Atypical Miller Fisher syndrome with complete bilateral ophthalmoplegia mimicking brainstem stroke

We report on three elderly patients with stroke-like onset of atypical Miller Fisher syndrome (MFS). The serum titer of anti-GQ1b IgG was markedly elevated in these patients. Their prognoses were sufficiently good with immunoadsorption therapy with or without intravenous immune globulin (IVIg) therapy. However, some neurological findings were not characteristic of typical MFS. Patient 1 suffered from prolonged dysesthesia in her left extremities, and Patients 2 and 3 showed no ataxia. Moreover, complete bilateral gaze limitation is rare in MFS. The sudden stroke-like onset along with the gaze limitation of these patients suggests that the unexpected elevation in the serum titer of anti-GQ1b IgG due to unknown immune dysregulation might have severely affected the third, fourth, and sixth nerves and this potent antibody rapidly attacked these nerves and induced stroke-like clinical features and complete ophthalmoplegia.