Cognitive abilities of patients with Lesch-Nyhan disease

Title

Cognitive abilities of patients with Lesch-Nyhan disease

Creator

Anderson L T; Ernst M; Davis S V

Identifier

Publisher

Journal of Autism and Developmental Disorders

Date

1992

Subject

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

Description

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.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Anderson L T; Ernst M; Davis S V, “Cognitive abilities of patients with Lesch-Nyhan disease,” Pediatric Palliative Care Library, accessed September 22, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16572.

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