The Frontotemporal Dementias (FTD) are a group of degenerative brain disorders that result from progressive damage to neurons in the anterior temporal and/or frontal lobes of the brain. The hallmark of FTD is a gradual, progressive decline in behavior and/or language which often begins between 50 to 60 years of age. Progression of the disease ranges between 2-10 years. Several subtypes of the disorder have been characterized and include: 1) behavioral variant FTD (bvFTD) characterized by loss of empathy and increasingly inappropriate social behavior; 2) FTD associated with language disabilities including semantic dementia and progressive nonfluent aphasia; and 3) FTD associated with motor neuron disease. Currently there are no therapies to cure or slow progression of FTD.
To enable the development of a clinical data set specific for FTD, a workshop was organized by NINDS in collaboration with the Association for Frontotemporal Dementias (AFTD) and NIA to facilitate the development of common data elements that address the unique clinical, pathological, imaging and genetic aspects of FTD. The FTD common data elements (CDEs) and pathology data sets identified at the workshop will contribute to the development of a FTD module for the NIA-funded National Alzheimer’s Coordinating Center (NACC). The purpose of the NACC is to facilitate collaborative research among the NIA-funded Alzheimer's Disease Centers (ADCs) nationwide. Development and incorporation of the FTD module into the NACC will enable the utilization of an existing set of clinical sites for collection and data management of clinical information from FTD patients. This infrastructure will provide the basis for future research initiatives for FTD including biomarker development, therapeutic discovery and early phase clinical trials.
Margaret Sutherland, Ph.D.
National Institute of Neurological Disorders and Stroke
Phone: (301) 496-5680
Last updated March 20, 2013