A Case of Sjogren-Larsson Syndrome

The Sjogren-Larsson syndrome is genetically determined syndrome with autosomal recessive inheritence and characterized by the three cardinal signs: congenital ichthyosis, spastic di/tetraplegia, and mental retardation. Ocular signs include ectropion, blepharitis, conjunctivitis, keratitis, and macular glistening spot. The authors have experienced a case of Sjogren-Larsson syndrome that showed classical triad and macular glistening spot in a 16 month old boy.

Clinical Analysis of Children's Perforating Eye Injuries in Middle Western Coast of Korea

We report a clinical ananlysis of children's perforating eye injuries which happened in middle western of Korea from Jan., 1988 to Apr., 1993. Total number of patients with perforating eye injuries was 40(40 eyes). The peak age group was 5-6 year of age. The cornea was the most common site of perforation. The most frequent cause of perforating eye injuries was knife and scissors(17.5%) and sharp pointed iron material(17.5%) and followed by glass(17.5%), wood(17.5%), pencil 7.5%), toy(5%), traffic accident(5%). According to this result obtained, the visual prognosis of perforating eye injuries in children is still poor. Special care should be taken to prevent such a severe eye trauma in children by every means.

A Case of Chronic Progressive External Ophthalmoplegia

Chronic progressive external ophthalmoplegia(CPEO) is rare syndrome, which is characterized by slowly progressive blepharoptosis, paralysis of extraocular muscle and has involvement of other organs, particularly the retina, heart, endocrine gland, and bony skeleton. Histological examination of muscle showes characteristic ragged red fibers. Electron microscopy reveals a number of abnormal mitochondria which contain paracrystalline inclusion bodies. We experienced a 50-year-old female with CPEO, that was pathologically proven by electron microscopy and bilateral levator levator advancements were given for ptosis.