Tapetoretinal degeneration in childhood presenting as a disturbance of behaviour

Four children presented at the age of 6 years with progressive disturbances of behaviour due to increasing visual handicap. In three of them routine ophthalmological examination was carried out in the early stages, but no objective evidence of eye disease was found. The severity and nature of the visual defect and its causal relationship to the behaviour disorder went unrecognized for periods ranging from 18 to 30 months. Eventually the development of retinal changes enabled a diagnosis of tapetoretinal degeneration to be made which was confirmed by the finding of extinction of the electroretinographic response. Transfer of the children to schools for the visually handicapped resulted in improvement in their behaviour disorder.Electroretinography is of great importance in the early diagnosis of juvenile tapetoretinal degeneration.