Cognitive Abilities and Behavioural Characteristics of Children With Idiopathic Infantile Hypercalcaemia

Abstract

An investigation of the psychological characteristics of 44 children aged 6-16 years with documented evidence of idiopathic infantile hypercalcaemia revealed cognitive abilities and behavioural patterns which distinguish these children from other mentally handicapped groups. The children were all mildly to severely mentally handicapped, but exhibited superior verbal skills compared to their visuo-spatial and motor abilities. They showed high rates of behavioural disturbance, particularly in terms of hyperactivity, social isolation, anxiety, and eating and sleeping difficulties. Other aspects of behaviour, spoken language and personality that were commonly encountered are also described and quantified. From these observations approaches to remedial teaching are suggested.

Using developmental trajectories to examine verbal and visuospatial short-term memory development in children and adolescents with Williams and Down syndromes

“…Jones & Smith, 1975;Lenhoff, Wang, Greenberg, & Bellugi, 1997;Wang, Doherty, Rourke, & Bellugi, 1995). Psychological and behavioural markers include mild-moderate intellectual disability (ID; Udwin, Yule, & Martin, 1987), a sociable disposition (e.g. Jones et al, 2000), heightened anxiety (Dykens, 2003) and hyper-sensitivity to sound (e.g.…”

Social-perceptual abilities in adolescents and adults with Williams syndrome

“…Given the rarity of this genetic syndrome, many studies have reported results based on small samples, with participants spanning a wide age range, and variability in performance has been often overlooked in interpreting empirical results. At the outset, interest in the syndrome was sparked by case studies of unusual social and linguistic behaviour (e.g., Bellugi, Bihrle, Neville, Jernigan, & Doherty, 1992;Bellugi, Marks, Bihrle, & Sabo, 1988;Udwin, Yule, & Martin, 1987), which led researchers to search for spared abilities in the context of general mental retardation and of severe specific cognitive deficits in visual-spatial construction skills. This paper focuses on the social component of the WMS profile, addressing the question of whether the unusual sociability and friendly personality characteristic of people with this neurodevelopmental disorder (Gosch & Pankau, 1994;Jones et al, 2000;Mervis & KleinTasman, 2000) is associated with relatively spared abilities in decoding mental state information from nonverbal facial cues.…”

Anxiety in children with Williams syndrome : association with negative reactivity, self-regulation, and sensory modulation.

“…Some of the earliest reports surfaced 20 years ago, with descriptions of exaggerated startle response to sound and anticipatory anxiety related to loud noise (Dilts et al, 1990). Although sensory modulation difficulties have been frequently noted in this population (e.g., Gosch & Pankau, 1994, 1997Leyfer et al, 2006;Udwin, 1990;Udwin et al, 1987), there have been few attempts to understand the nature of sensory-related problems. John and Mervis (2010) explored sensory problems in children with WS using the Short Sensory Profile (McIntosh, Miller, Shyu, & Dunn, 1999).…”

“…Specifically, studies have shown that individuals with WS have difficulty with inhibitory control as indexed by measures of hyperactivity, approach, and attention seeking behaviors (Arnold et al, 1985;Dilts et al, 1990;Dykens & Rosner, 1999;Klein-Tasman & Mervis, 2003;Tomc et al, 1990;Udwin, 1990;Udwin et al, 1998;Udwin & Yule, 1991). Individuals with WS also have been shown to demonstrate difficulties with set-shifting and emotion regulation, as indexed by measures of low adaptability, high preoccupations, low persistence, high levels of stress reaction, and increased temper tantrums Klein-Tasman & Mervis, 2003;Tomc et al, 1990;Udwin, 1990;Udwin et al, 1987).…”

“…Self-regulation has been associated with the neurobiological substrates of the OFC, the dorsolateral prefrontal cortex, and the anterior cingulate cortex (Whittle et al, 2006). Difficulties with self-regulation, such as poor inhibition, poor adaptability, perseveration, and hyperactivity, have been documented as weaknesses for individuals with WS (Arnold et al, 1985;Davies et al, 1998;Dilts et al, 1990;Dykens & Rosner, 1999;Gosch & Pankau, 1994;Klein-Tasman & Mervis, 2003;Tomc et al, 1990;Udwin, 1990;Udwin et al, 1998;Udwin & Yule, 1991;Udwin, Yule, & Martin, 1987). Further, executive functioning abilities have been associated with anxiety, problem behaviors, and sensory modulation difficulties in children with WS, underscoring the significance of these regulatory skills (John & Mervis, 2010 Gioia et al, 2000) to investigate the association between aspects of executive functioning and the probability of having an anxiety disorder in children with WS.…”