g(m1)-ganglioside and Reflex--Abnormal

Acute motor axonal neuropathy (AMAN) is a pure motor axonal subtype of Guillain-Barré syndrome (GBS) that was identified in the late 1990s. In Asia and Central and South America, it is the major subtype of GBS, seen in 30-65% of patients. AMAN progresses more rapidly and has an earlier peak than demyelinating GBS; tendon reflexes are relatively preserved or even exaggerated, and autonomic dysfunction is rare. One of the main causes is molecular mimicry of human gangliosides by Campylobacter jejuni lipo-oligosaccharides. In addition to axonal degeneration, electrophysiology shows rapidly reversible nerve conduction blockade or slowing, presumably due to pathological changes at the nodes or paranodes. Autoantibodies that bind to GM1 or GD1a gangliosides at the nodes of Ranvier activate complement and disrupt sodium-channel clusters and axoglial junctions, which leads to nerve conduction failure and muscle weakness. Improved understanding of the disease mechanism and pathophysiology might lead to new treatment options and improve the outlook for patients with AMAN.

Topics: Animals; Autoantibodies; Autoantigens; Axons; Campylobacter Infections; Campylobacter jejuni; Complement Activation; Diagnosis, Differential; Disease Models, Animal; Electrodiagnosis; G(M1) Ganglioside; Global Health; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Molecular Mimicry; Motor Neurons; Neural Conduction; Plasma Exchange; Randomized Controlled Trials as Topic; Ranvier's Nodes; Reflex, Abnormal; Sodium Channels

Topics: Acute Disease; Demyelinating Diseases; G(M1) Ganglioside; Gangliosides; Humans; Male; Peripheral Nervous System Diseases; Reflex, Abnormal; Young Adult

Guillain-Barré Syndrome (GBS) is a prototype of post-infectious autoimmune disease. A 76-year-old woman was treated for a renal abscess and developed muscle weakness in all four extremities, 18 days after the onset of infection. She was diagnosed with GBS on the basis of acute flaccid paralysis, hyporeflexia, nerve conduction studies (reduced amplitude of compound muscle action potentials), and high titers of IgG antibodies to GM1 and GalNAc-GD1a. GBS rarely occurs after sepsis and this case represents the first report of rapidly progressive GBS following Escherichia coli urosepsis.

Topics: Abscess; Aged; Autoantibodies; Disease Progression; Escherichia coli Infections; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunosorbent Techniques; Immunotherapy; Kidney Diseases; Neural Conduction; Paraplegia; Reflex, Abnormal; Time Factors

This is the first report of a case of Bickerstaff's brainstem encephalitis (BBE) associated with IgM antibodies to GM1b and GalNAc-GD1a. Subsequent to Campylobacter jejuni enteritis, the patient rapidly developed consciousness disturbance and hyperreflexia in addition to external ophthalmoplegia and cerebellar-like ataxia. EEG showed transient 7 Hz monorhythmic theta activities, predominantly in the front-central area. He received high doses of immunoglobulin intravenously and had completely recovered 3 months later. High anti-GM1b and anti-GalNAc-GD1a IgM antibody titers present during the acute phase decreased with his clinical improvement. An absorption study showed the anti-GM1b and anti-GalNAc-GD1a IgM antibodies to be cross-reactive. Anti-GM1b and anti-GalNAc-GD1a antibodies have been detected in some patients who developed Guillain-Barré syndrome after C. jejuni enteritis, whereas the anti-GQ1b IgG antibody is associated with BBE. Infection by C. jejuni bearing a GM1b-like or GalNAc-GD1a-like lipooligosaccharide may trigger the production of anti-GalNAc-GD1a and anti-GM1b IgM antibodies. It is not clear why our patient developed BBE rather than Guillain-Barré syndrome. These antibodies may, however, prove useful serological markers for identifying BBE patients who do not have the anti-GQ1b IgG antibody.

Topics: Ataxia; Autoantibodies; Brain Stem; Campylobacter Infections; Campylobacter jejuni; Child; Consciousness Disorders; Electroencephalography; Encephalitis; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin M; Male; Recovery of Function; Reflex, Abnormal

Topics: Acute Disease; Adult; Antibody Specificity; Autoantibodies; Autoantigens; Autoimmune Diseases of the Nervous System; Axons; Brain; Female; G(M1) Ganglioside; Gait Disorders, Neurologic; Humans; Magnetic Resonance Imaging; Motor Neurons; Plasma Exchange; Polyneuropathies; Reflex, Abnormal; Respiratory Tract Infections

To investigate the incidence of hyperreflexia in patients with Guillain-Barré syndrome (GBS), and its relation with electrodiagnosis of acute motor axonal neuropathy (AMAN), antiganglioside GM1 antibody, and Campylobacter jejuni infection. It was reported that patients with AMAN in northern China often had hyperreflexia in the recovery phase.. In 54 consecutive Japanese patients with GBS, sequential findings of tendon reflexes were reviewed. By electrodiagnostic criteria, patients were classified as having AMAN or acute inflammatory demyelinating polyneuropathy (AIDP). Anti-GM1 and anti-C jejuni antibodies were measured by enzyme linked immunosorbent assays.. Seven (13%) patients developed hyperreflexia with the spread of the myotatic reflex to other segments in the early recovery phase, one of whom already had hyperreflexia in the acute progressive phase. Of the seven patients, six had AMAN and all seven had anti-GM1 antibodies, whereas only two had anti-C jejuni antibodies. Hyperreflexia was more often found in patients with AMAN than AIDP (6/23 v 1/18, p=0. 002), and in patients with anti-GM1 antibodies than without them (7/26 v 0/28, p=0.01). Hyperreflexic patients had milder peak disabilities than patients without hyperreflexia (p=0.03). Increased motor neuron excitability in the hyperreflexic patients was supported by increased soleus H-reflex amplitudes and the appearance of H-reflexes in the small hand or foot muscles.. Hyperreflexia often occurs in patients with GBS especially with AMAN, anti-GM1 antibodies, and milder disease. Increased motor neuron excitability further characterises the subgroup of patients with GBS with AMAN and anti-GM1 antibodies.

Topics: Acute Disease; Adult; Antibodies; Antibodies, Bacterial; Campylobacter jejuni; Electrodiagnosis; Female; G(M1) Ganglioside; H-Reflex; Humans; Male; Middle Aged; Motor Neuron Disease; Polyradiculoneuropathy; Reflex, Abnormal; Reflex, Stretch; Syndrome