A new neurological syndrome with mental retardation, choreoathetosis, and abnormal behavior maps to chromosome Xp11

Choreoathetosis is a major clinical feature in only a small number of hered itary neurological disorders. We define a new X-linked syndrome with a uniq ue clinical picture characterized by mild mental retardation, choreoathetos is, and abnormal behavior. We mapped the disease in a four-generation pedig ree to chromosome Xp11 by linkage analysis and defined a candidate region c ontaining a number of genes possibly involved in neuronal signaling, includ ing a potassium channel gene and a neuronal G protein-coupled receptor.