조화운동못함증을 동반하지 않은 급성 눈근육마비 3례
Three Cases of Acute Ophthalmoplegia without Ataxia
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초록
소아기에 갑자기 발생한 눈근육마비를 주소로 내원하는 경우 중추신경계감염, 혈관성 질환, 뇌종양 등 다양한 원인들이 있다. ‘조화운동못함증을 동반하지 않은 급성 눈근육 마비(AO)'는 Odaka 등4)의 정의에 따르면, '눈근육마비를 일으키는 다른 질환들이 감별 진단된 경우, 조화운동못함증이나 사지 쇠약이 동반되지 않는, 4주까지 진행되는 비교적 양측성으로 관찰되는 눈근육마비'라고 할 수 있다. 저자들은 다른 뇌신경마비 없이 급성 눈근육마비로 내원한 환자에서 항GQ1b 항체 양성이었던 1례와 항강글리오시드 항체검사 음성이었으나 조화운동못함증 보이지 않아 AO로 임상적 진단이 가능하였던 환아 2례를 경험하였기에 이를 감별진단하고 치료함에 있어서 필요한 여러 검사들과 함께 항강글리오시드 항체의 측정이 상기 진단을 감별하는데 도움이 되나 음성으로 나올 경우에도 AO를 배제할 수는 없음을 문헌 고찰과 함께 보고하는 바이다.
Abstract
cute ophthalmoplegia is caused by various etiologies; cerebrovascular diseases, tumors, infections, diabetes mellitus, multiple sclerosis, and myasthenia gravis. Acute ophthalmoplegia without ataxia(AO), regarded atypical Miller-Fisher syndrome, can be defined as a progressive, relatively symmetric ophthalmoplegia by 4 weeks without ataxia or limb weakness, on circumstance of ruling-out other diseases. The additional features that are strongly supportive of the diagnosis of AO are as follows: 1) a history of infectious symptoms within 4 weeks before the onset of neurological symptoms; 2) cerebrospinal fluid albuminocytologic dissociation; and 3) presence of anti-GQ1b IgG antibody. AO has been sporadically reported, but there is still short of information for its clinical and laboratory characteristics in children. We report three children with AO, who were presented with acute ophthalmoplegia without other abnormal neurologic symptoms. All of the patients met the diagnostic criteria of AO, but only one of them had a positive serum anti-GQ1b antibody. So, we need to suspect the diagnosis of AO, even in the cases with negative result of serum anti- GQ1b antibody.
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