Cognitive abilities of patients with Lesch-Nyhan disease.
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.
Anderson
L T
LT
New York University School of Medicine.
Ernst
M
M
Davis
S V
SV
D000124:Achievement; D000293:Adolescent; D000328:Adult; D001364:Awareness; D001521:Behavior Therapy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000523:psychology / Q000628:therapy; D007360:Intelligence; D007805:Language Development Disorders / Q000175:diagnosis / Q000523:psychology / Q000628:therapy; D007926:Lesch-Nyhan Syndrome / Q000175:diagnosis* / Q000523:psychology / Q000628:therapy; D008297:Male; D009483:Neuropsychological Tests; D016728:Self-Injurious Behavior / Q000175:diagnosis / Q000523:psychology / Q000628:therapy; D012917:Social Adjustment; D012919:Social Behavior; D012931:Social Environment; D013850:Thinking