내경동맥 협착이 동반된 비전형적 Miller-Fisher 증후군 1례 (Atypical Miller-Fisher Syndrome)
A Case of Atypical Miller-Fisher Syndrome with Internal Carotid Artery Stenosis
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밀러-피셔 증후군은 눈근육마비, 조화운동못함증, 무반사를 3대 증상으로 하는 질환으로 길랑 바레 증후군, Bickerstaff 뇌줄기뇌염과 함께 Anti- GQ1b IgG 항체 증후군에 속한다. 그러나 모든 밀러 피셔 증후군이 3대 증상을 갖고 있지 않으며, 경우에 따라서는 두 가지나 한가지만의 임상양상을 갖고 있는 경우도 있다. 이를 두고 비전형적 밀러-피셔 증후군이라 하며, 진단은 특징적인 병력, 임상양상 및 면역학적 검사로 한다. 밀러-피셔 증후군은 일반적으로 심각한 합병증을 남기지 않지만 경우에 따라서는 소아에서 호흡부전으로 진행할 수 있기에 추적관찰이 필요하다. 저자들은 8세 남아에서 내경동맥 협착이 동반된 비전형적 밀러-피셔 증후군 환아를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Abstract
Miller-Fisher syndrome is an acute poly-neuropathy typically characterized by the triad of ophthalmoplegia, ataxia, and areflexia. Other poly-neuropathy diseases include Guillain-Barre syndrome and Bickerstaff brainstem encephalitis. However, not all cases of Miller-Fisher syndrome have the typical three symptoms. These diseases have been differentiated by clinical manifestations and anti-GQ1b IgG antibody testing. In Miller-Fisher syndrome, anti-GQ1b IgG antibody is present in over 95% of patients; however, the frequency is not so high in normal subjects or in those with other poly-neuropathy diseases. Infectious illnesses, such as respiratory diseases and gastrointestinal diseases, usually precede Miller-Fisher syndrome. In general, Miller-Fisher syndrome is a self-limiting illness; however, a few cases of Miller-Fisher syndrome progressing to respiratory failure have been reported, particularly in children. In this case, we report abnormal angiographic findings. We report a case of atypical Miller-Fisher syndrome with internal carotid artery stenosis treated with prednisolone.
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