[Overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome].

Abstract

A 29-year-old female developed diplopia, nasal voice and gait disturbance after an upper respiratory infection. On admission, she presented with bilateral external ophthalmoplegia, slight bilateral facial nerve palsy, dysarthria, dysphagia, cervical and brachial muscle weakness, ataxia and areflexia. She had serum anti-GT1a, anti-GQ1b and anti-galactocerebroside IgG antibodies. She was diagnosed with an overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Intravenous immunoglobulin therapy was effective for the ophthalmoplegia and ataxia, but did not improve the bilateral facial nerve palsy and brachial muscle weakness. The facial nerve palsy clearly worsened despite improvement in other symptoms, and therefore high-dose intravenous methylprednisolone therapy was added. The distinct response to treatment may be caused by different activity, production, clearance and reactivity to intravenous immunoglobulin of the autoantibodies. The present case suggests that treatment response and patterns of recovery differ according to the causative anti-ganglioside antibodies.

• Authors: Koji Shinoda, Hiroyuki Murai, Ken-Ichi Shibata, Shoko Samejima, Shuji Kaneto, Nobuyoshi Takashima, Kimihiro Tanaka
• Journal: Rinsho shinkeigaku = Clinical neurology (Rinsho Shinkeigaku) Vol. 52 Issue 1 Pg. 30-3 ( 2012) ISSN: 1882-0654 [Electronic] Japan
• PMID: 22260976 (Publication Type: Case Reports, Journal Article)

Chemical References

• Gangliosides
• Immunoglobulins, Intravenous
• MYL4 protein, human
• Myosin Light Chains
• Proteins
• GQ1b ganglioside
• Methylprednisolone

Topics

• Adult
• Female
• Gangliosides (immunology)
• Guillain-Barre Syndrome (complications, drug therapy, immunology, physiopathology)
• Humans
• Immunoglobulins, Intravenous (administration & dosage)
• Methylprednisolone (administration & dosage)
• Miller Fisher Syndrome (complications, drug therapy, immunology, physiopathology)
• Myosin Light Chains
• Proteins (immunology)
• Pulse Therapy, Drug
• Treatment Outcome