MRI follow-up study and 1H-MR spectroscopic finding in a patient with Kearns-Sayre syndrome

J Korean Neurol Assoc > Volume 16(3); 1998 > Article

Journal of the Korean Neurological Association 1998;16(3): 388-391.

Kearns-Sayre 증후군 환자의 추적 자기공명영상 및 수소 자기공명분광영상 소견

최 철희, 선우 일남, 김 태승*, 전 평**

연세대학교 의과대학 신경과학 교실, 병리학 교실*, 진단방사선과학 교실**

MRI follow-up study and 1H-MR spectroscopic finding in a patient with Kearns-Sayre syndrome

Chulhee Choi, M.D., Il Nam Sunwoo, M.D., Tae Seung Kim, M.D. *, Pyoung Jeon, M.D.**

Department of Neurology, Department of Pathology*, Department of Radiology** College of Medicine Yonsei University

Abstract

Serial Brain MRI was performed on a seventeen-year-old girl with Kearns-Sayre syndrome. At the age of 11, she complained bilateral ptosis. Two years later, bilateral blepharoplasty was done and brain MRI was taken. T2-weighted MRI sequence showed high signal intensity areas in the brainstem, thalamus and white matter of the cerebrum and cerebellum bilaterally. Four years later, chronic progressive external ophthalmoplegia developed and serial MRI and proton MRS were taken. Follow-up MRI showed similar but slightly progressed findings compared with previous films. The proton MR spectroscopic imaging demonstrated focal localization of abnormally increased lactate content in the involved area of the brain. Key words : Kearns Sayre syndrome, MR spectroscopy, lactate