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Sjogren–Larsson syndrome (SLS) is a triad of generalized ichthyosis, mental retardation, and spastic paralysis caused by accumulation of fatty aldehydes and fatty alcohols in body tissues.[1] The pathognomonic ocular manifestations… Click to show full abstract
Sjogren–Larsson syndrome (SLS) is a triad of generalized ichthyosis, mental retardation, and spastic paralysis caused by accumulation of fatty aldehydes and fatty alcohols in body tissues.[1] The pathognomonic ocular manifestations include crystalline juvenile macular dystrophy, cystoid foveal atrophy, and lack of macular pigment due the degeneration of Muller cells in the inner retina, retinal pigment epithelium (RPE) compromise from lipofuscin accumulation, leading to progressive RPE atrophy.[2‐4] These changes generally start from the age of 2 and progress with the age.
Journal Title: Indian Journal of Ophthalmology
Year Published: 2022
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