Atypical Miller-Fisher Syndrome Presenting as an Isolated Internal Ophthalmoplegia Following Epstein-Barr Virus Infection = 급성 Epstein-Barr virus 감염 후 내안근마비로 내원한 비정형 Miller-Fisher증후군

국문 초록 (Abstract)

Miller-Fisher 증후군(MFS)은 급성 외안근 마비, 조화운동불능 및 심부건 반사 소실의 3가지 증상을 특징으로 하는 말초 신경 질환이다. 진단은 임상적인 특징으로서 3가지 증상이 있으면 내릴 수 있으며, 뇌척수액의 세포단백해리와 혈청 항 GQ1b 항체 양성의 2가지 검사를 통해 도움을 받을 수 있다. 하지만, 3주징 가운데 일부만 보이는 국소적인 형태의 MFS도 있다. 즉, 외안근 마비 단독으로 나타나거나, 조화운동불능 없이 외안근마비와 심부건 반사 소실의 증상으로만 발현하는 경우도 있다. 동공이상은 MFS 환자의 약 40%에서 동반되는 것으로 알려져있다. 저자들은 급성 Epstein-Barr virus 감염 이후에 발생한 양측 동공의 산대 및 빛반사 소실 및 감소의 증상만으로 발현한 비정형 Miller Fisher 증후군 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

다국어 초록 (Multilingual Abstract)

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.

목차 (Table of Contents)

• Abstract
• Introduction
• Case Report
• Discussion
• 한글요약
• References

참고문헌 (Reference)

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