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.
What stage of dementia is aphasia?
Primary progressive aphasia is not a stage of dementia, but a specific type of dementia. There are three additional types of aphasia: Broca’s aphasia, Wernicke’s aphasia, and anomic aphasia.
Those with Broca’s aphasia typically experience severely reduced speech and limited vocabulary. They may be able to read and understand speech, but they usually have trouble writing.
Meanwhile, individuals with Wernicke’s aphasia can normally speak well and use long sentences. However, what they say often doesn’t make sense. They may struggle to understand language when spoken to, and their reading and writing abilities are usually impaired.
Anomic aphasia generally involves difficulty finding the right words both in speech and in writing. Individuals with this type of aphasia typically have trouble remembering common words like nouns and verbs. Their speech may be full of vague expressions of frustration due to their trouble speaking and saying what they wish to say.
Primary progressive aphasia involves progressively worsening language impairments. Those with PPA may have trouble understanding the meaning of complex verbal information and speak in a vague or repetitive manner. Over time, a person with this type of aphasia usually loses their ability to understand others, speak, read, and write. However, other thinking skills, such as insight, judgment, memory, and reasoning, aren’t necessarily impacted as people progress into more severe aphasia.
What is the number one trigger for dementia?
The most significant risk factors for dementia (an umbrella term for various types of cognitive decline) are age and family history or genetics. However, it’s possible to go to a great length to decrease the possibility of developing dementia by adjusting various lifestyle factors.
Alzheimer’s disease, dementia, frontotemporal degeneration, and other kinds of cognitive decline can come with a variety of symptoms, sometimes involving trouble with producing words or finding the correct words, not making sense when speaking, inappropriate social behavior, behavioral changes, and personality changes. Often, the first sign of dementia is memory loss, even with a mild form of dementia. Most people with initial symptoms of dementia can benefit from seeking a clinical diagnosis and treatment.
What is the life expectancy of someone with aphasia?
While there is limited research on this topic, studies suggest that once a person develops symptoms of aphasia, they usually live for approximately seven to 12 years. Over this time, a person’s ability to understand and use language usually declines.
How is aphasia different from dementia?
Aphasia is a type of dementia. While dementia is an overarching term describing cognitive decline, aphasia typically involves brain damage that leads to trouble understanding and expressing words.
How long can you live with dementia aphasia?
Limited research suggests that individuals live for approximately seven to 12 years after developing symptoms of aphasia.
What is the most aggressive form of dementia?
Creutzfeldt-Jakob disease usually leads to a very aggressive form of dementia that progresses quickly. This is a rare type of dementia that differs from the most common types due to its quick progression.
How fast do you deteriorate with aphasia?
The rate of deterioration for people with aphasia varies greatly depending on multiple factors, but symptoms usually worsen over time.
What is the two-finger test in dementia?
In the two-finger test, the examiner performs a hand gesture in which they interlock two fingers in a specific pattern. The person being examined for dementia is usually asked to replicate this hand gesture. This can test their memory, motor skills, and ability to follow visual instructions.
What are signs that dementia is getting worse?
Signs that dementia is worsening may include the following;
- Difficulty understanding what is being said to them
- Requiring help with most everyday activities
- Having very limited speech that may not make sense
- Eating less
- Difficulty swallowing
- Lack of bladder and bowel control
- Becoming bed-bound
There are multiple types of dementia, with Alzheimer’s dementia or Alzheimer’s disease being the most common. It involves amyloid plaques and neurofibrillary tangles of the protein tau in the brain. Each type of dementia may come with different symptoms as it progresses. Brain scans may shed light on which type of dementia a person has. For example, frontotemporal dementia involves damage to the temporal lobes due to certain proteins building up.
What are the three stages of aphasia?
Aphasia tends to be different from other forms of dementia in that it doesn’t have standardized stages. However, cognitive impairment and language skills tend to progressively worsen over time.
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