Miller-Fisher syndrome is a separate nosologic entity that is uncommon
in children (less than 1% of all Guillain-Barre cases). A seven-year-
old with an unremarkable medical history was admitted for a two-month
history of Miller-Fisher syndrome with cerebellar ataxia and slightly
impaired coordination. Electrophysiologic data were consistent with a
primarily axonal sensory neuropathy affecting all four limbs, accompan
ied with some degree of radicular involvement. A protein level of 0.56
gn was the only cerebrospinal fluid abnormality. Findings were normal
or negative from laboratory tests and serologic tests for bacteria. C
omputed tomography of the brain with thin slices through the posterior
fossa was normal. The outcome was favorable under methylprednisolone
therapy. At last follow-up three years later, the only abnormality was
absence of the deep tendon reflexes. The low incidence of Miller-Fish
er syndrome in children deserves to be emphasized. The cerebellar mani
festations and favorable course under methylprednisolone therapy are d
iscussed.