gq1b-ganglioside and Paralysis

Topics: Gangliosides; Humans; Male; Middle Aged; Miller Fisher Syndrome; Mydriasis; Neurologic Examination; Paralysis; Pharyngeal Diseases

Bickerstaff's brain-stem encephalitis is usually a monophasic post-viral inflammatory illness characterized by progressive ophthalmoplegia, ataxia and disturbance of consciousness (or hyper-reflexia). Since the clinical spectrum of Bickerstaff encephalitis may overlap with the Miller-Fisher and Guillain-Barré syndromes, the presence of anti- GQ1b antibodies and abnormal brain MRI can help to support its diagnosis. However, absence of anti-GQ1b antibodies and normal MRI do not exclude the diagnosis, which remains based on clinical criteria and exclusion of other etiologies. We report a case of recurrent Bickerstaff's brainstem encephalitis with no identifiable antecedent illness, and overlapping features of Miller Fisher and Guillain-Barré syndromes, in the presence of negative anti-GQ1b antibodies and repeatedly normal MRI of the brain.

Topics: Brain Stem; Consciousness; Electroencephalography; Encephalitis; Gangliosides; Glasgow Coma Scale; Humans; Male; Middle Aged; Miller Fisher Syndrome; Neurologic Examination; Ophthalmoplegia; Paralysis; Physical Therapy Modalities; Recurrence

A patient with acute oropharyngeal palsy associated with internal ophthalmoplegia was reported. A 13-year-old boy had fever and diarrhea for two days. Ten days after resolution of these symptoms, he noticed difficulty in speaking (day 1). Neurological findings on day 4 included bilateral mydriasis, right abducens nerve palsy, nasal voice with absent pharyngeal reflex. Although superficial sensation was preserved, vibratory sensation was reduced in distal limbs. Tendon reflexes were generally absent. Neither ataxia nor dysautonomia was seen. Serum anti-glycolipid antibody assay on day 4 disclosed elevated IgG antibodies to GQ1b and GT1a. His cerebrospinal fluid on day 21 contained 6 mononuclear cells/microl with 137 mg/dl of total protein. Nerve conduction study on day 5 showed minimal sensory nerve involvement. Quantitative sudomotor axon reflex test was normal in the lower extremities. Low dose pilocarpine eyedrops dilated his pupils. Although mild cerebellar-like ataxia appeared on day 5, intravenous immunoglobulin (0.4 g/kg/day for four days) rapidly improved his neurological abnormalities. IgG anti-GQ1b antibody might contribute not only oropharyngeal weakness but also internal ophthalmoplegia in this patient.

Topics: Acute Disease; Adolescent; Autoantibodies; Gangliosides; Humans; Immunoglobulin G; Male; Miller Fisher Syndrome; Ophthalmoplegia; Oropharynx; Paralysis

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