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- Title
Beyond retina in Sjogren-Larsson syndrome.
- Authors
Pawar, Neelam; Meenakshi, R; Maheshwari, Devendra; Fathima, A; Chakrabarty, Sabyasachi; Paranjape, Mihir
- Abstract
SLS patients should have routine ophthalmologic examination as cataract may be underreported in this syndrome. Ganemo A et al. found glistening dots around the foveola in 35 Swedish cases with SLS.[[2]] Van der Veen et al.,[[5]] Nanda et al.,[[3]] and Willemsen described FAF changes in the SLS patient with lack of normal FAF attenuation at the fovea.[[4]] Cataract screening should be done in all patients with SLS. Keywords: Cataract; crystalline retinopathy; Sjogrens-Larsson EN Cataract crystalline retinopathy Sjogrens-Larsson 2727 2728 2 07/15/22 20220701 NES 220701 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]],[[3]],[[4]] These changes generally start from the age of 2 and progress with the age.
- Subjects
RETINA; ICHTHYOSIS
- Publication
Indian Journal of Ophthalmology, 2022, Vol 70, Issue 7, p2727
- ISSN
0301-4738
- Publication type
journal article
- DOI
10.4103/ijo.IJO_2994_21