Table 7 Neuropsychological cluster recommendations

NP1. All individuals with TSC should receive an assessment of their intellectual ability to identify their profile of strengths and weaknesses. The majority of individuals with TSC have normal-range intellectual ability (IQ > 80), and 40–50% have intellectual disability (IQ < 70 and functional impairment in adaptive behavioral skills). Regardless of their level of intellectual ability, the majority of people with TSC have a very uneven profile of intellectual strengths and weaknesses. Intellectual ability is a very strong predictor of many TAND manifestations, and uneven intellectual profiles can be associated with many difficulties in daily life functioning, such as in school or work. For this reason, all individuals with TSC should receive a comprehensive assessment of their intellectual ability at the time of diagnosis using age- and developmentally appropriate measures. Evaluations should be repeated as clinically indicated, and to inform neuropsychological and scholastic assessments

NP2. Neuropsychological deficits are common in TSC, even in those with normal intellectual ability, and should be screened for. Specific neuropsychological deficits (in brain-referenced systems such as memory, attentional and executive skills, language, and visuospatial skills) are very common in TSC and are seen even in those with high to very high intellectual ability. For individuals with normal intellectual ability, neuropsychological deficits are seen across age, sex, and genotype (TSC1 or TSC2), and in those with and without epilepsy. Neuropsychological skills (and deficits) emerge during neurodevelopment and may not always be present at the time of a TSC diagnosis. For this reason, children and adults with TSC should have ongoing screening and monitoring for the presence or emergence of neuropsychological deficits

NP3. Neuropsychological deficits can have a major impact on the functional ability of an individual and may present in various ways. The presence of specific neuropsychological deficits may manifest in many different ways (e.g. anxiety and feeling overwhelmed, dysregulated behaviors, difficulties in education, or struggles in work). The manifestations may present differently based on the age and developmental level of the individual and on the specific neuropsychological deficit. Caregivers and healthcare providers should therefore establish how these deficits manifest in daily life. This may help understand current challenges, anticipate future difficulties, and inform interventions or support

NP4. Individuals with TSC who are known to have a diagnosed neurodevelopmental disorder (such as ADHD, autism, and known learning disorders, e.g., reading/writing/maths) should receive a formal neuropsychological evaluation for the presence of neuropsychological deficits. Even though all people with TSC are at risk of neuropsychological deficits, those with neurodevelopmental disorders such as ADHD, autism, and learning disorders are particularly likely to have specific neuropsychological deficits. For this reason, these individuals should not only receive screening for neuropsychological deficits but should be referred for formal evaluation. Neuropsychological evaluation may identify a particular profile of strengths and weaknesses to guide intervention, support, and accommodations

NP5. There are non-pharmacological coaching and training strategies that can be used to strengthen areas of neuropsychological deficits. There is an increasing body of evidence supporting a range of coaching and training strategies to strengthen or enhance specific neuropsychological skills (e.g., executive coaching for working memory, cognitive flexibility, or planning deficits). Caregivers and professionals are encouraged to explore such options when neuropsychological deficits are identified

NP6. Neuropsychological deficits may require specific accommodations or supports in education and/or the workplace. Accommodations or supports that are specifically designed for deficits in memory, executive skills, visuospatial, and language skills should be incorporated into individual educational plans (IEPs) or equivalent special educational frameworks and should also be considered in the workplace for adults