Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation

Toshiyuki Sakai, M.D.1), Masahide Kondo, M.D.1), Hidekazu Tomimoto, M.D.2) and Yuko Yamagishi, M.D.3)

1)Department of Neurology, Saiseikai Matsusaka General Hospital
2)Department of Neurology, Graduate School of Medicine, Mie University
3)Department of Neurology, Kinki University School of Medicine

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.
Full Text of this Article in Japanese PDF (361K)

(CLINICA NEUROL, 56: 694|697, 2016)
key words: Fisher syndrome, pharyngeal-cervical-brachial variant, urinary retention, anti-GQ1b antibody, intravenous immunoglobulin (IVIg)

(Received: 15-Mar-16)