An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation.
We report a 28-year-old woman with the overlap of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation. She showed total ophthalmoplegia, dysphagia, dysarthria, upper extremity weakness, cerebellar ataxia, slightly diminished superficial sensations in her hands and feet, urinary retention and constipation 14 days after preceding infection. Laboratory data showed elevations of antiganglioside antibodies to GT1b, GD1b, GQ1b, GD3 and GT1a in the IgG subclass. There was slight elevation of protein with no pleocytosis in cerebrospinal fluid. After administration of intravenous immunoglobulin (IVIg), only the titer of antiganglioside antibody to GQ1b was decreased, and she showed rapid improvement in dysphagia, urinary retention and constipation, and slow recovery in ophthalmoplegia and cerebellar ataxia. The elevations of antiganglioside antibodies to GQ1b may be pathologically related to autonomic involvement such as urinary retention and constipation in that IVIg seems to be effective. The present case suggests that GQ1b may also locate in the autonomic nerve that plays bladder and defecation functions, and that incidence of neurological symptoms and the response of treatment may differ according to each GQ1b localization.
Department of Neurology, Saiseikai Matsusaka General Hospital.
Toshiyuki
T
Sakai
Masahide
M
Kondo
Hidekazu
H
Tomimoto
Yuko
Y
Yamagishi
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D017776:Autonomic Pathways / Q000378:metabolism; D015415:Biomarkers / Q000032:analysis; D003248:Constipation / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D019846:Miller Fisher Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000178:diet therapy / Q000276:immunology; D016896:Treatment Outcome; D016055:Urinary Retention / Q000188:drug therapy / Q000209:etiology*
D001323:Autoantibodies; D015415:Biomarkers; D005732:Gangliosides; D016756:Immunoglobulins, Intravenous; C039129:GQ1b ganglioside