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Journal of the Korean Child Neurology Society 2012;20(1):39-42.
Published online March 30, 2012.
Atypical Miller-Fisher Syndrome Presenting as an Isolated Internal Ophthalmoplegia Following Epstein-Barr Virus Infection.
Hye Jin Park, Kye Hyang Lee
Department of Pediatrics, College of Medicine, Daegu Catholic University, Daegu, Korea. rosalia@cu.ac.kr
Acute external ophthalmoplegia, areflexia, and ataxia compose the classic Miller-Fisher syndrome (MFS). Pupillary dysfunction is present in half of patients with MFS. However, isolated internal ophthalmoplegia is rare in children with MFS. We report a case with MFS presenting with isolated internal ophthalmoplegia following an acute Epstein-Barr virus (EBV) infection. An 11-year-old girl with flu-like symptoms was diagnosed with having an acute EBV infection on prior admission. After discharge, she complained of anorexia, poor oral intake and dizziness associated with blurred vision when in the upright position. Physical examination on readmission 10 days later showed anisocoric, mydriatic pupils with an abnormal light reflex. The patient displayed no external ophthalmoplegia, ataxia or areflexia. Cerebrospinal fluid analysis showed elevated protein with no white blood cells. Serum anti-GQ1b antibody was negative. MFS was diagnosed under clinical findings and CSF albuminocytologic dissociation. Isolated internal ophthalmoplegia can be the sole manifestation of MFS. Therefore, MFS must be considered in the differential diagnosis for children presenting with unexplained acute internal ophthalmoplegia.
Key Words: Miller Fisher syndrome, Ophthalmoplegia, Anisocoria, Mydriasis


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