Acute ophthalmoplegia, ataxia, and areflexia (Fisher syndrome) in childhood

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A case of ophthalmoplegia, ataxia, and areflexia known as Fisher syndrome in a 3½-year-old boy is reported. The clinical features, diagnosis, and outcome are described. The relationship of Fisher syndrome to acute idiopathic polyneuritis (the Guillain-Barré syndrome) is discussed. This case is unique in that the syndrome developed coincident with infectious mononucleosis. The need to consider infectious mononucleosis in any acute neurologic illness in childhood is stressed.

In 1956, Fisher1 described a syndrome characterized by the acute onset of total bilateral external ophthalmoplegia, severe ataxia, and absence of the deep tendon reflexes. Although the onset of the disorder was alarming, the course was benign. Since that time numerous cases have been reported2–11 with less than 10 cases occurring in children and none reported in association with infectious mononucleosis.

Case report

A 3½-year-old Caucasian boy had total bilateral external ophthalmoplegia and ataxia. Eight days prior to admission he had had a flu-like syndrome with pharyngitis, diarrhea, vomiting, and a fever of 101 F, which lasted 5 days. On the sixth day his gait became unsteady; a mild, generalized headache developed and he appeared to stare continuously. His family history and medical history were normal. Ophthalmologic examination revealed an alert, cooperative patient who identified the Allen card pictures at half the normal visual distance for his age. His binocular near visual acuity was 20/40 near Snellen equivalent at 14 inches. There was moderate bilateral ptosis to the lower pupillary margin, but both eyelids could be elevated on command. Complete bilateral external ophthalmoplegia . . .



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