The pathophysiology, medical management and dental implications of fragile X, Rett, and Prader-Willi syndromes.

Fragile X, Rett, and Prader-Willi syndromes are a group of inherited disorders that often present with varying degrees of mental retardation and challenging behaviors. Dentists caring for individuals with these disorders must be familiar with the manifestations of these diseases and their associated features so they can garner the maximum level of cooperation from the patient. They must also be familiar with the medications (anticonvulsants, antihypertensives, antidepressants, antipsychotics, and central nervous system stimulants) used to treat the associated behaviors, because many of these pharmaceuticals cause clinically evident orofacial and systemic reactions, and may precipitate adverse interactions with dental therapeutic agents.