The severity of self-injury did not change over years. Age of onset was a predictor of outcome. The earlier the age of onset the worse the self-injury eventually became. The discussion describes research strategies, suggests dimensions along which self-injury can be classified, and highlights behavior not commonly described in patients with Lesch-Nyhan disease.

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This double-blind and placebo-controlled clinical trial in autistic children had three objectives: (a) to replicate earlier findings that haloperidol administration is associated with a significant reduction of behavioral symptoms; (b) to further assess its safety when given on a short-term basis; and (c) to assess whether it has an effect on discrimination learning. Forty-five children, 2.02 to 7.58 years old (M = 4.49), completed this crossover design, with random assignment to treatment sequences. Haloperidol was shown to be a powerful therapeutic agent when administered for 4 weeks and free of side effects; at doses ranging from 0.25 to 4.0 mg/day (M = 0.844), there was a clinically and statistically significant reduction of a variety of symptoms. Under the given conditions, the children failed to learn on either haloperidol or placebo.

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demonstrating the specific enzyme defect in red or white blood cells or skin fibroblasts. Family patterns of inheritance and mosaicism in tissues of the obligatory heterozygote (Migeon, Der Kaloustian, & Nyhan, 1968;Silver, Cox, Balis, & Dancis, 1972) indicate an X-linked recessive disease. Management strategies are supportive of kidney function by preventing excessive production of uric acid with allopurinol, thereby greatly increasing life expectancy. Questions concerning procedures for managing the behavioral consequences of the illness and for optimizing the life adjustment of these children thus become relevant.One of the most striking and disabling characteristics of the disease is a pattern of self-injurious behavior (SIB) that affects virtually all Lesch-Nyhan children (Lesch & Nyhan, 1964). Near the end of the second year of life, they begin to bite their fingers, lips, or arms with sufficient force to cause lacerations, expose tendons, and even amputate fingertips. If not prevented, SIB occurs at a very high rate, accompanied by general agitation and crying. Reliance on tubular arm splint restraints to prevent finger biting and extraction of teeth to prevent lip biting have constituted the only therapeutic approach to SIB in Lesch-Nyhan children.Although no direct studies have been reported that demonstrate normal pain sensi-

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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Five autistic and five normal children were allowed to register a sensory modality preference by bar pressing to select either a visual (slides) or an auditory (music) stimulus. The children were then taught a discrimination between the presence or absence of a compound auditory-visual stimulus (white noise and light). Testing for stimulus overselectivity revealed that the autistic children attended to only one aspect of the compound stimulus. In all cases this was the sensory modality that was selected during the preference test. Significant correlations were obtained between Gesell developmental scores and degree of overselectivity. Normal children registered an equal preference for music and slides and displayed no overselectivity.