gq1b-ganglioside and Cranial-Nerve-Diseases

A 35-year-old male developed sensory abnormality of peripheral limbs and oral cavity after prior infection with diarrhea and cold symptoms. Hyperrhinolalia, nasopharyngeal reflux, double vision, and wobbling in walking rapidly progressed. Neurological examination revealed palatoplegia, omnidirectional ophthalmoplegia, hyperreflexia, sensory disturbance of extremities, and truncal and limb ataxia due to decreased deep sensation. A peripheral nerve conduction study found a slight decrease in sensory nerve action potential of the median nerve and a decrease in F wave frequency of the median nerve. Serum IgM-CMV antibody was positive on admission. After IVIg therapy, palatoplegia and ataxia markedly improved. In this case, GalNAc-GD1a and GM2 antibodies, which are often detected after CMV infection, were positive in addition to the GT1a and GQ1b antibodies, and it was assumed that these findings were associated with the palatoplegia, which is included in cranial nerve palsy. Pathophysiologically, the present case is considered to be an overlap with acute oropharyngeal palsy (AOP), which is a rare subtype of Guillain-Barre syndrome, and Fisher syndrome (FS). The clinical aspects of the present case suggest a continuous spectrum between AOP and FS.

Topics: Adult; Antibodies, Viral; Autoantibodies; Biomarkers; Cranial Nerve Diseases; Cytomegalovirus; Cytomegalovirus Infections; Diagnostic Techniques, Neurological; Disease Progression; Gangliosides; Humans; Immunoglobulin M; Immunoglobulins, Intravenous; Male; Median Nerve; Miller Fisher Syndrome; Neural Conduction

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

A 52-year-old man developed diplopia, a nasal voice, dysphagia, hoarseness and slight bilateral facial palsies. There was no ataxia, areflexia, limb weakness or sensory involvement. Serum anti-GQ1b IgG antibody was present. Treatment with intravenous immunoglobulin started, and the patient responded with a rapid resolution of symptoms. The diagnosis is consistent with polyneuritis cranialis which is considered to be a Guillain-Barre syndrome variant, a forme fruste, but very rare. The diagnosis can be difficult and a thorough investigation is required. Electrophysiological examination, laboratory evaluations, imaging and cerebrospinal fluid examination are often required in the investigations. Cranial neuropathy can be the presentation of many disorders. Determination of anti-ganglioside antibodies as anti-GQ1b is valuable to the diagnosis, and shows the association with the Guillain-Barre syndrome.

Topics: Autoantibodies; Cranial Nerve Diseases; Diagnosis, Differential; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Neuritis

Topics: Antibodies, Anti-Idiotypic; Chronic Disease; Cranial Nerve Diseases; Diagnosis, Differential; Female; Gangliosides; Humans; Immunoglobulin E; Immunoglobulins, Intravenous; Miller Fisher Syndrome; Plasmapheresis; Radiography; Sinusitis

Topics: Aged; Autoantibodies; Campylobacter Infections; Campylobacter jejuni; Cranial Nerve Diseases; Diagnosis, Differential; Diarrhea; Feces; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Paralysis