g(m1)-ganglioside and Muscle-Weakness

We propose the finger drop sign as a new clinical variant of acute motor axonal neuropathy (AMAN) defined by immunological and radiological evidence. We identified eight consecutive patients who had AMAN. All of them developed prominent involvement of the finger extensors. We performed magnetic resonance imaging (MRI) of the extremity muscles and serological assays for antiganglioside antibodies and Campylobacter jejuni. Patients with AMAN showed characteristic and a markedly sustained weakness of the finger extensors with a distinctive pattern of the finger drop sign. Limb MRI revealed unevenly distributed abnormal signals in the muscles mainly innervated by the posterior interosseous nerve. All tested patients showed positivity for immunoglobulin G antibody against ganglioside complex of GM1 and phosphatidic acid. A pathophysiological understanding of this unique syndrome can provide further insight into antiganglioside-antibody-mediated axonal injury in Guillain-Barré syndrome.

Topics: Aged; Antibodies, Bacterial; Autoantibodies; Axons; Campylobacter jejuni; Electrodiagnosis; Electromyography; Female; Fingers; G(M1) Ganglioside; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Immunologic Factors; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Weakness; Neural Conduction; Neurologic Examination; Phosphatidic Acids; Physical Examination; Retrospective Studies

We describe the case of a 10-year-old child with the acute motor axonal neuropathy (AMAN) form of Guillain-Barré syndrome (GBS) with preserved tendon reflexes, 6 days after a bout of gastroenteritis. The child quickly showed weakness of the distal muscles of his four limbs, with preserved tendon reflexes and a raised CSF protein concentration with no cells. Nerve conduction studies showing motor axonal degeneration confirmed the diagnosis of GBS in spite of preserved tendon reflexes. The serum was positive for IgG antibodies to gangliosides GM1 and GD1b. The child received intravenous immunoglobulins, which resulted in a favorable progression. This case proves that GBS with normal tendon reflexes exists. The other cases of SGB with preserved tendon reflexes already described in the literature were the AMANs form with antibodies to gangliosides in the serum and only adults were affected.

Topics: Axons; Biomarkers; Child; Follow-Up Studies; G(M1) Ganglioside; Gangliosides; Gastroenteritis; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Muscle Weakness; Reflex, Stretch; Treatment Outcome

Topics: Female; G(M1) Ganglioside; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Middle Aged; Motor Neurons; Muscle Weakness; Neural Conduction; Neurologic Examination; Pyramidal Tracts; Reflex, Babinski

Serum IgM binding to GM1 ganglioside (GM1) is often associated with chronic acquired motor neuropathies. This study compared the frequency and clinical associations of serum IgM binding to a different antigen, a disulphated heparin disaccharide (NS6S), with results of IgM binding to GM1.. Serums and clinical features were retrospectively compared from 75 patients with motor neuropathies and 134 controls with amyotrophic lateral sclerosis (ALS), chronic immune demyelinating polyneuropathy (CIDP) and sensory neuropathies. Clinical correlations of positive IgM anti-GM1 testing found in 27 of 2113 unselected serums were also reviewed. Serum testing for IgM binding to NS6S and GM1 used covalent antigen linkage to ELISA plates.. High titre IgM binding to NS6S and GM1 each occurred in 43%, and to one of the two in 64%, of motor neuropathy patients. Motor neuropathy syndromes were present in 25 of 27 patients with high titre serum IgM binding to GM1 in the unselected serums. IgM anti-GM1 or NS6S antibody related motor neuropathy syndromes usually have asymmetric, predominantly distal, upper extremity weakness.. IgM binding to NS6S disaccharide is associated with motor neuropathy syndromes and occurs with similar frequency to IgM binding to GM1. Testing for IgM binding to NS6S in addition to GM1 increases the frequency of finding IgM autoantibodies in motor neuropathies from 43% to 64%. High titres of serum IgM binding to GM1, tested with covalent ELISA methodology, have 93% specificity for motor neuropathy syndromes. High titres of serum IgM binding to NS6S have specificity for immune motor neuropathies compared with ALS and CIDP.

Topics: Adult; Age of Onset; Aged; Disaccharides; Electrodiagnosis; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Heparin; Humans; Immunoglobulin M; Male; Middle Aged; Motor Neuron Disease; Muscle Weakness; Neural Conduction; Neuromuscular Diseases; Predictive Value of Tests; Young Adult

Polyethylene glycol-interferon alpha (PEG-IFNalpha) has been used as the standard treatment for hepatitis C virus (HCV) infection. There have been no previous reports of polyradiculoneuropathy with anti-ganglioside antibodies induced by PEG-IFNalpha-2b. We report a 59-year-old man who developed polyradiculoneuropathy during treatment with PEG-IFN alpha-2b for chronic HCV infection. Serum levels of anti-asialo-GM1 (GA1) and anti-GM1 antibodies were elevated. Cessation of therapy with double filtration plasmapheresis resulted in marked improvement in his symptoms accompanied by a reduction in the antibody level. PEG-IFN alpha-2b may induce peripheral neuropathy mediated by anti-GA1 and anti-GM1 antibodies.

Topics: Antiviral Agents; Autoantibodies; Autoantigens; Autoimmune Diseases of the Nervous System; Drug Carriers; Drug Therapy, Combination; G(M1) Ganglioside; Gait Disorders, Neurologic; Hepatitis C, Chronic; Humans; Interferon alpha-2; Interferon-alpha; Male; Middle Aged; Muscle Weakness; Plasmapheresis; Polyethylene Glycols; Polyradiculoneuropathy; Recombinant Proteins; Ribavirin

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

Topics: Adult; Autoantibodies; Brain Stem; Encephalitis; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Muscle Weakness; Ophthalmoplegia

Motor neuropathy with multifocal conduction blocks represents a recently identified autoimmune disorder of the peripheral nerve myelin. Association of motor neuropathies or neuronopathies with thyroid disorders, such as hyperthyroidism, hypothyroidism or thyroid neoplasms has been rarely described. We studied a 61-year-old man with a 2-year-history of slowly progressive weakness of the left limbs with atrophy and fasciculations. Nerve conduction velocity studies revealed multifocal motor conduction blocks. Serum IgM titer of antibodies against GM1 was elevated (1:1280; n.v. up to 1:640). Thyroid studies were compatible with Hashimoto's thyroiditis. Therapy with high dose intravenous immunoglobulins was followed by a prompt clinical recovery. Then the disease assumed an intravenous immunoglobulins dependent course with a full clinical, but transient, recovery. This is the first observation of an association of multifocal motor neuropathy with high titers of GM1 and Hashimoto's thyroiditis and reinforces the multifocal motor neuropathy autoimmune origin as well as the repeated clinical recoveries after intravenous immunoglobulins. This case also suggests to deeply investigate the thyroid function in patients with multifocal motor neuropathy.

Topics: Demyelinating Diseases; Electromyography; Enzyme-Linked Immunosorbent Assay; Fasciculation; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Male; Middle Aged; Motor Neuron Disease; Muscle Weakness; Muscular Atrophy; Neural Conduction; Thyroiditis, Autoimmune

Some humans develop the axonal form of Guillain-Barré syndrome after receiving bovine brain ganglioside. On sensitization with the ganglioside mixture, all of a group of rabbits injected developed high anti-GM1 IgG antibody titers, flaccid limb weakness of acute onset, and a monophasic illness course. Pathological findings for the peripheral nerves showed predominant Wallerian-like degeneration, with neither lymphocytic infiltration nor demyelination. IgG was deposited on the axons of the anterior roots, and GM1 was proved to be present on the axons of peripheral nerves. Sensitization with purified GM1 also induced axonal neuropathy, indicating that GM1 was the immunogen in the mixture. A model of human axonal Guillain-Barré syndrome has been established that uses inoculation with a bovine brain ganglioside mixture or isolated GM1. This model may help to clarify the molecular pathogenesis of the syndrome and to develop new treatments for it.

Topics: Animals; Antibodies; Antibody Specificity; Autoantibodies; Axons; Cattle; Chemotaxis, Leukocyte; Disease Models, Animal; G(M1) Ganglioside; Guillain-Barre Syndrome; Immunization; Immunoglobulin G; Immunoglobulin M; Immunohistochemistry; Male; Muscle Weakness; Peripheral Nerves; Rabbits; Wallerian Degeneration