Frontotemporal Lobar Degeneration (FTLD)
FTLD is a complex group of disorders characterized by shrinkage of the frontal and anterior temporal lobes of the brain. It is the most second common cause of dementia in people under the age of 65, after AD. The most prevalent of these disorders is frontotemporal dementia (FTD). People with FTD experience progressive changes in social, behavioral, and language skills, and some also develop parkinsonism and/or motor neuron disease. The changes seen in FTD patients sometimes include bizarre behaviors, including obsessions, larceny, or even sexual deviancy.
CNDR researchers discovered in 2006 a link between FTD and Amyotrophic Lateral Sclerosis (ALS). In both diseases, study of brain tissue revealed accumulations of a misfolded protein called TDP-43. Now these scientists are trying to understand the basic biology of TDP-43 and the mechanisms that lead to the formation of aggregates.