In Love and Loss – New York Times – 05/06/12

He threw away tax documents, got a ticket for trying to pass an ambulance and bought stock in companies that were obviously in trouble. Once a good cook, he burned every pot in the house. He became withdrawn and silent, and no longer spoke to his wife over dinner. That same failure to communicate got him fired from his job at a consulting firm.

Mr. French, now 71, has frontotemporal dementia — a little-known, poorly understood and frequently misdiagnosed group of brain diseases that eat away at personality and language. Although it was first recognized more than 100 years ago, there is still no cure or treatment, and patients survive an average of only eight years after the diagnosis.

This disease is different from Alzheimer’s, the most common form of dementia. But it is perhaps even more devastating, because it strikes younger people, progresses faster and, unlike Alzheimer’s, does not attack memory at first but begins with silence, apathy or bizarre personality changes. It is thought to afflict at least 50,000 to 60,000 people in the United States.

Frontotemporal dementia, also called frontotemporal degeneration or Pick’s disease, refers to a group of diseases that destroy nerve centers in the frontal and temporal lobes — the home of decision-making, emotion, judgment, behavior and language. Some forms of the disease also cause movement disorders. Most cases occur sporadically, in people with no family history of the illness — like Michael French — but a small percentage are inherited.

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