An uncommon form of dementia hits at a younger age; drug makers are searching for a treatment

A type of brain disorder that affects people in their 50s and 60s is called frontotemporal dementia (FTD), which encompasses a group of rare degenerative brain diseases. FTD is believed to be the most prevalent form of dementia in people under age 60 years, and as many as 40% of cases may be inherited.

A type of brain disorder that affects people in their 50s and 60s is called frontotemporal dementia (FTD), which encompasses a group of rare degenerative brain diseases. FTD is believed to be the most prevalent form of dementia in people under age 60 years, and as many as 40% of cases may be inherited. The first symptoms of FTD include strange behavior, which can be mistaken for indications of a psychiatric problem. At least two Massachusetts-based drug companies, Alkermes and Arkuda Therapeutics, are developing possible treatments for a certain strain of FTD that experts say affects approximately 5,000 people nationwide. Jary Larsen, PhD, a neuropsychologist at the University of California San Francisco (UCSF), has determined that at least 20 of his relatives likely suffered from that particular strain, known as GRN-related frontotemporal dementia. This strain is caused by mutations in a gene for progranulin, a protein found in tissues throughout the body. People with the mutated gene have unusually low levels of the protein. Larsen and his siblings each had a 50/50 chance of inheriting a defective gene for the disease from their father. At least three siblings turned to genetic testing to confirm they did not have that gene, Larsen says. Both the Mayo Clinic in Rochester, MN, and UCSF were recently awarded a 5-year grant totaling more than $63 million to advance treatments for FTD.