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.
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.
About the Author
Andrew E. Budson, MD, Contributor
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 Harvard Medical School Academy. Graduating cum laude from Harvard Medical School in 1993, he has given over 650 local, national, and international grand rounds and other talks; published over 100 scientific papers, reviews, and book chapters; and co-authored or edited seven books. His book Seven Steps to Managing Your Memory: What’s Normal, What’s Not, and What to Do About It explains how individuals can distinguish changes in memory due to Alzheimer’s versus normal aging; what medications, vitamins, diets, and exercise regimes can help; and the best habits, strategies, and memory aids to use; it is being translated into Chinese and Korean. His book Memory Loss, Alzheimer’s Disease, and Dementia: A Practical Guide for Clinicians has been translated into Spanish, Portuguese, and Japanese. His latest book, Six Steps to Managing Alzheimer’s Disease and Dementia: A Guide for Families teaches caregivers how they can manage all the problems that come with dementia—and still take care of themselves. Website: Andrew Budson, MD Facebook: Andrew Budson, MD Twitter: @abudson View all posts by Andrew E. Budson, MD