Introduction. Acute complete
external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem.
Ophthalmoplegia has been reported with acute
ataxia in
Miller Fisher syndrome (MFS) and Bickerstaff brainstem
encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b
antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE. Case Presentation. An 8-year-old Caucasian boy presented with complete
external ophthalmoplegia without ptosis,
cerebellar ataxia, and a disturbance of consciousness. He had recently recovered from a confirmed Campylobacter jejuni
infection. On subsequent laboratory testing he was anti-GQ1b antibody negative. He had a recurrence of
diplopia at four-week follow-up. Conclusions. This patient's recurrence of
diplopia was treated with a five-week course of oral
corticosteroids which did not worsen his condition, and this may be a therapeutic option for similar patients. We will discuss the symptoms and treatment of reported pediatric cases of anti-GQ1b antibody negative cases of MFS and the variation between cases representing a spectrum of illness.