2019-06-052019-06-052009ISAAC, David Leonardo Cruvinel et al. Distrofia macular cristalina em paciente com síndrome de Sjögren-Larsson: relato de caso. Arquivos Brasileiros de Oftalmologia, São Paulo, v. 72, n. 2, p. 239-242, 2009.0004-2749http://repositorio.bc.ufg.br/handle/ri/17611Presentation of a case of crystalline macular distrophy diag- nosed in a female patient with Sjögren-Larsson syndrome. The disease consists of clinical findings of spastic diplegia or tetraplegia, mental retardation, and congential ichthyosis. The eyes are affected in up to 100% of cases, and crystalline ma- culopathy is the main finding as described in this case report. On fundus examination multiple white dots were observed at ophthalmoscopy. The optical coherence tomography has shown not only the hipereflexive intraretinal spots but also macular atrophy with macular thickness reduction. The tomographic findings were first described in our country.porAcesso AbertoMacula lútea/patologiaSíndrome de Sjögren-Larsson/complicaçõesTomografia de coerência óptica/métodosRelatos de casos [tipo de publicação]Macula lutea/pathologyCase reports [publication type]Sjögren-Larsson syndrome/complicationsTomography, optical coherence/methodsArtigo10.1590/S0004-27492009000200021