g(m1)-ganglioside and Ataxia

Most inflammatory neuropathies, both acute and chronic, probably result from an immune attack against antigens of the peripheral nervous system. Specific antibodies in serum that react with the peripheral nervous system have been described in a number of inflammatory neuropathies. We review the pathophysiological significance of auto-antibodies and discuss their use for the diagnosis of patients with peripheral neuropathy.

Topics: Ataxia; G(M1) Ganglioside; Humans; Immunoassay; Immunoglobulin M; Motor Neurons; Neuritis; Paraproteinemias; Peripheral Nervous System Diseases

Bickerstaff's brainstem encephalitis (BBE) is a Guillain-Barré syndrome (GBS) spectrum disorder associated with predominantly central nervous system predilection. Patients exhibit a variable constellation of depressed consciousness, bilateral external ophthalmoplegia, ataxia and long tract signs. Although the pathophysiology is not fully understood, it has been associated with anti-GQ1b antibodies in two-thirds of patients. We present a patient with clinical features consistent with BBE and positive anti-GM1 and anti-GD1a antibodies. A diagnostic approach to the acutely unwell patient with brainstem encephalitis is explored in this clinical context with a literature review of the aforementioned ganglioside antibody significance. Intravenous immunoglobulin therapy is highlighted in BBE using up-to-date evidence-based extrapolation from GBS.

Topics: Adult; Ataxia; Autoantibodies; Brain Stem; Diagnosis, Differential; Electroencephalography; Encephalitis; G(M1) Ganglioside; Gangliosides; Glasgow Coma Scale; Humans; Male; Ophthalmoplegia

Topics: Adult; Anemia, Hemolytic, Autoimmune; Anti-Inflammatory Agents; Ataxia; Autoantibodies; Autoantigens; Autoimmune Diseases of the Nervous System; Diagnosis, Differential; Female; G(M1) Ganglioside; Humans; Ophthalmoplegia; Prednisone

Topics: Antibodies; Ataxia; Child; G(M1) Ganglioside; Gangliosides; Humans; Male; Myoclonus; Ocular Motility Disorders

Monospecific IgG antibodies to GD1b ganglioside (GD1b-specific antibodies) have been found in patients with acute ataxic neuropathy and Guillain-Barré syndrome, but the association of the GD1b-specific antibodies with specific neurological conditions has yet to be established. We tested sera from more than 10,000 patients with various neurological disorders, and found six sera, which contained IgG antibodies to GD1b, but not to LM1, GM1, GM1b, GD1a, GalNAc-GD1a, GT1a, GT1b and GQ1b. All six patients who carried GD1b-specific antibodies presented with acute onset of ataxia and monophasic course of the illness, of whom five demonstrated cerebellar-like ataxia. Four patients had antecedent symptoms of upper respiratory tract infection. The six patients demonstrated areflexia, and four complained of distal numbness. All the six patients who had the GD1b-specific antibodies carried IgG antibodies to complex of GQ1b/GM1 and GT1a/GM1. GD1b-specific antibodies were significantly absorbed by GQ1b/GM1 and GT1a/GM1 and anti-GQ1b/GM1 and -GT1a/GM1 antibodies were absorbed by GD1b. In conclusion, the GD1b-specific antibodies, which recognizes GQ1b/GM1 or GT1a/GM1 complex, are associated with acute ataxia.

Topics: Adult; Aged; Antibodies; Ataxia; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Humans; Male; Middle Aged; Retrospective Studies; Statistics, Nonparametric

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

Miller Fisher syndrome is a clinical variant of Guillain-Barré syndrome, characterized by acute-onset ophthalmoplegia, ataxia, and areflexia. It results from an immune response to a cross-reactive antigen between GQ1b ganglioside in human neurons and lipo-oligosaccharides of certain bacteria, e.g., Campylobacter jejuni. Anti-GQ1b antibody is a powerful diagnostic marker for Miller Fisher syndrome. However, only a small number of anti-GQ1b-negative Miller Fisher syndrome cases are documented. A 13-year-old boy demonstrated typical clinical features of Miller Fisher syndrome 1 week after C. jejuni enteritis, but was anti-GQ1b and anti-GM1b antibody-negative.

Topics: Ataxia; Blepharoptosis; Campylobacter Infections; Campylobacter jejuni; Child; Diplopia; G(M1) Ganglioside; Gangliosides; Humans; Male; Miller Fisher Syndrome; Neurologic Examination; Reflex

Antiganglioside antibodies are found in various neurological disorders that constitute a continuum from peripheral neuropathy to encephalitis. However, nystagmus has rarely been described in patients with ataxia associated with antiganglioside antibodies.. From January 2008 to July 2009, we identified 3 patients with acute ataxia and nystagmus in 2 University Hospitals of Korea, who were found to have anti-GD1b, anti-GM1, or anti-GQ1b antibodies.. In addition to acute ataxia, all 3 patients showed various combinations of nystagmus, which included central positional nystagmus (n = 3), vertical nystagmus (n = 1), and periodic alternating nystagmus (n = 1). The spontaneous and positional nystagmus were mostly detectable only with the elimination of fixation and magnification of the eyes using video goggles. Two patients also exhibited gaze-evoked nystagmus that was noticeable without the aid of video goggles. Patients had serum IgG antibodies to GD1b, GM1, or GQ1b. Cerebrospinal fluid examination, nerve conduction studies, and brain MRI were normal. In all patients, the symptoms and signs resolved over 3-12 months.. Various forms of nystagmus with acute ataxia may be a sole or predominant manifestation of disorders related to antiganglioside antibodies. The nystagmus indicates a central pathology involving the cerebellum or brainstem in this antibody-associated disorder. Antiganglioside antibodies should be measured in patients with nystagmus and acute ataxia of undetermined etiology.

Topics: Acute Disease; Adolescent; Adult; Ataxia; Autoantibodies; Autoantigens; Autoimmune Diseases; Cerebrospinal Fluid; Dizziness; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin G; Male; Nystagmus, Pathologic; Postural Balance; Sensation Disorders; Vertigo

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: Animals; Ataxia; Cerebral Cortex; Chromatography, Thin Layer; G(M1) Ganglioside; G(M2) Ganglioside; Gangliosides; Gangliosidoses; Heterozygote; Hexosaminidases; Humans; Leukocytes; Swine; Swine Diseases