Diagnosis generally involves a careful medical history with focus on behavioral changes reported by family members and others close to the patient; neuropsychological assessments of language, memory, executive functioning, and visual-spatial skills; and neuroimaging to determine the extent of atrophy.

There are no therapies that can either slow or halt the progress of frontotemporal dementia, although atypical antipsychotic agents targeted toward specific undesirable behaviors may make those behaviors more manageable, Dr. Knopman said.

Practical strategies for managing the disorders include keeping the patient from driving, making financial decisions, or from any situations in which judgment and reasoning are important. Full-time nursing facilities may be required for patients in advanced stages of disease.

Frontotemporal degenerative disorders have a stronger genetic component than Alzheimer's disease, with familial forms accounting for about 25% of all cases, Dr. Knopman said.

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