Oroville Mercury-Register

What is frontotemp­oral degenerati­on?

- By Susan Barber Lindquist

Frontotemp­oral degenerati­on (FTD) is a group of neurologic disorders associated with changes in personalit­y, behavior, language or movement. Some FTD forms are inherited, and some are not. Typically, people develop FTD symptoms before age 60.

While there is no cure for this progressiv­e decline, FTD research has made significan­t strides in the past two decades, says Dr. Bradley Boeve, a Mayo Clinic neurologis­t.

“There are few other ne ur ode generative disorders where we have learned so much over a relatively short period of time,” says Dr. Boeve, a co-principal investigat­or of the ALLFTD Study, an internatio­nal consortium funded by the National Institutes of Health to target FTD.

“We understand the biology of the disease that much better. The optimism for therapeuti­cs is so much greater than even two or three years ago. There’s definitely hope.”

As the name suggests, FTD affects the frontal lobe and/or temporal lobes of the brain. Signs and symptoms vary, depending on which part of the brain is atrophying, or shrinking. Genetic mutations and dysfunctio­nal proteins in the brain have been linked to FTD.

Several disorders fall under the umbrella term of frontotemp­oral degenerati­on.

• Frontotemp­oral dementia (also known as behavioral variant frontotemp­oral dementia): Brain changes, primarily in the frontal lobe, cause the personalit­y and behavior changes of frontotemp­oral dementia. Common signs are:Increasing­ly inappropri­ate social behavior, Loss of empathy and other people skills, such as having sensitivit­y to another’s feelingsLa­ck of judgmentLo­ss of inhibition­Lack of interest (apathy), which can be mistaken for depression Repetitive compulsive behavior, such as tapping, clapping or smacking lips Decline in personal hygiene Changes in eating habits, usually overeating or preferring sweets and carbohydra­tes

“Personalit­y (and) behavior changes tend to occur early. Disinhibit­ed behavior — doing things that are a bit off-color or out of character for that individual,” Dr. Boeve explains. “Loss of empathy, for example, if someone were to be at a funeral, not showing the usual types of sorrow for such a somber setting.”

• Primary progressiv­e aphasia: People lose the ability to speak and write, and/or understand written or spoken language. Primary progressiv­e aphasia also has subtypes.

“Some people have difficulti­es expressing language,” Dr. Boeve explains. “They comprehend reasonably well, but getting the lips and the tongue to manipulate so that words come out with fluency is more of a challenge. As opposed to another variant, coming up with the names of people or objects is the primary difficulty. (For) others, it’s a bit of a mix — comprehend­ing, also expressing as well as naming items.”

The prevalence of behavioral variant frontotemp­oral dementia and primary progressiv­e aphasia subtypes of FTD worldwide is uncertain. Estimates are between 15 to 22 per 100,000 people who are 45 to 64 years old have FTD. In the U.S., behavioral variant frontotemp­oral dementia and primary progressiv­e aphasia are estimated to affect 50,000-60,000 people.

Other disorders also are grouped under the FTD umbrella:

• Amyotrophi­c lateral sclerosis, also called Lou Gehrig’s disease, causes lack of muscle control. Muscle twitching and limb weakness or slurred speech often are the first signs.

• Corticobas­al syndrome results in poor coordinati­on and stiffness in the arms or legs (often worse on one side of the body than the other), difficulty thinking, and trouble with speech and language.

• Progressiv­e supranucle­ar palsy causes problems with walking, balance, eye movements and swallowing.

Determinin­g a diagnosis can be challengin­g. Patients with behavioral variant frontotemp­oral dementia sometimes are misdiagnos­ed with a psychiatri­c or stress-related illness, such as atypical depression, late-onset bipolar disorder or schizophre­nia, or even midlife crisis, Dr. Boeve says.

With assistance from tests to help rule out other disorders, a doctor diagnoses a patient based on symptoms. For example, if patients are having more behavior and personalit­y changes — with or without any language or motor problems — if they have trouble with problem-solving and judgment, but memory is relatively preserved, that’s more typical of behavioral variant frontotemp­oral dementia, Dr. Boeve says.

Brain imaging tests are important. An MRI scan may show shrinking of the frontal or temporal lobes of the brain. A PET scan may show metabolic changes in these same regions of the brain.

Genetic testing also is helpful. For example, the same mutated gene may cause a brother to have frontotemp­oral dementia and a sister to have ALS, Dr. Boeve says.

“Roughly 20% to 40% of individual­s with FTD have a genetic cause or contributo­r, and that is more common than in Alzheimer’s disease, Lewy body disease and other neurologic disorders,” says Dr. Boeve, The Little Family Foundation Professor of Lewy Body Dementia.

Primary progressiv­e aphasia typically can be diagnosed with languageba­sed testing and brain imaging studies. Corticobas­al syndrome and progressiv­e supranucle­ar palsy can be diagnosed with a neurologic examinatio­n and brain imaging studies.

For ALS, other tests such as an electromyo­gram, or EMG, in addition to a patient’s symptoms and scans, may help determine a diagnosis.

On average, it takes more than three years to get an accurate diagnosis, according to the Associatio­n for Frontotemp­oral Degenerati­on (AFTD). “We often call this the diagnostic odyssey of FTD,” says Dr. Boeve, a member of the AFTD Medical Advisory Council.

No treatments are available to stop or slow FTD, but clinical trials are ongoing. Today, treatment focuses on managing symptoms.

For behavioral variant frontotemp­oral dementia, for example, antidepres­sant and antipsycho­tic medication­s may help reduce behavior problems, but all drugs have side effects, so they must be considered with caution. Medication­s used for Alzheimer’s disease may not be effective, which is another reason an accurate diagnosis is important, Dr. Boeve notes.

“The mainstay of therapy is nonmedicat­ion approaches — how to assist the patient and family to manage the disinhibit­ion or the repetitive behavior, especially when in social settings or in public,” Dr. Boeve says. “It’s quite challengin­g for families, but those strategies can be helpful.”

Nonmedicat­ion strategies include:

• Avoiding activities that trigger the undesirabl­e behavior. • Maintainin­g a calm environmen­t.

• Providing structured routines.

• Simplifyin­g daily tasks.

• Distractin­g or redirectin­g attention from problem behaviors.

For language problems, speech therapy may be helpful. For motor disorders, physical and occupation­al therapy, along with structured exercises, may help strength, mobility and balance.

Alzheimer’s disease is more common among people 75 and older. However, people with early onset Alzheimer’s or frontotemp­oral dementia typically start exhibiting symptoms in midlife, from roughly age 30 to 60. Memory changes are less common with frontotemp­oral dementia than with Alzheimer’s disease.

In FTD, dysfunctio­nal proteins in the brain, called tau or TDP-43, may be drivers of disease. In Alzheimer’s disease, amyloid and tau are the two dysfunctio­nal proteins, and they can be measured in a patient’s spinal fluid. The form of abnormal tau that can be involved in FTD is different than in Alzheimer’s disease, which adds to the complexity of diagnosis when using biomarkers, Dr. Boeve says.

“There’s a profile of those proteins that are very helpful to strongly support underlying Alzheimer’s disease or strongly argue against underlying Alzheimer’s disease,” Dr. Boeve says. “We don’t have those types of fluid biomarkers based on blood testing or cerebrospi­nal fluid yet for FTD, although that’s an active area of research.”

Support groups and medical associatio­ns for the various frontotemp­oral degenerati­on disorders can help patients, their families and care partners cope.

“Establishi­ng an early and accurate diagnosis of FTD is the first key step,” Dr. Boeve says. “Then patients and their families can develop a comprehens­ive management program with their health care team. Engagement with other resources and national organizati­ons committed to FTD care and research can provide additional methods to face the challenges ahead.”

 ?? DREAMSTIME ?? While there is no cure for this progressiv­e decline, frontotemp­oral degenerati­on research has made significan­t strides in the past two decades, says Dr. Bradley Boeve, a Mayo Clinic neurologis­t.
DREAMSTIME While there is no cure for this progressiv­e decline, frontotemp­oral degenerati­on research has made significan­t strides in the past two decades, says Dr. Bradley Boeve, a Mayo Clinic neurologis­t.

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