Neurobehavioral outcomes following epilepsy surgery in tuberous sclerosis complex: A retrospective study

Tuberous sclerosis complex (TSC) is a rare genetic disorder with a high prevalence of drug-resistant epilepsy. In such cases, epilepsy surgery may render 60% of suitable candidates seizure-free. Although seizure reduction may contribute to improvements in cognitive or language skills, little is known about neurobehavioral changes overall.

This retrospective single-center study included children with TSC who underwent epilepsy surgery and had ≥ 12 months of follow-up data. We collected pre- and postoperative neurobehavioral data, structured around the TSC-Associated Neuropsychiatric Disorders (TAND) questionnaire, from medical and neuropsychological records. Surgical outcomes were dichotomized as seizure-free or not seizure-free.

Of 21 surgeries, 13 (62%) achieved complete seizure freedom, and 15 (71%) achieved seizure freedom from the targeted seizure type. The most prevalent TAND problems at baseline were in language (n = 19), motor skills (n = 17), and attention (n = 13). After surgery, 18 (86%) showed improvement in at least one cluster, most often language (n = 12) or attention (n = 7). Postoperative behavioral worsening in at least one cluster was observed in 13 (62%), most often in repetitive behaviors (n = 5) and tantrums (n = 4). Surgical outcomes were unrelated to the improvement or worsening of symptoms.

Neurobehavioral improvement was often observed following epilepsy surgery in TSC, with seizure outcomes unrelated to neurobehavioral outcomes. However, some worsening of behavior was observed. Many TAND items were not routinely documented in the clinical records, underscoring the need for a more structured, prospective approach to neuropsychiatric comorbidity assessment in TSC.