Cognitive abilities of patients with Lesch-Nyhan disease

Cognitive abilities of patients with Lesch-Nyhan disease

Anderson L T; Ernst M; Davis S V

Journal of Autism and Developmental Disorders

1992

Male; Child; Humans; Adult; Adolescent; Female; Child Preschool; Social Environment; Combined Modality Therapy; Social Adjustment; Social Behavior; Behavior Therapy; Awareness; Intelligence; Neuropsychological Tests; Achievement; Lesch-Nyhan Syndrome/di [Diagnosis]; Intellectual Disability/di [Diagnosis]; Intellectual Disability/px [Psychology]; Intellectual Disability/th [Therapy]; Language Development Disorders/di [Diagnosis]; Language Development Disorders/px [Psychology]; Language Development Disorders/th [Therapy]; Lesch-Nyhan Syndrome/px [Psychology]; Lesch-Nyhan Syndrome/th [Therapy]; Self-Injurious Behavior/di [Diagnosis]; Self-Injurious Behavior/px [Psychology]; Self-Injurious Behavior/th [Therapy]; Thinking; alertness; behavioral; Lesch-Nyhan syndrome; trajectory; characteristics; attempted suicide; suicide

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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