밀러-피셔 증후군의 임상양상과 치료경과
The Characteristics and Prognosis of Miller Fisher Syndrome
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목적: 밀러-피셔 증후군으로 진단된 환자의 임상양상과 경과를 보고하고자 한다. 대상과 방법: 급성안운동 마비, 운동실조, 반사소실을 모두 보인 밀러-피셔 증후군 환자의 의무기록을 바탕으로 안과적 이상과 신경학적 이상을 분석하고, 항-GQ1b 항체를 비롯한 검사 소견을 분석하고 치료 경과를 조사하였다. 결과: 총 10명의 환자 중 9명에서 선행감염이 있었고, 전신적인 감염질환을 앓은 후 급성안운동 이상으로 인한 복시 소요기간은 평균11 ± 9.7일 지속되었다. 외안근 마비의 양상은 양안 모두를 침범한 경우가 7명(70%)이었고, 수평사시와 수직사시가 동반된 경우가 5명(50%)이었다. 다른 임상소견으로 동공마비 4명(40%), 안검하수 4명(40%), 사지위약 3명(30%), 구음장애 3명(30%), 안면마비가 1명(10%) 관찰되었다. 뇌 자기공명영상검사상 갓돌림신경 조영증강과 뇌척수액 검사상 알부민세포해리가 각각 2명(20%)이었다. 혈청검사상 항-GQ1b 항체가 8명(80%)에서 양성소견을 보였다. 총 6명(60%)의 환자는 정맥면역글로불린 치료를 시행하였고, 4명(40%)의 환자는 경과관찰하였다. 안운동마비로 인한 정면 주시 복시의 평균 회복 기간은 치료군에서 2.9 ± 1.2개월, 경과관찰군에서 3.1 ± 1.7개월이었으며, 운동실조는 치료군에서 1 ± 0.4개월, 경과관찰군에서 1 ± 0.9개월로 두 경우 모두 유의한 차이를 보이지 않았다(각각 p>0.05). 결론: 수 주 이내 감염이 발생한 과거력이 있으면서 안운동마비, 운동실조, 반사소실 등의 증상을 보이는 환자의 경우 임상적으로 밀러-피셔 증후군을 의심해야 하고, 항-GQ1b 항체검사를 시행하는 것이 진단에 도움이 되며, 최종적인 예후는 치료 여부와 무관하게 비교적 양호하였다.
Abstract
Purpose: In the present study, the clinical characteristics and prognosis of patients clinically diagnosed with classic Miller Fisher syndrome were evaluated. Methods: We retrospectively investigated the clinical and laboratory findings as well as treatment outcomes using the medical records of patients diagnosed with Miller Fisher syndrome. Symptom triad including acute ophthalmoplegia, ataxia, and areflexia were evaluated. Results: This study included 10 patients. Nine patients had antecedent infectious illness which took an average of 11 ± 9.7 days for onset of diplopia from antecedent infectious systemic illness. Seven patients showed bilateral paralytic strabismus. Specifically, 5 patients showed the involvement of vertical and horizontal extraocular muscles. Pupil impairment and blepharoptosis were observed in 4 patients, limb weakness in 3 patients, dysarthria in 3 patients and facial palsy in 1 patient. Two patients showed contrast enhancement of the abducens nerve on brain magnetic resonance imaging (MRI) and 2 patients showed albumin-cell dissociation on cerebrospinal fluid (CSF) analysis. Eight patients had anti-GQ1b antibodies in their blood serum analysis. Six patients were treated with intravenous immunoglobulins and the other patients were observed with regular follow- ups. The duration of diplopia was 2.9 ± 1.2 months in the treatment group and 3.1 ± 1.7 months in the control group (p > 0.05). The duration of ataxia was 1 ± 0.4 months in the treatment group and 1 ± 0.9 months in the control group (p > 0.05). Conclusions: Miller Fisher syndrome should be considered in patients with antecedent infection; acute ophthalmoplegia, ataxia and areflexia as well as anti-GQ1b antibody can be helpful for diagnosis. Final outcomes in the treated group were not significantly different from the control group and all patients showed good final outcomes.
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