What Is Primary Progressive Aphasia Dementia?
Various types of dementia can impact individuals in unique ways. One such form is primary progressive aphasia. To understand this condition, it may be helpful to examine its symptoms, the various variants of this condition, and the treatments available for individuals and their families.
A subtype of frontotemporal dementia: What is primary progressive aphasia dementia?
Primary progressive aphasia (PPA) dementia is a neurodegenerative condition that can affect an individual’s speech in numerous ways. Categorized as a rare disease by the Genetic and Rare Diseases Information Center of the National Center for Advancing Translational Sciences (NCATS), PPA affects fewer than 200,000 people across the United States.
What is dementia?
Dementia tends to cause various challenges related to cognitive functioning. Regardless of the form of dementia a person is diagnosed with, the condition is typically the result of cerebral changes that cause neurons to stop functioning correctly.
According to the National Institute on Aging, dementia is not a normal part of the aging process, but it is more common among older individuals—Often those over 65. Some people may have more risk factors, as may be the case in people who have close family members with Alzheimer’s disease. However, having a family member with dementia does not guarantee that a person will develop the disease.
While Alzheimer’s tends to affect people who are over 65, PPA dementia often begins prior to the age of 65, with a mean age of onset between 59 and 64, depending on the subtype.
Dementia can affect people in different ways, but the following are some common symptoms:
- Memory loss and confusion
- Difficulty completing everyday tasks
- A tendency to repeat questions
- Loss of interest in activities
- Poor judgment
- Problems with balance
- A tendency to wander or get lost
How does primary progressive aphasia affect people?
Primary progressive aphasia (PPA) is a rare condition that affects some people living with dementia. PPA tends to affect a person’s speech and language processing faculties gradually. Not everyone experiences PPA in the same way. Researchers have identified three variants of PPA: the semantic variant, the nonfluent/agrammatic variant, and the logopenic variant.
Semantic variant of Primary Progressive Aphasia Dementia
A person with the semantic variant of PPA (svPPA) may experience trouble understanding single words they previously understood. In time, they may have difficulty identifying objects or even people.
Non-fluent/agrammatic variant: Speech and language is affected
Someone living with the non-fluent/agrammatic variant of PPA (naPPA) may mispronounce words or speak or write with different word order, even if they never showed signs of poor grammar before. They typically still understand single words but may have difficulty comprehending complex sentences.
Logopenic variant of PPA diagnosis
People with the logopenic variant of PPA (lvPPA) may pause to find the right word during conversations, even though they may understand the word when it is presented to them. When they cannot remember a word, they may use substitute phrases like “that thing.” While researchers often use these variants, or sub-types, to discuss symptoms, a person can have symptoms from multiple variants.
Causes of the rare nervous system syndrome, primary progressive aphasia disease
Primary progressive aphasia (PPA) is believed to be caused by malfunctioning cells in brain regions responsible for language processing. Doctors may diagnose PPA after ruling out other potential causes, such as stroke, cancer in the brain tissue, or head trauma.
If you are experiencing trauma, support is available. Please see our Get Help Now page for more resources.
The neurodegeneration from the death of brain cells may be caused by Alzheimer’s disease or two types of frontotemporal lobar degeneration (FTLG): FTLG with abnormal tau protein accumulation (FTLD-tau) or FTLG with abnormal TDP-43 accumulation (FTLD-TDP-43). In all three of these forms of neurodegeneration, abnormal proteins tend to attack regions of the brain responsible for language, often in the frontal, parietal, or temporal areas of the left half of the brain.
Treatments for primary progressive aphasia symptoms
While there currently is no cure for primary progressive aphasia (PPA), various treatments may mitigate symptoms. PPA treatment tends to involve a team of professionals, including neurologists, neuropsychologists, psychiatrists, social workers, physical and occupational therapists, and speech pathologists.
Medical and psychiatric care for frontotemporal dementia and its subtypes
A neurologist often provides the diagnosis of PPA dementia which is a subtype of frontotemporal dementia, while a neuropsychologist may assess a client’s cognitive function through various tests. Psychiatrists may assist clients with behavioral symptoms, which may involve prescription medication, particularly in cases where Alzheimer’s disease (AD) is suspected of being the cause of neurodegeneration.
Consult a medical doctor before starting, changing, or stopping a medication for any condition. The information in this article is not a replacement for medical advice or diagnosis.
Speech and language therapy as the disease progresses
Speech and language pathologists may also be part of a treatment team for individuals with PPA. These professionals may be able to tailor treatment techniques according to each individual's symptoms. Speech therapy may also involve training loved ones of a person with PPA to teach them communication skills relevant to the specific language-related symptoms the person experiences.
Social work assistance
Social workers may also be able to help families adjust to the challenges posed by social and behavioral symptoms. For example, as family roles change with caregiving responsibilities, a social worker may support family members in adjusting throughout this process, which may be ongoing as the condition progresses.
Social workers may also ensure family members care for their mental health and offer them information about family counseling and support groups. As PPA dementia tends to affect people in the later stages of life, social workers may advise families on advanced directives, living wills, and estate planning.
Comprehensive care team assistance for brain cells, slowed speech, and memory problems
As a progressive condition, PPA dementia can lead to other symptoms aside from language challenges. For example, individuals with PPA may start to develop memory problems and challenges related to attention, behavior, and decision-making. Some people may also experience physical challenges, such as problems with swallowing or moving in certain ways. A comprehensive care team with the above professionals may be beneficial to address these and other symptoms that a person living with PPA can experience.
Therapy for individuals with PPA diagnosis and their families
Individuals with PPA may benefit from speaking with a licensed therapist, especially toward the beginning of their condition. Doing so may allow them to adjust to the reality of living with a progressive condition. The therapeutic process may also help them learn to communicate effectively with loved ones, who may benefit from therapy as well.
Communication becomes less verbal, the memory becomes more challenging, and brain cells become more damaged
Family members may experience distress as they observe their loved one slowly experience increasing challenges with memory, speech, or overall communication. The loved ones of a person with PPA and other forms of dementia may experience challenging emotions related to the anticipation of loss. They may feel a mix of sadness and frustration with the caregiving process. Therapy may help them process these emotions in a safe environment free of judgment.
As a person’s dementia progresses, they may require more intensive care from loved ones, which may make it difficult for them to leave home for therapy appointments. In these circumstances, online therapy through a platform like BetterHelp may be a more viable option, as they can participate in therapy from home via audio, video, or live chat. With some online therapy platforms, caregivers can also reach out to their therapist in between sessions through in-app messaging, and the therapist can respond as soon as they are available.
Research shows that online therapy can be effective for caregivers of individuals with dementia. One systematic review published in the Journal of Medical Internet Research looked at 17 studies with more than 2,200 family caregivers of individuals living with dementia. The researchers included 14 of the studies in a meta-analysis, which found that “internet-based supportive interventions significantly ameliorated depressive symptoms, perceived stress, anxiety, and self-efficacy in dementia caregivers.”
Takeaway
Therapy may also help caregivers and loved ones, who often have to adapt their communication style depending on the specific language-related symptoms a person experiences. Therapy for loved ones may also be helpful as they take on greater caregiving responsibilities as the condition progresses. Consider reaching out to a provider online or in your area to develop a treatment plan.
- Previous Article
- Next Article