[A case of Fisher syndrome showing pharyngeal-cervical-brachial weakness with an elevation of anti-GQ 1 b and anti-GT 1 a antibodies].
A 15-year-old boy developed ataxic gait, diplopia and hoarseness. Within 3 days after the onset, he had additional symptoms of dysphagia and dysarthria. He was admitted to our hospital 7 days after the onset of the disease. On admission, he had total ophthalmoplegia, ataxia, areflexia, facial diplegia, bulbar palsy and weakness of the neck and upper arms. Serum anti-GQ 1 b and anti-GT 1 a antibodies were significantly elevated. A diagnosis of Fisher syndrome associated with pharyngeal-cervical-brachial weakness was made. He was placed on a high dose of intravenous immunoglobins (12.5 g/day x 2 days) and had steroid pulse therapy (methylprednisolone 1 g x 3 days), which resulted in an almost complete recovery. There have been no reports of Fisher syndrome associated with brachio-pharyngeal-palsy. As in the case of the pharyngeal-cervical-brachial variant of Guillain Barré syndrome, anti-GT 1 a antibodies may be associated with Fisher syndrome with pharyngeal-cervical-brachial weakness.
Furiya
Y
Y
Department of Neurology, Hirai Hospital.
Murakami
N
N
Kataoka
H
H
Suzumura
A
A
Takayanagi
T
T
D000293:Adolescent; D001132:Arm; D001323:Autoantibodies / Q000097:blood*; D005732:Gangliosides / Q000276:immunology*; D006801:Humans; D008297:Male; D019846:Miller Fisher Syndrome / Q000150:complications / Q000276:immunology*; D009123:Muscle Hypotonia / Q000209:etiology*; D009333:Neck; D010609:Pharyngeal Muscles
D001323:Autoantibodies; D005732:Gangliosides; C039129:GQ1b ganglioside