Primary progressive aphasia involves many losses: Here’s what you need to know

illustration of a woman holding a hand to her forehead, with pixelated squares scattered around her head representing a memory problem

When you think about progressive brain disorders that cause dementia, you usually think of memory problems. But sometimes language problems — also known as aphasia — are the first symptom.

What’s aphasia?

Aphasia is a disorder of language because of injury to the brain. Strokes (when a blood clot blocks off an artery and a part of the brain dies) are the most common cause, although aphasia may also be caused by traumatic brain injuries, brain tumors, encephalitis, and almost anything else that damages the brain, including neurodegenerative diseases.

How neurodegenerative diseases cause aphasia

Neurodegenerative diseases are disorders that slowly and relentlessly damage the brain. After ruling out a brain tumor with an MRI scan, you can usually tell when aphasia is from a neurodegenerative disease, rather than a stroke or other cause, by its time course: Strokes happen within seconds to minutes. Encephalitis presents over hours to days. Neurodegenerative diseases cause symptoms over months to years.

Alzheimer’s disease is the most common neurodegenerative disease, but there are other types as well, such as frontotemporal lobar degeneration. Different neurodegenerative diseases damage different parts of the brain and cause different symptoms. When a neurodegenerative disease causes problems with language first and foremost, it is called primary progressive aphasia.

How is primary progressive aphasia diagnosed?

Primary progressive aphasia is generally diagnosed by a cognitive behavioral neurologist and/or a neuropsychologist who specializes in late-life disorders. The evaluation should include a careful history of any language and other problems that are present; a neurological examination; pencil-and-paper testing of thinking, memory, and language; blood tests to rule out vitamin deficiencies, thyroid disorders, infections, and other medical problems; and an MRI scan to look for strokes, tumors, and other abnormalities that can affect the brain’s structure.

The general criteria for primary progressive aphasia include:

  • difficulty with language is the most prominent clinical feature at the onset and initial phases of the neurodegenerative disease
  • these language problems are severe enough to cause impaired day-to-day functioning
  • other disorders that could cause the language problems have been looked for and are not present.

There are three major variants of primary progressive aphasia

Primary progressive aphasia is divided into different variants based on which aspect of language is disrupted.

Logopenic variant primary progressive aphasia causes word-finding difficulties. Individuals with this variant have trouble finding common, everyday words such as table, chair, blue, knee, celery, and honesty. They know what these words mean, however.

Semantic variant primary progressive aphasia causes difficulty in understanding what words mean. When given the word, individuals with this variant may not understand what a table or chair is, which color is blue, where to find their knee, what celery is good for, and what honesty means.

Nonfluent/agrammatic variant primary progressive aphasia causes effortful, halting speech in which individuals know what they want to say but cannot get the words out. When they can get words out, their sentences often have incorrect grammar. Although they know what the individual words mean, they may have trouble understanding a sentence with complex grammar, such as, “The lion was eaten by the tiger.”

Different primary progressive aphasia variants are caused by different diseases

These primary progressive aphasia variants are not diseases themselves. They are symptoms of brain problems. Not sure what I mean? Consider three other symptoms: fever, headache, and chest pain. As you know, each of these symptoms may be caused by different underlying diseases.

The logopenic variant of primary progressive aphasia is usually caused by Alzheimer’s disease. Does that surprise you? What this means is that although Alzheimer’s disease typically begins with memory loss, in some individuals it can start with trouble finding words. Memory problems typically begin a few years later. (Why do we call it Alzheimer’s disease if it doesn’t start with memory problems? Because Alzheimer’s disease is defined by the pathology that we see under the microscope when we examine the brain tissue, not by its symptoms.)

The semantic variant of primary progressive aphasia is usually caused by frontotemporal lobar degeneration, and specifically by accumulation of TDP-43. TDP-43 is an abnormal protein that accumulates in — and ultimately kills — brain cells.

The nonfluent/agrammatic variant of primary progressive is also usually caused by frontotemporal lobar degeneration, but this time it is most often due to tau pathology. Tau accumulation leads to tangles inside cells that damage and then destroy them.

Can primary progressive aphasia be treated?

The treatments available for primary progressive aphasia are generally strategies and systems to help individuals with these disorders communicate better.

  • Thinking of information related to the word they are looking for can sometimes help individuals with logopenic variant primary progressive aphasia. For example, if they are searching for the word lion, thinking of yellow, Africa, big cat, mane, and similar words may help.
  • Using your tone of voice, facial expression, and body language can be helpful to communicate with individuals with semantic variant primary progressive aphasia, as can pantomiming the message you are trying to convey.
  • Using pictures, either on paper or in a tablet-based application, can be helpful to individuals with all variants of primary progressive aphasia.

Unfortunately, there are no cures for primary progressive aphasia, and no medications that have been shown to be effective. Most patients with primary progressive aphasia develop other cognitive problems over time, leading to a more general dementia.

If you suspect that you (or your loved one) may have primary progressive aphasia, start by meeting with your doctor. If your doctor is concerned, they will send you (or your loved one) to the right specialist.

Concussion care for children and teens: What parents need to know

photo of a tween girl in bed with her back against pillows, looking ill and holding her left hand to the side of her face

Concussions are very common — in fact, they are the most common kind of traumatic brain injury (TBI). While most people recover completely, concussions sometimes lead to lifelong problems, as we’ve learned from the experiences of former National Football League players.

That’s why it’s important that we do everything we can to not just prevent concussions in children and teens, but to give them the right treatment when a concussion happens.

The problem for doctors, parents, and coaches has been that while we want to do the right thing when a child gets a concussion, it’s not always easy to know what the right thing is. To help, the Centers for Disease Control and Prevention (CDC) reviews all the research and makes recommendations to help guide us as we care for children with concussions.

Every child is different, and concussion care should reflect that

The recommendations reflect the fact that every child who has a concussion is different. Every injury is different, obviously, but it’s more than that. Some children are more likely to have trouble, such as those who have had prior concussions or have learning problems, mental health problems, or neurological problems.

Interestingly, children whose families are stressed for reasons such as poverty can take a longer time to recover from concussions. And there is a bit of a wild-card factor too: sometimes children unexpectedly take a long time to recover — or, conversely, recover very quickly.

What are the concussion care recommendations?

Practice guidelines developed by the CDC for health providers include these points:

  • Most children with concussions don’t need CT or MRI scans. If there was a severe injury or the child is having severe or unusual symptoms, then it’s worth doing to be sure there isn’t internal bleeding, a fracture, or some other injury. Most of the time with concussions, there is nothing to see — and it’s not worth the risk or expense involved in these imaging studies.
  • Use the right tool to make the diagnosis. There are some symptoms we associate with concussion, like bad headache, dizziness, loss of memory of the accident. But because it isn’t always clear, it’s helpful to use a checklist or questionnaire that is validated, meaning that it’s been shown to accurately pick out those with a concussion from those who simply have a bad clunk to the head and not a concussion.
  • When a child has a concussion, assess for risk factors for a prolonged recovery. As I said above, some children take longer to get better — and while we can never predict for sure, it’s important to think about that at the time of the injury.

What should parents know about concussions?

  • Most children and teens with concussions get completely better within one to three months. But it’s important that children, families, and coaches know what all the symptoms are after a concussion, and understand what’s normal and what is a sign of a problem. For example, trouble sleeping, dizziness, and moodiness can be normal, but if any of those symptoms are getting worse, it’s important to call the doctor.
  • Parents can help children return to normal activities after a concussion. Rest — of not just the body, but the mind too — is important for the first two to three days after a concussion, but after that it’s important to start getting back to normal. When people rest completely for longer than that, it actually takes them longer to get better.

Getting back to normal after a concussion

We used to think that total rest of the brain and body after a concussion was the best treatment. Increasingly, research shows that resuming normal activities is the better treatment. For example, recent research analyzing many studies showed that exercise can help speed recovery from concussion. The tricky part is figuring out how best to resume normal activities, because it is different for each child.

The basic idea is to start slow and see how the child does. If they do okay, they can do a bit more schoolwork or exercise. If they don’t do okay — meaning they have more symptoms — they should do less and go more slowly.

The process of getting back to normal life can take a few days, or a few months. It has to be tailored to each child and each situation, which is why collaboration with your pediatrician is so important. It’s also really important not to rush the process, especially when it comes to returning to a sport where concussions are common, such as football, hockey, or soccer. If a child gets another concussion while they are still recovering, it will take them much longer to get better, and put them at risk of permanent disabilities.

To learn more, visit the CDC’s Heads Up page.

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Enjoy avocados? Eating one a week may lower heart disease risk

Three dark green whole avocados and two light green half avocados, one holding the pit, arranged like petals of a flower against a yellow background

The creamy, pale green flesh of an avocado is full of nutrients closely tied to heart health. Now, a long-term study finds that eating at least two servings of this popular fruit per week is linked to a lower risk of cardiovascular disease.

Study co-author Dr. Frank Hu, the Frederick J. Stare Professor of Nutrition and Epidemiology at the Harvard T.H. Chan School of Public Health (HSPH), puts this finding in perspective. "This study adds to the evidence to support the benefits of healthy fat sources like avocados to help prevent cardiovascular disease," he says. A key take-home message is to substitute avocados for less-healthy foods such as butter, cheese, and processed meats, he adds.

Who was in the study?

The study included more than 110,000 people involved in two long-running Harvard studies: the Nurses’ Health Study and the Health Professionals Follow-up study. Most of the participants were white; they ranged in age from 30 to 75 and were free of heart disease and cancer when the study began.

Researchers assessed the participants’ diets via questionnaires given at the start of the study and then every four years. One question asked how much and how often people ate avocado. A serving was considered a half an avocado or one-half cup, cubed.

What were the findings?

During the 30-year follow-up, researchers documented 9,185 heart attacks and 5,290 strokes among the participants. Compared with people who never or rarely ate avocados, those who ate at least two servings each week had a 16% lower risk of cardiovascular disease and a 21% lower risk of experiencing a heart attack or related problem due to coronary artery disease. (Coronary artery disease refers to a narrowing or blockage in the blood vessels that supply the heart; it’s the most common type of cardiovascular disease.)

What makes avocados a heart-healthy choice?

Hass avocados, which have dark green, nubbly skin, are the most popular variety in the United States. They’re abundant in healthy fats, fiber, and several micronutrients associated with cardiovascular health:

  • Oleic acid. This monounsaturated fat is also plentiful in olives. Half an avocado has around 6.5 grams of oleic acid, or about the same amount found in a tablespoon of olive oil. Research shows that replacing foods high in saturated fat (such as butter, cheese, and meat) with those rich in unsaturated fats (such as avocados, nuts, and seeds) helps lower blood levels of harmful LDL cholesterol, a key culprit in coronary artery disease.
  • Fiber. One serving of avocado provides up to 20% of the daily recommended dietary intake of fiber, a nutrient that’s often lacking in the typical American diet. Fiber-rich diets may lower heart disease risk as much as 30%, probably because fiber helps lower not only cholesterol, but also blood pressure and body weight.
  • Vitamins, minerals, and more. Half an avocado provides 15% of daily recommended intake of folate (vitamin B9), 10% of potassium, and 5% of magnesium, as well as various plant-based compounds called phytochemicals. All of these nutrients — along with oleic acid and fiber — have been independently linked to better heart health.

The good news is that there are so many delicious ways to add avocado to your meals, says Dr. Hu. "I make avocado toast for breakfast, use avocado as a spread for sandwiches, and add them to salads," says Dr. Hu. Some people add avocado to their smoothies — and of course, there’s always guacamole (try this recipe from the HSPH’s Nutrition Source).