The frequency of scans within the recommended range of every 1-3 years should be clinically determined, with scans performed more Stem Cell Compound Library concentration frequently in those asymptomatic SEGA patients who

are younger, whose SEGA are larger or growing, or who are developmentally or cognitively disabled such that they cannot reliably report subtle symptoms. (Category 2A) Individuals without SEGA by the age of 25 years do not need continued surveillance imaging, but those with asymptomatic SEGA present in childhood should continue to be monitored by MRI for life because of the possibility of growth. There is insufficient evidence to determine the recommended frequency of MRI surveillance in this latter group, but important clinical factors that would favor shorter intervals include SEGA with proximity to foramen of Monro, large size, or recently discovered. However, once stability is clearly established, it may be possible to increase the interval of surveillance monitoring over time. (Category 3) Strong evidence demonstrates superior efficacy for the treatment Selleck ATM/ATR inhibitor of infantile spasms with vigabatrin in patients with TSC34, 35, 36 and 37; therefore, vigabatrin should be first-line treatment. However, the prescribing clinician should be aware of possible side effects, particularly possible retinal toxicity, and how to monitor for these. Adrenocorticotropin hormone

(ACTH) can be used as second-line therapy if treatment with vigabatrin fails. (Category 1) Routine EEG is recommended in individuals with known or suspected seizure activity, but frequency should be determined by clinical need rather than a specific defined interval. If changes in sleep, behavior, or cognitive or neurological function are not explained by routine EEG, 24-hour video EEG should be considered to assess for unrecognized or subclinical seizure activity. (Category 2A) Early epilepsy treatment may be of benefit in infants and children during the first 24 months of life if ictal discharges occur, with

or without clinical manifestations.38 Other than for infantile spasms in TSC, there is little evidence to guide specific anticonvulsant treatment. In many general, this should follow that of other epilepsies, but it should be noted that the prevalence of medically refractory epilepsy is high in TSC even with adequate trials of currently available anticonvulsant medications.30 and 39 Epilepsy surgery and vagus nerve stimulation may be considered for medically refractory TSC patients, but evaluation should take place at epilepsy centers with experience and expertise in TSC, and special consideration should be given to children at younger ages experiencing neurological regression. (Category 2A) Given that the physical features of TSC such as SEGA, epilepsy, or renal failure may present with TAND-like behaviors, sudden and rapid changes in TAND should prompt an urgent overall physical workup in such individuals.