in GBS patients compared to patients with other neuropathies but not compared to healthy controls. The frequencies of increased T cell reactivity to GM1 and GM3 in CIDP patients were intermediate between those of GBS patients and controls. We suggest that T cell reactivity to gangliosides might play a contributory role in the pathogenesis of GBS and perhaps CIDP.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cell Proliferation; Chi-Square Distribution; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Odds Ratio; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; T-Lymphocytes; Tetanus Toxoid

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disorder of the peripheral nervous system with a probable auto-immune pathogenesis. The nature of the responsible autoantigens is unclear in most patients. We used the Western immunoblot technique to seek antibodies to peripheral nerve protein antigens. Sera from eight of 32 (25%) CIDP patients, 12 of 37 (32%) Guillain-Barré syndrome (GBS) patients, zero of 30 (0%) chronic idiopathic axonal polyneuropathy patients and two of 39 (5%) healthy control subjects contained anti-peripheral nerve protein antibodies. The frequency of such antibodies was significantly greater in both CIDP (p = 0.04) and GBS (p = 0.003) patients than in normal control subjects. For CIDP patients, there were non-significant trends for antibodies to be more common in females and in those who responded to treatment with either intravenous immunoglobulin or plasma exchange. The commonest antibodies were directed against a band at 28 kDa, resembling that labelled by a monoclonal antibody against myelin protein zero (P0). Six CIDP and seven GBS patients' sera reacted with this band. These results support the view that antibodies to myelin proteins, and especially P0, are present in the serum of some patients with CIDP and GBS.

Topics: Adult; Aged; Antibodies; Blotting, Western; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Molecular Weight; Myelin Proteins; Peripheral Nerves; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Time Factors

To determine the possible role of anti-GM1 ganglioside antisera from patients with Gullain-Barr*e syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP) in the development of nerve dysfunction.. The effect of the anti-GM1 antibody positive antisera obtained from 4 GBS patients and 1 CIDP patient on membrane potential and ionic currents in rat single myelinated nerve fibers was investigated using the voltage clamp technique and compared with that of the anti-GM1 negative antisera obtained from 3 healthy controls and 2 GBS patients.. In the presence of active complement, anti-GM1 positive antisera from 5 patients including 4 GBS patients and 1 CIDP patient significantly suppressed Na+ current more than anti-GM1 negative antisera.. This study supports the notion that anti-GM1 antibody is one of the causative factors of conduction abnormality in GBS patients.

Topics: Adult; Animals; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Guillain-Barre Syndrome; Humans; Immune Sera; Male; Membrane Potentials; Middle Aged; Nerve Fibers, Myelinated; Patch-Clamp Techniques; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Potassium Channels; Rats; Rats, Sprague-Dawley; Sodium; Sodium Channels