A Diagnosis of Primary Progressive Aphasia? What to Expect…

Primary Progressive Aphasia (PPA) is caused by the gradual degeneration of brain tissue in the parts of the brain that control speech and language.

The way PPA progresses varies from person to person and from one subtype of PPA to another. Grethcen Szabo, a speech language pathologist at the Adler Aphasia Center in NY says:

Many of the individuals with PPA that we have seen have not been given a firm diagnosis and very few have information on the specific subtype.

First steps

The best way to receive a detailed evaluation and prognosis about PPA is a referral to a neurologist or neuropsychologist who has experience with PPA. Make sure you ask your neurologist to refer you to a speech-language pathologist (SLP) for speech therapy. If you can’t find a neurologist who has worked with other patients with PPA, you can make an appointment with a speech-language pathologist (SLP) who has PPA experience and who can refer you to a neurologist that is familiar with this condition.

The early stage

Initially, for a time period that can last a couple of years, a person diagnosed with PPA will exhibit a progressive loss of speech and language capabilities (aphasia). At this stage, patients may not notice any changes in other mental functions and activities of daily living.

Symptoms may include: slowed or halting speech, decreased use of language, word finding difficulty, written or spoken sentences with abnormal word order, substitution of words, mispronouncing words, talking around a word, difficulty understanding conversation, sudden difficulty understanding simple words, problems writing, problems reading, difficulty with spelling, difficulty with arithmetic.

In the earlier stages, patients with PPA and their caregivers need resources to help them manage the challenges they are experiencing. These could include both physical and emotional challenges. Local speech therapy centers or individual SLPs who are experienced with PPA can provide many of these resources.

The later stages

Due to the progressive nature of PPA, language abilities will continue to deteriorate over many years.

With time, PPA may affect other cognitive abilities such as memory, reasoning and visual perceptual skills. The rate of progression of PPA varies and it is unknown why some people progress more rapidly than others.

At later stages of PPA progression, patients may need to transition from a speech therapy center to a memory-care center or an assisted living center.

Where to find more information

We have dedicated resources that provide further information about understanding, diagnosing, and managing PPA. For any questions please email us at answers@aphasia.org

Image: rawpixel via Unsplash

Comments

33 Comments

  • Audrey Rowe
    October 24, 2018 at 8:21 am

    Thank you for adding more resources and information on PPA. My husband had a recent diagnosis and we are struggling to understand and manage. We live in a rural area and can’t find resources or support groups nearby. If anyone knows of resources near West Virginia please share.

  • Daniel Silver
    October 24, 2018 at 9:12 am

    My wife, Ann suffered form PPA. Over a five year period she gradually lost. It only speech but executive function. At the end she could no longer reason. Yet she had memory. She could get up in the morning, shower,!blow dry her hair, lay out her clothes for the day and make a light breakfast.
    Yet she could behave like a three year old child. Could not understand the need to go to a doctor’s appointment and would kick and fight to prevent leaving. It was a steady decline with no respite. She seemed to lose the ability to swollow and stopped eating or drinking and passed away. Very sad.

  • Susan Hanson
    October 24, 2018 at 11:29 am

    My husband died of Semantic PPA in Dec. 2116, 8 years after his illness was diagnosed. The last two months he deterioted very fast as far as level of anxiety and non compliant with taking medications and eating. Up to the last few weeks, I involved in daily activities like jigsaw puzzles and a card game and word finds.
    I am pleased you are getting more information available about PPA for those currently suffering with it and for their care givers.’

  • Amanda Piburn
    October 24, 2018 at 7:49 pm

    There are so few people in our area with PPA. I would love to join an email group of caregivers and patients. Is that available? My husband was diagnosed a year ago.

  • Beth
    October 24, 2018 at 10:05 pm

    My mom was diagnosed with PPA (logopenic variant) in 2016. She had been exhibiting symptoms for at least a year before that. She did receive speech therapy for about a year through CalState Northridge. Thank you for the resources.

  • Kathy
    October 26, 2018 at 9:47 am

    Thank you for the continued information and updates. I want to address a couple of the comments from people living in rural areas and not finding a support group as I was in that situation. I found a teleconference support group in my home state through the AFTD association. Although my husband has not staged into AFTD just the ability to talk to others going through a similar experience has been helpful. The group is limited to people living in my state…apparently since we have other commonalities. So look at other associations, your local Office for Aging, Alzheimer’s Association or perhaps Aphasia.org has some groups they can recommend.

  • Laura Marcinkowski
    October 26, 2018 at 11:02 am

    I have PPA

  • Anonymous
    October 26, 2018 at 11:03 am

    I have PPA

  • Laura Marcinkowski
    October 26, 2018 at 11:24 am

    I have PPA. I am in the early stages (honeymoon period) and I read Kimberly Williams-Paisley book on dementia and I must say that I would not want to be her mother. .I was diagnosed by study I belong to in Houston, Texas by Houston-Methodist Hospital.
    .

  • Donald Platt
    October 27, 2018 at 7:29 pm

    I believe I have PPA (follow up w/Dr.11/2/18 ;& IHave an apt. @U.MI. Dec. 14,2018). What I would like to know? Do you know of good support group in MI.I’m thinking of moving to the Kalamazoo area this spring (2019) ; is this a good idea? You don’t paint a good picture in your comments page.

  • Ben McPherson
    October 29, 2018 at 3:35 pm

    My beautiful and loving wife passed away in February, 2018 in our 53rd year of marriage. Through her decline, she was the same sweet and kind person I met in 1960. Her personality and behavior never changed. We excluded AZ with an Amyvid PET scan which I recommend having done. Marie gradually lost interest in reading and her speech, but kept a beautiful smile until the end.

  • Ben McPherson
    October 31, 2018 at 3:37 pm

    Laura Marcinkowski:
    My wife was diagnosed with PPA by Dr. Paul Schulz, head of neurodegenerative studies at UT Med in Houston. He gave Marie a PET scan using florbetapir, a nucleotide that diagnoses ALZ by showing amyloid plaques. Marie was clear and PPA was his diagnosis. Marie died in February 2018 and never showed any changes in personality or behavior. Best of luck.

  • Pam Upchurch
    December 26, 2018 at 2:04 am

    My 58 yr old husband was diagnosed with PPA 14 months ago after a 2 1/2 yrs of trying to get a diagnosis of what was happening to him. He’d been having symptoms for 5 years. I would love to be able to communicate with someone about what to expect because there’s very little info out there about this.

  • Elaine Stueven
    January 1, 2019 at 9:43 pm

    My daughter was recently diagnosed with PPA after seeing many doctors in a 3 year period and gradually losing her ability to express herself more and more with words, mainly nouns and verbs, and also not reading much due to either lack of comprehension or retention of what she was reading. It also affected her writing ability. I noticed we lost her in large gatherings where so many people talking at once. She got so frustrated and cried often when trying to communicate because she graduated with a degree in “Communicative Disorders” and knew something was terribly wrong. Mayo Clinic in MN finally diagnosed her with PPA and is going back in January for 2 different Pet Scans to confirm it and to do other tests. She cried upon the diagnosis, but has now accepted it better than me.
    This is so heartbreaking for me as a Mom and still pray for a miracle. I am 82 and was starting to depend on her more and more emotionally and for decisions I need to make as I live alone. I wish we would have known sooner as it’s become harder to talk about serious issues this last year and now I can’t burden her with anything deep even if she did understand.
    I too wish there would be a place caregivers and family’s could share and exchange dialect on this disease.

  • Tonya Gleeson
    February 4, 2019 at 7:29 pm

    Ben McPherson:

    Can you tell me how long it was from your wife’s diagnosis until her death? (which I’m sorry to hear about) . I am in beginning stages and have not been officially diagnosed however I am 26 year nurse that finally figured out my own symptoms. My husband and I are trying to plan as we have younger children. My only symptom so far has been difficulty with word find. I did have some frontal cerebral atrophy on a recent head CT performed for a headache. I am still working so that is why my husband and I are trying to plan. Im sorry to ask questions but this is a devastating diagnosis especially since I have young children. Any information would be appreciated. Thank you.

  • Anonymous
    August 6, 2019 at 1:28 pm

    PPA
    Will start to proceed for disabilities benefits

    Any thoughts?

  • Tecla Legge
    August 8, 2019 at 6:49 pm

    Thanks to all of you sharing your experiences and your questions about how to proceed. My 79 year old husband has been very generally diagnosed as having “dementia”, but from what I and our children perceive, it is most definitely and prominently aphasia, of several years progression. I wonder about seeking a true diagnosis from his neurologist, whether it is important to get that nailed down? I perceive from the medical people little interest if looking at treatment, no matter what the diagnosis is, beyond a simple “dementia”. A visit with a social worker provided some very good communication on feelings, and simple plans on making life easier (weekly calendar, plan short trips, replace our beloved dog).

    I had begged for and finally gotten him some speech therapy, but I don’t know what to ask from the therapists, and my husband does not find the advice compelling enough to follow-up (computer word games?). However, one helpful therapy exercise was writing out a script of questions for my husband to follow when having long-distance phone conversations with our adult children.

    For now, we can still enjoy watching television, movies, some reading, and gardening together. The stress of going through airports has grown to where I just canceled a long planned trip, which gives me a selfish sorrow, but to my husband is a great relief.

  • Anonymous
    August 18, 2019 at 5:26 pm

    There is so little information available and very few support groups. This is such a devastating, frustrating and confusing disease process. My husband, Mike, is 75 years old and was diagnosed about

  • Breta
    August 18, 2019 at 5:49 pm

    My husband was formally diagnosed with logopenic PPA about ten years ago. After seeing a neurologist for about 3 years he saw no reason to continue. She told him there was no cure and nothing she could do to stop the progression. Over this past year he has gotten much worse. He complains of getting worse frequently. Over this past year he has become very forgetful as well. There is so little information available. This disease process is confusing, devastating and extremely frustrating especially for the patient and their primary caregiver/s. Many times family members and friends are little to no help. It’s difficult to understand much less explain what is happening or why to your loved one. There is no support group where we live. I find there is very little information to help you navigate this disease process. I am very interested in any information I can find to help me help my husband and myself work our way through this process.

  • Margaret
    August 19, 2019 at 2:09 pm

    To Anonymous who posted 8/6/19 – My husband was diagnosed this past May. We filed for disability as soon as we were able (end of June) because I have heard that it can take a long time. I was told the average is 3-6 months. Many times people are denied the first time. My husband’s speech therapist just told us of one of her patients (she did not say what her medical condition is… and I of course didn’t ask) but that it took her 2 years for her disability to be approved. That was discouraging to hear. Especially since my husband had been out of work for roughly 2 years. We did contact an attorney. Thankfully they are on a contingency basis. There is a LOT of paperwork that needs filled out. Not going to lie, it can be overwhelming. The last time I checked the progress of the claim (you can do that on-line) it indicates they are looking into his medical records. I wish you all the best. The “not knowing what’s next” is so difficult.

  • Renee
    September 19, 2019 at 4:07 pm

    Regarding the disability benefits application, start now. Even though PPA is one of the compassionate allowance conditions for social security disability (supposed to be quicker disability approval), it still took us almost three years to get my husband approved for disability. My husband is 56 and started having signs at 52, but because we had just lost our son in an accident, we really thought it was related to that. He was finally diagnosed with PPA in 2016. It is so hard watching someone that was always so outgoing and independent progress to barely being able to speak and having trouble with simple tasks. He seems to find very little joy in life. I worry about needing outside help in the near future and the hefty costs associated with that.

  • Sue
    October 11, 2019 at 9:25 pm

    It took several years to finally get a diagnosis of PPA. Went to neurologist here in California, terrible, arrogant man. Finally went to Phoenix and the Mayo Hosp. Caring, thorough and they gave us all we could use. We have been married 8 years and his condition started failing about 1-2 years after our marriage. He no longer can speak, and his hearing is another issue. Because he cannot shut all background noise out when I speak or he is spoken to, he becomes anxious and can get very angry. Speech pathologist suggested using a Boogie Board, tablet like item that can be purchased on Amazon. I will ask him questions and he will write answers, but that is becoming more difficult, answers not clear and sometimes he will answer no when he means yes. We have to be patient. Swallowing liquids becoming very scary, he has difficulty swallowing. Foods are not a problem. He takes small bites and chews thoroughly. Sleeping more and gets depressed very easily. On antidepressant and using CBD oil for anxiety. And this is what has happened in the last six years. Wonderful, loving intelligent man. Grandfather and great-grandfather who is greatly loved.

  • Anonymous
    October 28, 2019 at 7:00 pm

    My husband has PPA too. The most frustrating thing has been the lack of information for PPA patients and their caregivers. His symptoms are not like Alheizemers or dementia. I don’t know what he can or cannot understand. So frustrating!

  • Al Sladek
    November 16, 2019 at 11:35 pm

    Hello, My name is Al. I am 77 years old. I live in a retirement home with a memory care unit. I do not need that kind of care now but am very concerned about the future, I started to have noticeable word finding problems in my middle forties. The problem was not enough to be of concern. My medical providers considered my memory problems to be senior moments. I would reflect on the previous year’s memory every year into my seventies. I would see no difference over the previous year, but I could think back 5 years and realize a change in frequency of memory lapses. The change seemed manageable and medical opinion was normal aging. The progression was slow and steady for 25 years. However the last couple of years the change has been noticeable one year to the next. Previously this change was noticeable over 5 year periods. I have seen neurologists at the VA hospital in Los Angeles in the last ten years and have had 2 brain MRIs and 2 PET scans. The latest PET scan found evidence of amyloid plaques in the anterior area of the left parietal lobe. Evidence in the white matter only. No evidence in the gray matter. I only have problem with word finding. When I am trying to express myself in a conversation a word I need I just cannot remember. The word may come to me 10 seconds, 10 minutes or a week later. I have never had a word that I did not remember at some time in the future. I do not have speech problems other than forgetting words. I do not have problems understanding others speech. I know the meaning of the words I am trying to remember but just cannot bring the memory of them into my brain. The best definition I have seen is anomic aphasia. I would like to contact anyone with a similar problem or any information relative to this kind of memory problem. Any websites or research in this area would be particularly appreciated.
    Al

  • Abir
    November 27, 2019 at 8:09 pm

    Al Sladek
    I have the same exact problem. I’m 54 years old and this problem started 3 years ago and it’s becoming worse. More words and names I can’t remember. I just started researching this a couple of weeks ago when things became too obvious to ignore or attribute to aging only. I will be seeing my PCP and I guess that will be the beginning of a long road.

  • James Childress
    December 27, 2019 at 7:54 pm

    12/27/19
    My wonderful wife of 20 years was diagnosed with PPA in Nov/14. Over the past five years each year her memory has declined to the point she now remembers very little. She has only a few words she uses now for almost any types of communication about any subject. Her eating habits have changed dramatically. Where before she ate only healthy foods, she now eats cookies, pies, cakes. My wife was always very concerned about what she wore, now she wears the same clothes over and over. She would shower almost every day, now I must remind her. My point is not only does she have very few words she uses, but her memory and personality have also changed dramatically. Our children and I are very concerned about what comes next? Are there reasons she can’t remain at home in the future? We weren’t blessed with a lot of money and see no way to afford care facilities, but have just enough money not to qualify for Medicaid. Has anyone faced those same obstacles.

  • Tom D
    December 30, 2019 at 1:53 pm

    Following a recent PET scan, my 63 year old wife was diagnosed with PPA about 10 days ago. I’m reading everything I can to understand the illness and prepare for whatever comes our way. Still sorting out first/next steps. Probably going to pursue a consult/second opinion with another doc… not because we doubt the first doc, just for more insight and to be absolutely as certain as we can be.

  • Bonnie Czekanski
    January 21, 2020 at 8:00 pm

    Does anyone have any recommendations for neurologist in Sarasota, FL area with experience w/ PPA diagnosis for my husband?

    We know of UF Health/Gainsville & Mayo/Jacksonville, but both are too far away & not practical for the evaluation & testing required for PPA diagnosis.

    Have been in touch w/ Mesculam CtrNorthwestern study in Chicago, as this is our goal, but they cannot consider his acceptance until we have PPA diagnosis.

    Very grateful for any suggestions.
    Bonnie Czekanski
    Email: hoffman_b@prodigy.net

  • Karen Louis
    January 29, 2020 at 10:31 am

    My 59 yr old husband was diagnosed with CTE 3 yrs ago and then his DR changed it to PPA 2 yrs ago. I agree with everyone, there are very few resources. His 2 Neurologists just offered antidepressants, anti anxiety and sleeping medications. But nothing else.(it does help though) We did try speech therapy, but after 6 mos, the therapist said that it really wasn’t working, so we stopped. He was approved for SSD (social security disability) so at least his not being able to work has not affected us as bad as it could, financially.
    I would love to start a support group, even just an email or phone one. I live in northern VA. Please contact me if interested.
    Kglouis@verizon.net

  • scared
    January 31, 2020 at 3:03 pm

    I’m 43 years old. I have been noticing for quite a few years that I have trouble with my vocabulary and remembering words that use to be so easy to me. It’s so frustrating to me, and I can’t understand what is happening to me. Most do not take it seriously because of my age, but I know myself and when something is not right. In the last couple of years I have noticed I had rather just not talk at all, or I had rather text something than to have to say it out loud. I hate the thought of having to tell my husband a simple story about something that happened during the day, much less something traumatic that happens. Most recently I experienced a very traumatic time in my life losing my older brother after an extended time in the hospital following a terrible car wreck. While I cared for him daily alone at the hospital for a few months, and then eventually lost him because he chose to starve himself to death, from depression and the lack of resources give to him to help him cope with becoming quadriplegic, it seems that I have become much worse with my memory and daily activities. I assume some of this is due to grief, but some I feel like has been excelled by the stress of the trauma. After reading so many of these.. it seems to describe so much of my symptoms. Could this be related to FTD? My aunt was diagnosed at the age of 56 in 2008 on May 7. She passed away on August 17, 2008 with FTD. Until I read about this, I was so afraid that I had inherited the gene that she had for the FTD. I am the one that cared for her, and noticed the differences and took her to the neurologists and doctors until they diagnosed her with FTD. She was a nurse for 28 years. That was the most horrible thing to watch someone go through and decline so fast. I’m so afraid that I will not be here to see my granddaughter grow up. I know that my eating habits have changed dramatically, as well as my weight and my showering, and the way I dress and everything else about me. I know that there is something wrong with me.

  • Sherrie Le
    April 5, 2020 at 5:44 pm

    My friend was diagnosed with PPA about a year ago. Her neurologist referred her to speech therapy and to an Alzheimer’s group. The Alzheimer’s group was interesting but not real helpful as her disease is quite different. She and her husband would really like to join a group to learn more about how to communicate with each other and help her as the disease progresses. They live in Minnetonka, Minnesota. Is there anything they can do to stop or slow the disease progression?

    I told her I would research this for her. She wants to talk to others who have PPA.

  • KR
    July 17, 2020 at 12:20 pm

    Is there a group in San Jose, CA area that provides support for PPA?

    Thanks!

  • Anonymous
    October 22, 2020 at 6:39 pm

    Is there a PPA support group in or around New Salem Massachusetts?

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