gq1b-ganglioside and Blepharoptosis

Topics: Action Potentials; Aged; Asymptomatic Diseases; Ataxia; Autoantibodies; Blepharoptosis; Calcium Channels, P-Type; Calcium Channels, Q-Type; Diplopia; Electrodiagnosis; Electromyography; Female; Gangliosides; Humans; Hypesthesia; Lambert-Eaton Myasthenic Syndrome; Median Nerve; Miller Fisher Syndrome; Muscle Weakness; Mydriasis; Neural Conduction; Oculomotor Muscles; Tibial Nerve; Ulnar Nerve

We describe the case of an 85-year-old gentleman admitted with bilateral ptosis and complete bilateral ocular paralysis. Initial differential diagnoses included myasthenia gravis, diabetic cranial neuropathy, an ischaemic event and possible occult neoplasm. Investigations did not support any of the differentials and Miller Fisher syndrome (MFS) was considered. Anti-GQ1b IgG antibody was positive, supporting the possibility of anti-ganglioside syndrome. This gentleman was treated with intravenous immunoglobulin (IVIG) and made a full recovery.

Topics: Aged, 80 and over; Autoantibodies; Biomarkers; Blepharoptosis; Gangliosides; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Male; Miller Fisher Syndrome; Ophthalmoplegia; Predictive Value of Tests; Treatment Outcome

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

Ophthalmoplegia without ataxia, areflexia or both has been designated as atypical Miller Fisher syndrome (MFS) or acute ophthalmoplegia (AO). This entity, first reported by Chiba et al. is associated with anti-GQ1b IgG antibodies.We report a patient with isolated acute ophthalmoplegia with high titer of anti-GQ1b IgG antibody activity in the acute phase in whom treatment with intravenous immunoglobulin (IVIg) led to the clinical recovery and the decrease in antibody titer.

Topics: Acute Disease; Adult; Blepharoptosis; Diplopia; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Miller Fisher Syndrome; Neural Conduction; Neurologic Examination

Topics: Acute Disease; Adult; Autoantibodies; Autonomic Nervous System Diseases; Blepharoptosis; Bradycardia; Cranial Nerve Diseases; Cranial Nerves; Diplopia; Disease Progression; Early Diagnosis; Gangliosides; Humans; Immunoglobulins, Intravenous; Male; Miller Fisher Syndrome; Oculomotor Nerve Diseases; Ophthalmoplegia; Respiratory Tract Infections; Treatment Outcome; Vagus Nerve Diseases; Virus Diseases

Anti-GQ1b IgG antibody syndrome comprises a wide range of diseases presenting with ophthalmoplegia and ataxia. Anti-GQ1b antibodies have been strongly associated in the literature with Miller Fisher Syndrome, with acute ophthalmoplegia associated with Guillain-Barré syndrome, and with isolated ophthalmoplegia. Acute ophthalmoplegia presents as various combinations of external and internal ophthalmoplegia. Reported here is a novel case of isolated ptosis as a manifestation of ophthalmoplegia. The present finding of bilateral ptosis and areflexia with anti-GQ1b IgG antibody positivity helps confirm the existence of the syndrome. Further research is needed on diagnosis and treatment.

Topics: Acute Disease; Ataxia; Autoantibodies; Autoimmune Diseases of the Nervous System; Blepharoptosis; Child, Preschool; Gangliosides; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Immunologic Factors; Male; Ophthalmoplegia; Reflex; Syndrome; Treatment Outcome

Topics: Autoantibodies; Blepharoptosis; Diagnosis, Differential; Electromyography; Gangliosides; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Miller Fisher Syndrome; Motor Neurons; Neuromuscular Junction; Neuromuscular Junction Diseases; Oculomotor Muscles; Ophthalmoplegia; Peripheral Nerves; Predictive Value of Tests; Reflex, Abnormal