Approximately 45% to 50% of people with tuberous sclerosis complex (TSC) develop intellectual disabilities. However, profiles of strengths and weaknesses are highly variable person-to-person and are often very uneven within an individual regardless of their overall intellectual ability.
Even in people with above average and high intellectual abilities, the rates of specific neuropsychological deficits (e.g., in attentional, memory, or executive skills) are very high and can be associated with significant challenges in daily life (e.g., in school, relationships, or the workplace). This is even more likely to be the case for those with TSC known to have neurodevelopmental disorders such as autism, attention deficit hyperactivity disorder (ADHD), or learning disorders.
Understanding your/your loved one’s profile can help to understand current difficulties and predict future ones. Performing evaluations in preparation for transitions in school, in preparation for post-secondary training or for the workplace and implementing neuropsychological intervention plans is valuable.
The consensus panel recommends the following:
Neuropsychological cluster recommendations
- Everyone with TSC should receive a comprehensive assessment of their intellectual ability at the time of diagnosis to identify their profile of strengths and weaknesses.
- Children and adults with TSC should have ongoing (at least annual) screening and monitoring for the presence or emergence of neuropsychological difficulties.
- Neuropsychological deficits can have a major impact on functional ability and may present in various ways, such as anxiety and feeling overwhelmed, dysregulated behaviours, difficulties in education, or struggles in work.
- Individuals with known neurodevelopmental disorders (e.g., ADHD, autism, learning disorders) should receive a formal neuropsychological evaluation to understand their profile of strengths and weaknesses, not just be screened for such deficits.
- Non-pharmacological coaching and training strategies should be used to strengthen areas of neuropsychological deficits, for example, executive coaching for working memory, cognitive flexibility or planning deficits.
- Neuropsychological deficits may require specific accommodations or supports in education and/or the workplace.
Reviewed by Ashley Pounders, MSN, FNP-C, Director of Medical Affairs, TSC Alliance, November 2023.
