Further electroretinographic studies of patients with mucopolysaccharidoses.
Electroretinographic studies were performed on many of the patients studied by Gills et al in 1965. An additional 13 patients with mucopolysaccharidoses were also studied. The ERG is normal in all patients with the Morquio syndrome (MPS IV), the Maroteaux-Lamy syndrome (MPS VI) and pseudo-Hurler polydystrophy. The ERG is abnormal in patients with the Hurler syndrome (MPS I), the Sanfilippo syndrome (MPS III) and the Scheie syndrome (MPS V). Some patients with the Hunter syndrome had normal ERGs while others had abnormal ERGs.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003318:Corneal Opacity / Q000209:etiology* / Q000473:pathology; D003394:Craniofacial Dysostosis / Q000503:physiopathology; D004392:Dwarfism / Q000503:physiopathology; D004596:Electroretinography; D005132:Eye Manifestations; D005260:Female; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D008607:Intellectual Disability / Q000150:complications; D008297:Male; D008875:Middle Aged; D009083:Mucopolysaccharidoses / Q000145:classification / Q000150:complications / Q000503:physiopathology*; D008059:Mucopolysaccharidosis I / Q000503:physiopathology; D016532:Mucopolysaccharidosis II / Q000503:physiopathology; D009085:Mucopolysaccharidosis IV / Q000503:physiopathology; D012160:Retina / Q000503:physiopathology*; D012174:Retinitis Pigmentosa / Q000503:physiopathology; D013577:Syndrome