Visual dysfunction of type I and VI mucopolysaccharidosis patients evaluated with visual evoked cortical potential

Purpose: 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 gr...

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Detalles Bibliográficos
Autores: Gomes, Bruno Duarte, Souza, Givago, Viana, Gustavo Monteiro, Souza, Isabel Cristina Neves de, Feio, Patrícia do Socorro Queiroz, Schwartz, Ida Vanessa Doederlein, Marinho, Diane Ruschel, Silva Filho, Manoel da, Giugliani, Roberto, Silveira, Luiz Carlos de Lima, Silva, Luiz Carlos Santana da
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2012
País:Brasil
Institución:Universidade Federal do Rio Grande do Sul (UFRGS)
Repositorio:Repositório Institucional da UFRGS
Idioma:inglés
OAI Identifier:oai:www.lume.ufrgs.br:10183/180808
Acceso en línea:http://hdl.handle.net/10183/180808
Access Level:acceso abierto
Palabra clave:Mucopolissacaridoses
Terapia de reposição de enzimas
Glicosaminoglicanos
Mucopolysaccharidosis
Enzyme replacement therapy
Visual evoked cortical potential
Glycosaminoglycans
Descripción
Sumario:Purpose: 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.

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