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What is frontotemporal dementia?

Concept of confusion, yellow cutout of head with scribbles and question marks in brain and top of head opening; turquoise blue background

Many people know the form of dementia called Alzheimer’s disease. But what is frontotemporal dementia (FTD)? Damage to nerves in certain parts of the brain causes a group of frontotemporal disorders, affecting behavior and language as I’ll describe below.

Early signs of frontotemporal dementia

Have you noticed someone behaving differently? Is your coworker doing odd things, such as slapping each door as they walk down the hall? Or has your previously kind and caring spouse lost their capacity for empathy, such that when you told them about your cancer diagnosis, they complained that your treatment schedule would interfere with their golf game? If so, they might be showing early signs of the behavioral variant of FTD.

Maybe there’s a problem with language, rather than behavior. Perhaps it started with difficulty finding words (like any older adult), but is your sibling now having trouble with grammar and getting out an intelligible sentence? Or does your friend not know the meaning of some ordinary words, like pizza, lemonade, wood, or metal? If so, they might be showing signs of primary progressive aphasia, which may also be due to FTD.

A common pathology inside the brain

What’s the connection between these behavior and language problems? Why are they both part of FTD?

Both have the same underlying causes: a family of abnormal proteins that can be seen under the microscope. In fact, more than a dozen different pathologies can cause FTD. Each of them can lead to either behavioral variant frontotemporal dementia or the language difficulties of primary progressive aphasia.

Location, location, location

How can the same pathology — the same abnormal protein — lead to either behavior problems or language problems, or sometimes both? The answer is, it depends on where the pathology is.

The frontal lobes of your brain, behind your forehead, regulate and guide your personality, judgement, and behavior. So, if the frontotemporal pathology is in this region, it will cause changes in personality, judgement, and behavior.

The left temporal lobe (near your left ear and temple) and a part of the left frontal lobe just above it are the critical brain regions for language. When these areas are affected by frontotemporal pathology, language problems develop.

How does frontotemporal dementia compare with Alzheimer’s disease?

Frontotemporal dementia affects people in middle age, usually between ages 45 and 65, although one-quarter of individuals are diagnosed after age 65. Alzheimer’s usually affects people over 65.

In terms of symptoms, people with frontotemporal dementia experience either language or behavior problems, whereas people with Alzheimer’s disease — the most common cause of dementia — usually have memory problems.

Because more than 12 different abnormal proteins can cause frontotemporal dementia, it has a very variable time course. From the time of diagnosis, people with frontotemporal dementia need nursing home–level care in two to 20 years. The typical range with Alzheimer’s disease is four to 12 years.

Who is at risk for FTD?

Up to 40% of cases of frontotemporal dementia run in families, but that means at least 60% of cases do not. Unfortunately, everyone is at risk for frontotemporal dementia as they approach middle age.

What are common signs of the behavioral variant?

There are six common signs of behavioral variant frontotemporal dementia, and most people with the disorder have at least three of them. They are:

  • loss of self-control
  • apathy or inertia (not wanting to do anything)
  • loss of sympathy or empathy
  • repetitive or compulsive, ritualistic behavior
  • uncontrolled or unusual eating
  • difficulty doing complicated tasks.

One individual I cared for with this disorder would walk up to strangers, stand closer than would be comfortable, and say loudly, “You’re handsome!” Another would eat almost anything left out in the kitchen. One woman I treated with this disorder tried to pick up men from a restaurant — while her husband was sitting at the next table. A previously kind and shy grandfather with frontotemporal dementia began to ask his daughter-in-law for sexual favors.

What are common signs of the language variants?

Two variants of primary progressive aphasia are part of the frontotemporal dementia family of diseases. Common signs are:

  • difficulty getting words and sentences out, although the meaning of words is preserved (nonfluent or agrammatic variant). People become frustrated because they know what they want to say but find it difficult or impossible to do so.
  • losing the meaning of words (semantic variant). I had one patient who did not know the meaning of the words shoe, pants, foot, knee, elbow, and many other words related to clothing and parts of the body.

Can frontotemporal dementia be treated?

Currently, there is no cure or way to slow these disorders down, so treatment is supportive. SSRI medications (selective serotonin reuptake inhibitors) can help with some disinhibited behaviors.

Speech therapy can be helpful, at least initially, with primary progressive aphasia, but thus far no medications are effective.

What can I do if I suspect that someone has frontotemporal dementia?

FTD is difficult to diagnose. Because it affects people in middle age, dementia is usually not suspected. Early in the disease, people are often thought to be having a midlife crisis, depression, or perhaps a drug or alcohol problem. Many marriages end prior to the diagnosis because the spouse with the disorder has grown self-absorbed and inconsiderate over several years.

If you do suspect the disorder, start by simply asking the person if there is anything that you can help with. You may find out that it is another problem entirely. But if it is becoming clear that this or another form of dementia may be involved, encourage them and their family to discuss this possibility with their doctor.

About the Author

photo of Andrew E. Budson, MD

Andrew E. Budson, MD,

Contributor; Editorial Advisory Board Member, Harvard Health Publishing

Dr. Andrew E. Budson is chief of cognitive & behavioral neurology at the Veterans Affairs Boston Healthcare System, lecturer in neurology at Harvard Medical School, and chair of the Science of Learning Innovation Group at the … See Full Bio View all posts by Andrew E. Budson, MD

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RECIPES SPORTS THE-BEAUTY

What makes your heart skip a beat?

Light tracings from an electrocardiogram in the background against a red backdrop; heart rhythm tracings in thicker white lines forming into a heart shape in the middle

Love isn’t the only reason your heart may skip a beat. While abnormal heartbeats can be alarming, they’re usually harmless. They occur for different reasons. Which types are common — and when should you be concerned?

Palpitations

Your heartbeat normally keeps a predictable pace: speeding up when you’re active and slowing down when you rest. But many people notice odd heart sensations called palpitations at least once in a while. People usually say it feels as though their heart has skipped a beat, or is racing or pounding.

“One common scenario is a person who feels their heart is racing, but if you look at their electrocardiogram (ECG), It’s totally normal,” says cardiologist Alfred E. Buxton, professor of medicine at Harvard Medical School.

A heightened awareness of normal heart rhythms may occur more in people who wear smartwatches with heart rate monitors, he adds. “People with a resting heart rate of 60 beats per minute are concerned when their heart rate goes up to 90, but that’s still in the normal range,” he says.

Ectopic beats

The sensation that your heart has skipped a beat also occurs when the heart’s upper chambers (atria) or lower chambers (ventricles) contract slightly earlier than normal.

During the next beat, the atria pause a bit longer to get back into a normal rhythm. The heart’s lower chambers (ventricles) then squeeze forcefully to clear out the excess blood that accumulates during that pause. They also can contract earlier than usual, which may make you feel like your heart has briefly stopped and restarted.

Known as ectopic beats, both types of these premature contractions may cause a brief pounding sensation. However, this is nothing to worry about. “I often tell my patients that the fact they feel these beats is usually a sign that their heart is healthy. A weak, sick heart can’t exert a forceful beat,” says Dr. Buxton.

AV block and bundle branch block

Electrical impulses tell your heart to pump. They travel through the right and left sides of your heart. But sometimes the impulses travel more slowly than normal or irregularly, causing a condition called AV block. There are various degrees of AV block, some benign, others associated with extremely slow heart rates that may be dangerous.

Another electrical conduction irregularity is a bundle branch block. This results from an abnormal activation pattern of the ventricles that squeeze blood out of the heart to the rest of the body. The most common is right bundle branch block, which usually doesn’t cause obvious symptoms. It may be spotted during an ECG, and can simply reflect the gradual aging of the heart’s conduction system. However, sometimes a right bundle branch block is caused by underlying damage from a heart attack, heart inflammation or infection, or high pressure in the pulmonary arteries.

A left bundle branch block may occur as an isolated phenomenon, or may be caused by a variety of underlying conditions. In some cases, left bundle branch block may lead to abnormal function of the left ventricle, a condition that is sometimes corrected by special pacemakers.

Atrial fibrillation

An electrical misfire in the atria can cause atrial fibrillation, an uncoordinated quivering of the atria that raises the risk for stroke. Commonly known as afib, this heart rhythm problem can come and go, lasting only a few minutes or sometimes for days or even longer. And while some people report a fluttering sensation in their chest or a rapid, irregular heartbeat during an episode of afib, other people don’t have any symptoms.

Certain smartwatches that can record a brief ECG may be able to detect afib. But Dr. Buxton says they’re not sensitive or specific enough to reliably diagnose the problem. “Sometimes the watch tells you that you have afib when you don’t, or vice versa,” he says.

The heart rate monitoring feature may be helpful, however. In people younger than 65, the heart rate can soar to 170 beats per minute or higher during a bout of afib. But for those in their 70s and 80s, who are more likely to have afib, the heart rate usually doesn’t get that high.

When should you be concerned about irregular heartbeats?

An irregular heartbeat, such as racing, fluttering, or skipping a beat, is usually harmless. Even in cases when palpitations are frequent and bothersome (which occurs rarely), reassurance may be the only treatment needed.

But you should contact your doctor if you notice other symptoms accompanying an unusual heartbeat, such as feeling

  • chest pain
  • dizzy
  • lightheaded
  • tired
  • breathless
  • as though you’re going to faint.

People who have been told they have a bundle branch block may need periodic ECGs to monitor their condition. They should also be alert to symptoms such as dizziness or fainting, which can happen if the blockage worsens or occurs on both sides and causes a low heart rate.

About the Author

photo of Julie Corliss

Julie Corliss, Executive Editor, Harvard Heart Letter

Julie Corliss is the executive editor of the Harvard Heart Letter. Before working at Harvard, she was a medical writer and editor at HealthNews, a consumer newsletter affiliated with The New England Journal of Medicine. She … See Full Bio View all posts by Julie Corliss

About the Reviewer

photo of Robert H. Shmerling, MD

Robert H. Shmerling, MD,

Senior Faculty Editor, Harvard Health Publishing; Editorial Advisory Board Member, Harvard Health Publishing

Dr. Robert H. Shmerling is the former clinical chief of the division of rheumatology at Beth Israel Deaconess Medical Center (BIDMC), and is a current member of the corresponding faculty in medicine at Harvard Medical School. … See Full Bio View all posts by Robert H. Shmerling, MD

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RECIPES SPORTS THE-BEAUTY

Can electrical brain stimulation boost attention, memory, and more?

A brain shape against a dark background, filled with vibrant, multicolored strands of light representing brain waves

Imagine this as a morning routine that replaces your first cup of coffee:

You wake up feeling a bit foggy, so you slip on a wearable device that looks like an extra-thick headband. You turn on the power source and settle in while electrical current flows into your brain. Twenty minutes later, feeling more focused and energized, you start your busy day feeling grateful for this new technology.

If this scenario sounds strange to you, I’m with you. And yet, hype around transcranial direct current stimulation (tDCS) is growing for an expanding list of conditions such as depression, ADHD, and even Alzheimer’s disease. A recent ad for one tDCS device urges you to “elevate your performance.” But before you give this a try, read on.

What is transcranial direct current stimulation?

Brain stimulation therapies aim to activate or inhibit parts of the brain. tDCS has been around for years, but its popularity has spiked over the last decade.

tDCS devices use headgear that may look like a swim cap or headband to position electrodes against the scalp. When a power source is switched on, the electrodes deliver low levels of electrical current to the brain. A typical session lasts 20 to 30 minutes and may be repeated over days or weeks.

Three better-known brain stimulation therapies are:

  • Transcranial magnetic stimulation (TMS): A device worn over the forehead stimulates specific areas of the brain by changing nearby magnetic fields. TMS is cleared by the FDA to treat depression that hasn’t responded to standard medicines, and for obsessive-compulsive disorder.
  • Electroconvulsive therapy (ECT): An electric current flowing through electrodes placed at specific locations on the scalp causes a brief seizure while a patient is under anesthesia. In use since the late 1930s, ECT can be highly effective for severe depression that hasn’t responded to standard therapies. It uses higher levels of electrical current than tDCS. That’s why it requires close medical supervision and is generally administered in a hospital or specialized clinic.
  • Deep brain stimulation (DBS): Electrodes surgically implanted in specific areas of the brain generate electrical pulses. DBS is used to treat conditions such as Parkinson’s disease, epilepsy, or tremors that don’t improve with medicines.

What claims are made for tDCS?

The brain normally functions by sending and receiving tiny electrical signals between nerve cells. Stimulating specific regions of the brain with low levels of electricity might improve focus or memory, mood, or even dementia, according to tDCS advocates.

Some claims say tDCS can

  • improve mental clarity, focus, and memory
  • increase energy and motivation
  • relieve so-called brain fog following COVID-19, Lyme disease, or other conditions
  • reduce depression or anxiety
  • reduce cravings among smokers or people with drug addiction
  • improve symptoms of ADHD or Alzheimer’s disease.

Does tDCS work?

The jury is still out. Research suggests that tDCS holds promise for certain conditions, but techniques tested through research may differ from devices sold commercially for at-home use. For example, electrodes may be positioned more precisely over an area of the brain, and how current is delivered, session length, or number of sessions may differ.

Currently, small, short-term studies show that tDCS may benefit people with:

  • Depression: An analysis of 10 randomized trials found some participants were more likely to report fewer symptoms of depression, or remission of depression, after a course of tDCS treatment compared with sham treatment.
  • Alzheimer’s disease: A review of seven studies found that tDCS lasting 20 to 40 minutes improved memory and other cognitive measures in people with mild to moderate Alzheimer’s disease.
  • ADHD: One randomized trial of 64 adults with ADHD found improved attention after 30 minutes of tDCS daily for a month.

The FDA has not cleared tDCS for any health condition, and it is considered investigational. More research with positive results and reassuring safety data are needed before tDCS gets a thumbs-up from regulators.

That’s probably why some ads for tDCS note in fine print that it is not a medical device and is only for recreational use.

Does tDCS have downsides?

While the FDA assesses tDCS as safe for adults, there are downsides to consider. For example, treatment may cause itching, irritation, or small burns at the sites of the electrodes. Some users complain of fatigue or headache.

There are no large, long-term studies of tDCS, so overall safety is uncertain. Some experts believe at-home use raises many questions, such as how much of the brain beyond targeted areas is affected, what inconsistent approaches to tDCS use might do, and how long changes in the brain — intended or not — could last.

Very limited research has been done in children. So, the consequences for a child’s developing brain aren’t clear.

Finally, tDCS devices can be expensive (several hundred dollars or more), and generally are not covered by health insurance.

The bottom line

It’s not yet clear how tDCS should be used, or who is most likely to benefit from it. If you’re interested in pursuing tDCS, understand that there’s still a lot we don’t know.

If you’re more skeptical and risk-averse (like me), you may want to wait for more definitive research regarding its benefits and risks — and for now, stick with your morning coffee to clear your mind.

Follow me on Twitter @RobShmerling

About the Author

photo of Robert H. Shmerling, MD

Robert H. Shmerling, MD,

Senior Faculty Editor, Harvard Health Publishing; Editorial Advisory Board Member, Harvard Health Publishing

Dr. Robert H. Shmerling is the former clinical chief of the division of rheumatology at Beth Israel Deaconess Medical Center (BIDMC), and is a current member of the corresponding faculty in medicine at Harvard Medical School. … See Full Bio View all posts by Robert H. Shmerling, MD