A Case of Leigh's Disease Accompanied by Limitation of Bilateral Ocular Movement.

Chang, Yong Ik; Lee, Young Chun

Case Report

Journal of the Korean Ophthalmological Society 2001;42(6):927-931.
Published online June 1, 2001.

A Case of Leigh's Disease Accompanied by Limitation of Bilateral Ocular Movement.

Yong Ik Chang, Young Chun Lee

Department of Ophthalmology, Uijongbu St. Mary's Hospital, College of Medicine, The Catholic University of Korea.

양안 안구운동 장애로 표현된 레이씨 병 1 예

장용익(Yong Ik Chang),이영춘(Young Chun Lee)

Abstract

PURPOSE
Leigh's disease is a mainly autosomal recessive inherited progressive disorder, with onset in the first 2 years of life, and has ocular signs such as nystagmus, gaze palsy, nuclear ophthalmoplegia and various neurologic signs. This disease finally results in death due to respiratory difficulty after repeated fever, dehydration, poor oral intake, lethargy, and convulsion. Leigh's disease is one of central nervous diseases considered in case infants or children who have paralytic esotropia or rapidly progressive limitation of ocular movement. METHODS: Authors experienced a case of 35-month-old male who had had 15 of right esotrophia and limitation of ocular movement in abduction for 1 month. At first visit, we diagnosed as a right 6th nerve palsy. However, the patient showed rapidly progressive limitation of ocular movement in all direction except in adduction and esotropia in both eyes. RESULTS: The patient presented ocular signs earlier than other neurologic symptoms and signs. He was consulted to the department of pediatric neurology and radiology, and diagnosed as Leigh's disease.

Key Words: Leigh's disease;Limitation of ocular movement