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dc.contributor.authorGomes, Bruno Duartept_BR
dc.contributor.authorSouza, Givagopt_BR
dc.contributor.authorViana, Gustavo Monteiropt_BR
dc.contributor.authorSouza, Isabel Cristina Neves dept_BR
dc.contributor.authorFeio, Patrícia do Socorro Queirozpt_BR
dc.contributor.authorSchwartz, Ida Vanessa Doederleinpt_BR
dc.contributor.authorMarinho, Diane Ruschelpt_BR
dc.contributor.authorSilva Filho, Manoel dapt_BR
dc.contributor.authorGiugliani, Robertopt_BR
dc.contributor.authorSilveira, Luiz Carlos de Limapt_BR
dc.contributor.authorSilva, Luiz Carlos Santana dapt_BR
dc.date.accessioned2018-07-31T02:33:30Zpt_BR
dc.date.issued2012pt_BR
dc.identifier.issn1663-2699pt_BR
dc.identifier.urihttp://hdl.handle.net/10183/180808pt_BR
dc.description.abstractPurpose: To evaluate the visual system of patients suffering from type I or VI mucopolysaccharidosis (MPS) by recording the visual evoked cortical potential (VECP). Methods: Two patients with MPS VI and 2 patients with MPS I were tested before and after enzyme replacement therapy (ERT). A control group of 20 subjects was tested for statistical comparison. VECP was elicited by monocular stimulation with 1-Hz phase-reversal checkerboard patterns at 0.5 and 2 cycles per degree and with 16° of visual field. In all patients, both eyes were tested. VECP amplitude and latency were measured and compared with tolerance limits obtained from controls. Results: MPS I and VI patients have a severe visual impairment that can be quantified by measuring VECPs. Even after several weeks of ERT, the visual impairment remained unaltered, indicating that the treatment had no significant influence on the visual conditions of MPS patients. Visual responses to high spatial frequencies were more deeply impaired than responses to low spatial frequencies. This can be explained by the kind of damage in the visual system that preferentially targets the eye optics. Conclusion: VECPs can be used to monitor the degree of visual impairment of MPS patients and to check ERT efficacy.en
dc.format.mimetypeapplication/pdfpt_BR
dc.language.isoengpt_BR
dc.relation.ispartofCase reports in ophthalmology. Basel. Vol. 3, no. 1 (Jan./Apr. 2012), p. 104-112pt_BR
dc.rightsOpen Accessen
dc.subjectMucopolissacaridosept_BR
dc.subjectMucopolysaccharidosisen
dc.subjectEnzyme replacement therapyen
dc.subjectTerapia de reposição enzimáticapt_BR
dc.subjectVisual evoked cortical potentialen
dc.subjectGlicosaminoglicanaspt_BR
dc.subjectGlycosaminoglycansen
dc.titleVisual dysfunction of type I and VI mucopolysaccharidosis patients evaluated with visual evoked cortical potentialpt_BR
dc.typeArtigo de periódicopt_BR
dc.identifier.nrb000835674pt_BR
dc.type.originEstrangeiropt_BR


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