Abstract
Parents of 42 patients with Lesch-Nyhan disease completed a questionnaire systematizing caregiver observations of the subject's behavior during a wide variety of daily events. Responses were grouped in nine categories reflecting different aspects of cognitive skills. Only 1 boy appears to have any significant generalized cognitive impairment. The patients' memory for both recent and past events is excellent, their emotional life has a normal range of reactions and is appropriate; they have good concentration, are capable of abstract reasoning, have good self-awareness, and are highly social. However, they are behind in academic ability, with only 15% at grade level for math and reading. Implications for designing educational activities, parenting or caregiver strategies, and research methodology are discussed.
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This research was supported in part by National Institute of Mental Health Training Grant
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Anderson, L.T., Ernst, M. & Davis, S.V. Cognitive abilities of patients with Lesch-Nyhan disease. J Autism Dev Disord 22, 189–203 (1992). https://doi.org/10.1007/BF01058150
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DOI: https://doi.org/10.1007/BF01058150