TY - JOUR
T1 - An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome
AU - Shinoda, Koji
AU - Murai, Hiroyuki
AU - Shibata, Ken Ichi
AU - Samejima, Shoko
AU - Kaneto, Shuji
AU - Takashima, Nobuyoshi
AU - Tanaka, Kimihiro
PY - 2012
Y1 - 2012
N2 - 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.
AB - 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.
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U2 - 10.5692/clinicalneurol.52.30
DO - 10.5692/clinicalneurol.52.30
M3 - Letter
C2 - 22260976
AN - SCOPUS:84863026683
SN - 0009-918X
VL - 52
SP - 30
EP - 33
JO - Clinical Neurology
JF - Clinical Neurology
IS - 1
ER -