Acute onset of a bilateral areflexical mydriasis in Miller-Fisher syndrome: a rare neuro-ophthalmologic disease.
Miller-Fisher syndrome (MFS) is characterized by variable ophthalmoplegia, ataxia, and tendon areflexia. It seems to be a variant of Guillain-Barré syndrome (GBS), but unlike in GBS, there is a primitive involvement of the ocular motor nerves, and in some cases there is brainstem or cerebellum direct damage. The unusual case of MFS in the current study started with a bilateral areflexical mydriasis and a slight failure of accommodative-convergence. Ocular-movement abnormalities developed progressively with a palsy of the upward gaze and a bilateral internuclear ophthalmoplegia to a complete ophthalmoplegia. In the serum of this patient, high titers of an IgG anti-GQ1b ganglioside and IgG anti-cerebellum. anti-Purkinje cells in particular, were found. The former autoantibody has been connected to cases of MFS, of GBS with associated ophthalmoplegia, and with other acute ocular nerve palsies. The anti-cerebellum autoantibody could explain central nervous system involvement in MFS. The role of these findings and clinical implications in MFS and in other neuro-ophthalmologic diseases are discussed.
Caccavale
A
A
Neuro-Ophthalmology and Retina Service, Magenta General Hospital, Milan, Italy.
Mignemi
L
L
D000065:Accommodation, Ocular; D000208:Acute Disease; D001323:Autoantibodies / Q000032:analysis; D002531:Cerebellum / Q000276:immunology; D002648:Child; D003290:Convergence, Ocular; D003907:Dexamethasone / Q000627:therapeutic use; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D019846:Miller Fisher Syndrome / Q000150:complications* / Q000276:immunology / Q000628:therapy; D015878:Mydriasis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D009886:Ophthalmoplegia / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D010956:Plasmapheresis; D011689:Purkinje Cells / Q000276:immunology
D001323:Autoantibodies; D005732:Gangliosides; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; C039129:GQ1b ganglioside; D003907:Dexamethasone