Now this scares me somewhat......
Frontotemporal Degeneration: The Dementia Doctors Mistake For A Midlife Crisis
Date: April 24, 2014 Publication: Daily Health News Source: Bradford C. d!ckerson Print:
A loved one turns 50 (or so), impulsively buys a flashy sports car that he can’t afford, aggressively propositions women in restaurants, swears at his boss, then lobs insults at you when you suggest that he settle down. You might think that he’s having a somewhat tardy midlife crisis…but he’s not.
The real problem may be a type of dementia called frontotemporal degeneration (FTD) that leaves memory intact while causing disturbing changes in behavior, personality and/or language. FTD occurs equally in men and women. People with this progressive brain disorder often alienate friends and family, jeopardize their jobs, maybe even get into legal trouble—yet often they’re completely unable to recognize that they have a problem. And even if they do see their doctors, they’re likely to be misdiagnosed with depression or some other psychiatric problem, at least in the early stages.
That’s why they need all of us to be aware of the warning signs…so patients can get the help they need and appropriate plans can be made for their future. Here’s what you should know about FTD…
RECOGNIZING AN UNRECOGNIZED DISORDER
One reason why FTD often goes undiagnosed is that it tends to strike earlier than other forms of dementia, typically developing in a person’s 50s or 60s—which people may think of as “too young” for dementia. Alzheimer’s disease, in comparison, appears 10 to 13 years later, on average. Another source of confusion is the fact that FTD patients usually ace memory tests used to detect Alzheimer’s—for instance, they often have no trouble keeping track of day-to-day events and staying oriented to space and time. Also, many people have never heard of FTD (also called frontotemporal dementia or Pick’s disease) even though it accounts for up to 20% of all dementia cases.
The term FTD actually encompasses several related disorders, all of which are characterized by progressive damage to the frontal lobe, a brain area associated with decision-making and behavior control…and/or the temporal lobe, which is associated with emotion and language.
Though genetics plays a role in about 15% to 20% of FTD cases, the majority of cases do not appear to be inherited. Doctors aren’t sure what triggers FTD, though they do know that in some cases the disorder occurs when a protein called tau degenerates and is no longer able to perform its main function, which is to stabilize the structure of brain cells, said Bradford C. d!ckerson, MD, an associate professor of neurology at Harvard Medical School and director of the Frontotemporal Disorders Unit at Massachusetts General Hospital. Other cases result from changes in other proteins, he added. As a result, neurons die or become shrunken and misshapen…scar tissue forms…and there is an accumulation of abnormal protein within brain cells.
As a consequence of the brain degeneration, patients experience progressive changes in behavior, language and/or motor skills. Symptoms may include any or all of the following…
Personality changes—including loss of empathy…heightened aggression…and increasingly inappropriate social behavior (hypersexuality, excessive swearing, laughing at others’ misfortunes, etc.).
Impulsivity, distractibility, poor judgment and impaired decision-making skills. Examples: Impulsive spending or financial risk-taking…inability to plan and prepare a meal…inattention to personal hygiene…driving like a reckless teenager.
Compulsive behaviors, such as repetitive hand clapping, incessant humming or shoplifting…and intense cravings for sweets or other carbohydrates.
Apathy, lack of motivation, listlessness, irritability—symptoms easily mistaken for depression.
Increasing problems with language, such as difficulty naming familiar objects, expressing thoughts and comprehending words. Ultimately, some patients are rendered incapable of speaking…and some lose the ability to comprehend the speech of others.
Muscle rigidity, weakness and tremors, which can lead to trouble balancing and walking. Early on, such symptoms often are misconstrued as signs of Parkinson’s disease. Eventually patients become unable to perform daily activities.
Sadly, FTD is ultimately fatal, with the duration of the disease ranging from two years to more than 10 years.
It’s tragic to think that, during the early stages, an undiagnosed patient might so alienate his/her family and friends—who are understandably bewildered and upset by the person’s disturbing behavior—that he winds up alone during the later stages, with no loved ones to help him. That’s one reason why a diagnosis is so crucial.
GETTING HELP
“In many people with FTD, the changes in the brain reduce insight and self-awareness to a degree that the patient is not able to recognize his impairments,” Dr. d!ckerson said. So if you suspect that a loved one has FTD, insist on accompanying him to see his doctor and request a referral to a neurologist with expertise in the disorder. No single diagnostic test exists to confirm or rule out FTD. Diagnosis is based on a detailed cognitive and neurological exam…a neuropsychological exam to assess behavior, language and decision-making functions…and neuroimaging tests such as MRI or PET scans to check for atrophy in various areas of the brain.
There is no known way to cure FTD or stop the progression of the disease, but there are treatments that can help ease some of the symptoms. For instance…
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Antidepressant selective serotonin reuptake inhibitors may be able to help control aggressive behaviors, impulsivity and carbohydrate cravings.
Antipsychotic or mood-stabilizing medications may be able to help manage irrational or compulsive behaviors and ease agitation. However, these drugs can have serious side effects, including accelerating heart disease, so the benefits need to be carefully weighed against the risks, Dr. d!ckerson said.
Speech and language therapy can help a patient learn alternative ways to communicate (such as with gestures or by pointing to pictures), reducing frustration and improving quality of life.
Occupational therapy makes it easier for a patient to perform basic activities of daily living and to avoid falls.
Counseling or psychotherapy rarely helps FTD patients, Dr. d!ckerson noted—but it almost always helps their families.
FTD eventually progresses to the point that patients need 24-hour care. The sooner a patient is properly diagnosed, the sooner plans can be put in place to make his life as secure and comfortable as possible.
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