Determining the neurocognitive profile of children with tuberous sclerosis complex within the Western Cape region of South Africa.

BACKGROUND: Tuberous sclerosis complex (TSC) is a multisystem genetic disorder associated with a wide spectrum of cognitive impairments that can often result in impaired academic, social and adaptive functioning. However, studies investigating TSC have found it difficult to determine whether TSC is associated with a distinct cognitive phenotype and more specifically which aspects of functioning are impaired. Furthermore, children with TSC living in low-income and middle-income countries, like South Africa, experience additional burdens due to low socio-economic status, high mortality rates and poor access to health care and education. Hence, the clinical population of South Africa may vary considerably from those populations from high-income countries discussed in the literature. METHODS: A comprehensive neuropsychological battery composed of internationally recognised measures examining attention, working memory, language comprehension, learning and memory, areas of executive function and general intellectual functioning was administered to 17 children clinically diagnosed with TSC. RESULTS: The exploration of descriptive data indicated generalised cognitive difficulties in most cognitive domains, aside from memory. With only two participants performing in the average to above-average ranges, the rest of the sample showed poor verbal comprehension, perceptual reasoning, working memory, processing speed, disinhibition, and problems with spatial planning, problem solving, frustration tolerance, set shifting and maintaining a set of rules. Furthermore, correlational findings indicated several associations between socio-demographic and cognitive variables. CONCLUSIONS: Importantly, this is the first study to comprehensively examine multiple domains of neurocognitive functioning in a low-resource setting sample of children with TSC. Current study findings showed that children with TSC have generalised impairments across several cognitive domains, rather than domain-specific impairments. Therefore, although examining individual aspects of cognition, such as those found in previous literature, is important, this approach is limiting. With a comprehensive assessment, including understanding the associations between domains, appropriate and directed support can be provided to ensure all aspects of development are addressed and considered.

Has maternal sensitivity been comprehensively investigated in sub-Saharan Africa? A narrative scoping review.

Child development is strongly influenced by maternal characteristics. Maternal sensitivity, as well as risks to and outcomes of sensitive maternal style, are well studied in industrialised western contexts, but it is unclear if this is the case for other contexts. Sub-Saharan Africa has been subjected to and continues to negotiate socio-economic and psychological sequelae of colonial and race-based politics: exploring the nature and outcomes of early caregiver input in such challenging conditions is imperative. This scoping review thus aims to 1) evaluate the nature and extent of quantified observational assessments of dyadic interactions, with a focus on maternal sensitivity, in Sub-Saharan Africa and 2) ascertain which risk and outcome factors have been examined in relation to maternal sensitivity. Study quality and cross-cultural appropriateness will also be considered. The search using expanded search terms yielded 20 papers -four characterizing maternal sensitivity or style, eight examining maternal sensitivity in relation to risks and outcomes, and eight intervention studies examining efforts to improve maternal sensitivity. Most research was conducted in South Africa - only seven studies were conducted in four other countries. Researchers used a wide array of coding schemes, mostly developed in the west. Ten studies made some adaptations to measures. Language issues and cultural considerations were often not explicitly addressed. Taken together, very limited research on this important topic exists. For the work that does exist, questions around westernized assumptions, language, and appropriateness of measures remain. Substantially more research, informed by both culturally flexible conceptualizations and methodological rigour, is required.