안중증근무력증의 안과적 임상 양상
Ophthalmologic Manifestations of Ocular Myasthenia Gravis
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목적: 안중증근무력증 환자의 안과적 임상 양상을 알아보고자 하였다. 대상과 방법: 2011년 1월부터 2022년 8월까지 본원 안과 및 신경과에 내원하여 안중증근무력증으로 진단받은 환자 중 6개월 이상추적 관찰이 가능하였던 58명을 대상으로 안과적 임상 양상 및 경과를 후향적으로 분석하였다. 결과: 총 58명의 환자에서 발병 시 평균 연령은 55.52 ± 15.60세로 남성 29명, 여성 29명이었다. 항아세틸콜린수용체항체검사에서양성 31명으로 양성률은 53.45%였고, 반복신경자극검사는 양성 19명, 음성 38명으로 33.33%에서 이상을 보였다. 항체 양성군에서전신근무력증으로 더 많이 진행하였으며(p=0.011), 더 많은 흉부 이상 소견을 보였다(p<0.001). 복시와 안검하수가 동반된 복합군에서수평, 수직복시가 모두 있는 경우가 많았으며(p<0.001), 사시의 양상이 복시 증상 단일군에서는 내사시, 복합군에서는 외사시가 가장많아 차이가 있었다(p=0.029). 또한 증상 복합군에서 항체 농도가 높게 측정되었으며(p=0.034), 반복신경자극검사에서 이상 소견을보이는 경우가 많았고(p=0.022) 더 많은 흉부 이상 소견을 보였다(p=0.022). 결론: 안중증근무력증 환자에서 항아세틸콜린수용체항체 양성군에서 전신 중증근무력증으로 진행한 경우가 많았고, 복시와 안검하수가 동반된 복합군에서 항아세틸콜린수용체항체 농도가 높았으며, 수평사시와 수직사시가 함께 동반되는 경우가 많았다.
Abstract
Purpose: We investigated the ophthalmic manifestations observed in patients diagnosed with ocular myasthenia gravis. Methods: In total, 58 patients diagnosed with ocular myasthenia gravis visited the ophthalmology and neurology departments between January 2011 and August 2022. Patients were followed for > 6 months; their ophthalmic manifestations were analyzed retrospectively. Results: The study included 29 males and 29 females with a mean age of 55.52 ± 15.60 years. Among the patients, 31 tested positive for anti-acetylcholine receptor antibodies, resulting in a positive rate of 53.45%. Abnormalities in repeated nerve stimulation tests were observed in 33.33% of the patients. The antibody-positive group exhibited a higher frequency of progression to generalized myasthenia gravis (p = 0.011) and a higher incidence of chest abnormalities (p < 0.001) compared to the antibody- negative group. Horizontal and vertical diplopia were commonly observed in a complex group of patients with diplopia and blepharoptosis (p < 0.001); there was a difference in the pattern of strabismus between the diplopia single group with esotropia and the complex group with exotropia (p = 0.029). In addition, the combination group demonstrated a higher antibody titer (p = 0.034), a higher frequency of abnormalities in repeated nerve stimulation tests (p = 0.022), and a higher incidence of chest abnormalities (p = 0.022). Conclusions: The anti-acetylcholine receptor antibody-positive group had a higher incidence of progression to generalized myasthenia gravis; moreover, the complex group of patients with diplopia and ptosis exhibited elevated levels of anti-acetylcholine receptor antibodies and frequently accompanied both horizontal and vertical strabismus.
참고문헌 (27)
- Berrih-Aknin S, "Diagnostic and clinical classification of autoimmune myasthenia gravis", J Autoimmun, 48-49, pp.143~148 (2014)
- Kupersmith MJ, "Development of generalized disease at 2 years in patients with ocular myasthenia gravis", Arch Neurol, 60, pp.243~248 (2003)
- Vaphiades MS, "Ocular myasthenia gravis", Curr Opin Ophthalmol, 23, pp.537~542 (2012)
- Kupersmith MJ, "Ocular motor dysfunction and ptosis in ocular myasthenia gravis : effects of treatment", Br J Ophthalmol, 89, pp.1330~1334 (2005)
- Wang LL, "Clinical features and prognosis of ocular myasthenia gravis patients with different phenotypes", Chin Med J(Engl), 128, pp.2682~2684 (2015)
- Kelly JJ Jr, "The laboratory diagnosis of mild myasthenia gravis", Ann Neurol, 12, pp.238~242 (1982)
- Barton JJ, "Ocular aspects of myasthenia gravis", Semin Neurol, 20, pp.7~20 (2000)
- Weinberg DA, "Ocular myasthenia : a protean disorder", Surv Ophthalmol, 39, pp.169~210 (1994)
- Cleary M, "The pattern of extra-ocular muscle involvement in ocular myasthenia", Strabismus, 16, pp.11~18 (2008)
- Oh SJ, "Electrophysiological and clinical correlation in myasthenia gravis", Ann Neurol, 12, pp.348~354 (1982)
- Kamarajah SK, "Ocular presentation of myasthenia gravis : a natural history cohort", Muscle Nerve, 57, pp.622~627 (2018)
- Evoli A, "Pattern of ocular involvement in myasthenia gravis with MuSK antibodies", J Neurol Neurosurg Psychiatry, 88, pp.761~763 (2017)
- 박수연, "Incidence and Prevalence of Myasthenia Gravis in Korea: A Population-Based Study Using the National Health Insurance Claims Database", Journal of Clinical Neurology, 12(3), pp.340~344 (2016), 대한신경과학회[DOI]
- 김승민, "The Epidemiology of Myasthenia Gravis in Korea", Yonsei Medical Journal, 57(2), pp.419~425 (2016), 연세대학교의과대학[DOI]
- de Meel RHP, "Ocular weakness in myasthenia gravis : changes in affected muscles are a distinct clinical feature", J Neuromuscul Dis, 6, pp.369~376 (2019)
- Ariatti A, "Prognostic factors and healthrelated quality of life in ocular myasthenia gravis(OMG)", Int J Neurosci, 124, pp.427~435 (2014)
- Peeler CE, "Clinical utility of acetylcholine receptor antibody testing in ocular myasthenia gravis", JAMA Neurol, 72, pp.1170~1174 (2015)
- Marcuse F, "The association between anti-acetylcholine receptor antibody level and clinical improvement in myasthenia gravis", Eur J Neurol, 29, pp.1187~1197 (2022)
- Kim JH, "Childhood ocular myasthenia gravis", Ophthalmology, 110, pp.1458~1462 (2003)
- Mazzoli M, "Factors affecting outcome in ocular myasthenia gravis", Int J Neurosci, 128, pp.15~24 (2018)
- Hong YH, "Prognosis of ocular myasthenia in Korea : a retrospective multicenter analysis of 202 patients", J Neurol Sci, 273, pp.10~14 (2008)
- Carr AS, "A systematic review of population based epidemiological studies in myasthenia gravis", BMC Neurol, 10, pp.46~ (2010)
- Nair AG, "Ocular myasthenia gravis : a review", Indian J Ophthalmol, 62, pp.985~991 (2014)
- Lazaridis K, "Myasthenia gravis : autoantibody specificities and their role in MG management", Front Neurol, 11, pp.596981~ (2020)
- Kerty E, "EFNS", ENS guidelines for the treatment of ocular myasthenia, 2014, pp.21, 687~693
- Farmakidis C, "Treatment of myasthenia gravis", Neurol Clin, 36, pp.311~337 (2018)
- Benatar M, "Efficacy of prednisone for the treatment of ocular myasthenia(EPITOME) : a randomized, controlled trial", Muscle Nerve, 53, pp.363~369 (2016)
