Finally, with respect to the nervous system, cerebellar dysplastic gangliocytoma is the most frequently associated entity, with a prevalence of 15-32% associated with CS; retardation in psychomotor development can be present in 12 to 20% of cases; in patients with PTEN alteration, approximately 50% of cases have been associated with motor or language delay but with normal intelligence in adulthood [10,25]. The gene discussed is PTEN; the disease is Cowden syndrome 1.