Identifying Determinants for Neurobehavioral Morbidity in Tuberous Sclerosis Complex

Tuberous Sclerosis Complex (TSC) is a multisystem disorder characterized by the growth of hamartomas in multiple organ systems. The syndrome was described as early as 1835 by Rayer, and later by von Recklinghousen. In 1880, Bourneville described the syndrome in three patients and was the first to use the term ‘tuberous sclerosis’ to describe the potatolike consistency and hypertrophic sclerosis of the brain gyri at autopsy. The classical triad of seizures, intellectual disability, and adenoma sebaceum (angiofibromas) was first noted by Vogt in 1908. Since these early descriptions, many other presentations of TSC have been recognized and the diagnostic criteria have been refined to include clinical, radiological and pathological characteristics. The diagnostic criteria for TSC were most recently revised in 19983, (Table 1, diagnostic criteria). TSC has a birth incidence of 1:6000.