ganglioside--gd1b and Cerebellar-Ataxia

Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy which follows a precipitating event in approximately two thirds of cases. Although its pathogenesis is unclear, it is likely to be a consequence of an immune-mediated process. In the literature there are three case reports of GBS following subarachnoid hemorrhage, subdural hematoma, and facial bone fracture after head trauma.The unique feature of our case with GBS after subdural hematoma is the presence of cerebellar symptoms. We believe that GBS results from an aberrant immune response following trauma that somehow mistakenly attacks the nerve tissue of its host, and we discuss the effects of the trauma of head injury on cellular and humoral immunities and the absence of antiganglioside antibody (anti-GD1b IgG, which is accused of ataxia and cerebellar symptoms) in this case report.

Topics: Aged; Autoantibodies; Biomarkers; Brain; Cerebellar Ataxia; Cerebellum; Disease Progression; Female; Gangliosides; Guillain-Barre Syndrome; Head Injuries, Closed; Hematoma, Subdural, Acute; Humans; Magnetic Resonance Imaging; Nerve Fibers, Myelinated; Plasmapheresis; Subdural Space; Treatment Outcome

The anti-GD1b antibody is known to bind to the cerebellar granular layer or spinocerebellar Ia fibers. A few cases of anti-GD1b positive acute inflammatory demyelinating polyneuropathy with prominent cerebellar ataxia were reported. Recently, we encountered a middle-aged woman with Guillain Barré syndrome (GBS) with severe cerebellar ataxia and relatively mild motor weakness. Anti-GD1b Ig G antibody and anti-GM1 Ig G antibody titers were markedly elevated in her serum. She was diagnosed with acute motor axonal neuropathy (AMAN) with prominent cerebellar ataxia based on the results of the serial nerve conduction study suggesting axonal neuropathy. This case presents the clinico-pathogenic role of autoantibodies to the GD1b and the GM1 in acute inflammatory neuropathy.

Topics: Autoantibodies; Axons; Cerebellar Ataxia; Female; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulins, Intravenous; Middle Aged; Polyneuropathies

We report a 30-year-old woman who presented symptoms of oropharyngeal palsy and glove-stocking type sensory disturbance followed by acute cerebellar ataxia, the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), gastroenteric symptoms, urinary disturbance and orthostatic hypotension. She did not have any preceding infection. She was diagnosed as having Guillain-Barré syndrome with autonomic failure. Autonomic failure such as sinus tachycardia and nocturnal ventricular arrhythmia in addition to motor and sensory dysfunction was palliated by immunoadsorption. During the course of her illness, there were elevations of antiganglioside antibodies to GT1a and GQ1b in the IgG subclass, and to GD1b and GQ1b in the IgM subclass. The elevation of anti-GD1b antibody and anti-GQ1b antibody may be pathologically related to autonomic failure, cerebellar ataxia and SIADH.

Topics: Adult; Autoantibodies; Autonomic Nervous System Diseases; Cerebellar Ataxia; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Inappropriate ADH Syndrome

Topics: Adult; Antibodies; Cerebellar Ataxia; Diarrhea; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunotherapy; Neurologic Examination; Treatment Outcome

A 28-year-old man was admitted after developing acute onset unstable gait following acute enteritis. Neurological examination revealed mild weakness in four limbs, areflexia and ataxia. Serum obtained from the patient during the acute stage contained a high titer of anti-GD1b IgG antibody. Because the patient showed obvious cerebellar ataxia unrelated to muscle weakness, without ophthalmoplegia or proprioceptive sensory disturbance, we concluded that he had ataxic form of Guillain-Barré syndrome (GBS) (Richter, 1962). Although ataxic GBS is not an established conception, one should pay attention to the possible existence of such a rare GBS variant. It is necessary to accumulate additional case reports to clarify the association between ataxic GBS and anti-ganglioside antibodies.

Topics: Adult; Biomarkers; Cerebellar Ataxia; Diagnosis, Differential; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Treatment Outcome

Topics: Autoantibodies; Cerebellar Ataxia; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Neurologic Examination

A 79-year-old man with sensory dominant polyneuropathy, cerebellar ataxia, and palatal myoclonus had serum IgM M-protein that specifically bound to GM1, GD1b, and asialo-GM1. IgM with the same specificity was detected in his cerebrospinal fluid. Results of immunohistochemical studies showed specific binding of this monoclonal IgM to the cerebellar granular layer, dentate nucleus, inferior olive, and gray matter of the cerebrum and spinal cord. Monoclonal antibody GGR12, monospecific to GD1b, had an immunostaining distribution similar to that of the patient's IgM M-protein. The binding of M-protein may be associated with the development of cerebellar ataxia and palatal myoclonus in this patient.

Topics: Aged; Antigens, Tumor-Associated, Carbohydrate; Cerebellar Ataxia; Chromatography, Thin Layer; Enzyme-Linked Immunosorbent Assay; Epitopes; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin M; Immunohistochemistry; Male; Myelin Proteins; Peripheral Nervous System Diseases