FRONTOTEMPORAL DEMENTIA AND PARKINSONISM LINKED TO CHROMOSOME-17 - A CONSENSUS CONFERENCE

We held an international consensus conference on frontotemporal dement ia, behavioral disturbances, and parkinsonism linked to chromosome 17 to determine whether these are homogeneous or heterogeneous disorders, to agree on terminology, and to develop strategies for further resear ch. The group identified 13 kindreds with sufficient evidence for link age, finding in common to all a critical 2 cM between markers D17S791 and D17S800. There was agreement that (1) despite previous description s that have emphasized one or another clinical or neuropathological fe ature, the kindreds share clinical and neuropathological features; (2) until more specific information about the genetic defects becomes ava ilable, this disorder is best termed frontotemporal dementia and parki nsonism linked to chromosome 17; and (3) further research will be enha nced by identifying the gene or genes responsible for this disorder, d etecting additional cases within known families and, in new families, correlating mutations with phenotypes and more fully delineating the c linical, neuropsychological, and neuropathological characteristics of this disorder.