J Korean Ophthalmol Soc > Volume 60(12); 2019 > Article
Journal of the Korean Ophthalmological Society 2019;60(12):1301-1306.
DOI: https://doi.org/10.3341/jkos.2019.60.12.1301    Published online December 15, 2019.
Primary Localized Amyloidosis in Extraocular Muscles.
Sun Hyup Han, Yoon Duck Kim, Kyung In Woo
Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. eyeminded@skku.edu
외안근을 침범한 원발 국소아밀로이드증
한선협 · 김윤덕 · 우경인
성균관대학교 의과대학 삼성서울병원 안과학교실
Correspondence:  Kyung In Woo,
Email: eyeminded@skku.edu
Received: 9 August 2019   • Revised: 4 September 2019   • Accepted: 6 December 2019
Abstract
PURPOSE
Primary localized amyloidosis is a disease characterized by the deposition of abnormal protein fibrils in a specific tissue without systemic involvement. We report a rare case of primary amyloidosis involving the extraocular muscle. CASE SUMMARY: A 51-year-old female visited our clinic due to diplopia. There was a 3 mm exophthalmos and restricted ocular motility in infraduction. Right hypertropia with 25 prism diopters was observed in the primary position. Orbital imaging scans showed fusiform enlargement of the inferior and medial rectus muscles with significant contrast enhancement in the right eye. A calcified nodule embedded in the medial rectus muscle was observed. Empirical steroid pulse therapy was conducted, but the patient showed little response. Subsequently, an incisional biopsy of inferior rectus muscle was performed. A histopathological examination showed amyloid deposits in hematoxylin and eosin staining, and a positive birefringence in Congo-red staining under polarized light, confirming amyloidosis. A systemic work up showed no evidence of systemic amyloidosis besides increased levels of rheumatoid factor. CONCLUSIONS: When infiltrative myopathy of the extraocular muscle is suspected in a patient with exophthalmos and enlargement of extraocular muscles, primary amyloidosis should be considered in the differential diagnosis.
Key Words: Extraocular muscles;Infiltrative myopathy;Primary amyloidosis


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