Lavy, T and Harris, CM and Shawkat, F and Thompson, D and Taylor, D and Kriss, A (1995) Electrophysiological and eye-movement abnormalities in children with the Bardet-Biedl syndrome. J Pediatr Ophthalmol Strabismus , 32 (6) 364 - 367.
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We investigated 17 patients with clinical features of Bardet-Biedl syndrome by electroretinogram (ERG), visual-evoked potentials (VEP), and electro-oculographic (EOG) eye movement assessment. The ERGs were grossly abnormal in 16 cases. Pattern VEPs were generally well preserved, but showed a tendency to increase in latency and decrease in amplitude with age. These results confirm other reports that the retinopathy appears to be a progressive rod-cone dystrophy initially affecting mainly extramacular areas but involving the macula at later stages. Seven of 11 patients showed abnormalities of either optokinetic nystagmus, vestibulo-ocular reflex, or both. These eye-movement abnormalities have not been reported previously, and are further evidence of central nervous system involvement in this syndrome.
|Title:||Electrophysiological and eye-movement abnormalities in children with the Bardet-Biedl syndrome.|
|Keywords:||Adolescent, Aging, Child, Child, Preschool, Electrophysiology, Eye Movements, Female, Humans, Hypogonadism, Infant, Intellectual Disability, Male, Nystagmus, Optokinetic, Obesity, Ocular Motility Disorders, Photoreceptor Cells, Reflex, Vestibulo-Ocular, Retinitis Pigmentosa, Syndrome|
|UCL classification:||UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Child Health|
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