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.