How to Diagnose Progressive Aphasia

Three Parts:Recognizing Signs and SymptomsGetting Diagnosed By Your DoctorTreating Progressive Aphasia

Progressive aphasia is a disorder in which people have trouble with communication, both written and spoken. It presents as a gradual decline in language and communication skills, affecting people's abilities to express their thoughts (either in written or spoken format), and to understand or "find" words. To diagnose progressive aphasia, your doctor will do a series of clinical evaluations both to rule out other conditions that may present similarly, as well as to assess the type and severity of your communication decline. Note that progressive aphasia is usually called "primary progressive aphasia," "progressive aphasia without dementia," or "PPA."

Part 1
Recognizing Signs and Symptoms

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    Watch for speech problems that get worse over time.[1] Progressive aphasia is when you lose your skills and abilities associated with language and communication over time, in a way that exceeds a natural age-related decline. Things to be on the lookout for include:[2]
    • Language abilities that become more and more impaired with time.
    • Problems with written language in addition to spoken language.
    • Difficulty "finding" words (i.e. loss of precision of vocabulary), or substituting incorrect words due to forgetfulness. This may include forgetting the names of things, and frequently "talking around" a word by describing it or referring to it without using its actual name, as well as forgetting a person's name despite recognizing the individual.
    • Stuttering and/or trouble pronouncing words (or mispronouncing words).
    • Difficulty understanding words and sentences (note that this must be due to a word comprehension problem and NOT a hearing problem), which may lead to confusion or continually asking for clarification.
    • Slowed speech, incomplete sentences, incorrect word order, grammatically incorrect sentences, and/or decreased use of language as a means to communicate.
    • Trouble with written language, such as incorrect spelling, and problems reading and understanding written information.
    • Becoming mute (this is a very late sign).
    • Note that the signs and symptoms will vary depending upon the specific area of the brain that is damaged, which varies from person to person.
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    Recognize signs of the "agrammatic/nonfluent" subtype of PPA.[3] This subtype of progressive aphasia presents primarily with speech difficulties. Speech will generally consist of fewer words, missing words, incomplete sentences, grammatically incorrect sentences, and difficulty "finding" (or remembering) the correct words to refer to things. Generally, in this subtype of PPA, comprehension of what other's are saying remains intact. It is predominantly a disorder of word production.
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    Look out for signs of the "logopenic" subtype of PPA.[4] This subtype of progressive aphasia mainly affects word finding. Speech is normally preserved, other than challenges remembering the names of objects, and difficulty maintaining the precision of vocabulary that may have been used in the past.
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    Watch for signs of the "semantic" subtype of PPA.[5] The primary problem in the semantic subtype of progressive aphasia is losing word comprehension. For instance, you may not recognize or understand the meaning of words that you previously understood. you may frequently ask for clarification, or appear confused, when trying to make sense of what others are saying.
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    Consider whether you are in the population most frequently affected by PPA.[6] PPA most commonly affects individuals between the ages of 40 and 80 years old, with the peak incidence occurring around 60 years old. It affects twice as many men as it does women. It has been hypothesized that there may be a genetic component to progressive aphasia, as it happens more commonly in individuals with a family history of cognitive decline (such as other family members who have been diagnosed with dementia or aphasia).
    • It is important to understand that, while progressive aphasia always presents initially as symptoms only related to language and communication (without other symptoms that are commonly associated with dementia, such as memory loss), PPA may progress with time to become a more widespread dementia syndrome.
    • However, the diagnosis of PPA relies on presenting during the initial few years with deficits only in language and communication skills.
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    Take note of how it started.[7] Progressive aphasia presents as a gradual decline in your language and communication abilities. It is not associated with a triggering event, and there is no definitive "time of onset" of the condition. Rather, most people notice subtle challenges with language and communication coming on gradually, with no other signs or symptoms of mental deterioration.
    • If there is a history of a triggering event, such as a stroke or a traumatic brain injury, you are likely NOT suffering from progressive aphasia.
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    Keep a diary of how your symptoms are changing (or worsening) with time. Note that one of the hallmarks of progressive aphasia is that it continues to "progress" (i.e. worsen) with time.[8] It is not something that all of a sudden begins to show improvements, and it does not have a fluctuating course (with periods of being worse followed by periods of being better).
    • The best way to keep track of, and to remember, your symptoms and their progression over time is by keeping a diary.
    • Having a written record is always more reliable than going by memory alone, and the written record will help your doctors to diagnose the condition as well.

Part 2
Getting Diagnosed By Your Doctor

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    Receive a detailed clinical exam.[9] The first step when you see a medical professional will be to receive a detailed clinical exam. Your doctor will assess your ability to read and to understand language, as well as your ability to speak and to effectively articulate your thoughts.
    • In addition to examining your language and communication abilities, your doctor will do a full mental and neurological exam to assess for any other cognitive or brain-related abnormalities.
    • Your doctor will ask you questions about how you are functioning in day-to-day life, and areas of your life that have been impacted by your condition.
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    Rule out other conditions that may present similarly.[10] The reason that your doctor will ask so many questions and perform such a detailed exam is mainly to rule out the possibility of other conditions. If other areas of your life are affected, and if you show other signs of mental deterioration, you may have another condition such as Alzheimer's disease which has more widespread manifestations than simply affecting language and communication.
    • Alzheimer's disease is the main disease that is confused diagnostically with progressive aphasia.[11]
    • The main difference between the two is that Alzheimer's disease is a widespread form of dementia that will negatively impact many areas of your life, such as memory, language and communication, knowing appropriate social behaviour, and being able to perform tasks required for day-to-day life.
    • Progressive aphasia, on the other hand, presents as a decline only in language and communication abilities (it is only this area of the brain that is damaged, which differs from a more widespread form of dementia). Therefore, declines in function will ALL be related to language and communication issues, with little to no memory problems or other concerns.
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    Have a head CT or MRI.[12] As part of the diagnostic process, you will most likely receive either a head CT scan or an MRI. This is because, in addition to ruling out other mental conditions, your doctor will want to rule out potential signs of a stroke or traumatic brain injury. This is best done with the combination of a neurological exam and imaging techniques (either CT or MRI).
    • If your CT or MRI is consistent with the possibility of progressive aphasia, and your clinical exam supports this as well (i.e. your clinical exam shows mental deteriorations associated with language and communication only, and affecting no other areas of your life), your doctor will assign you a diagnosis of progressive aphasia.
    • It is key to understand that progressive aphasia cannot officially be diagnosed (i.e. proven) until the time of autopsy (that is, following death when doctors look at your brain and evaluate the specific areas of brain damage).[13]
    • Similarly, other forms of dementia such as Alzheimer's Disease also cannot be officially diagnosed until after death, at the time of autopsy. They are all "clinical diagnoses," meaning that your doctor will evaluate the imaging results (from CT or MRI) combined with your clinical signs and symptoms and assign you the best possible diagnosis that accounts for everything, as far as he or she can tell.
    • This is also why regular follow-up exams are important, to see if any of your signs or symptoms change. For instance, if you initially presented to your doctor with only language and communication deficits, and subsequently begin experiencing significant memory loss and other cognitive problems, your diagnosis may be changed in light of the new symptoms you are experiencing.

Part 3
Treating Progressive Aphasia

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    See a speech therapist.[14] Because the impact on language and communication varies from person to person (depending upon which area of the brain has been damaged), the approach of the speech therapist will vary depending upon your specific presentation. He or she will focus on finding ways to help you to more effectively manage your condition in social situations, and to preserve as many of your communication abilities as possible.
    • Note that, while speech therapists can help you to manage your condition, they cannot cure it.
    • The actual "pathophysiology" (the damage to your brain) is not reversible and will only continue to worsen with time, as this is what the disease entails.
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    Learn new communication strategies.[15] Given that the pathophysiology of progressive aphasia is not reversible, and will only continue to progress with time, it is important to explore alternate modes of communication that can help you to cope. Your speech therapist may suggest non-verbal strategies for communication, such as gesturing or pointing, which many patients find helpful when their condition becomes more severe.
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    Connect with an Aphasia Community Group.[16] One of the key elements for people as their progressive aphasia worsens is finding a community of people who understand the condition, and who can be there as an emotional and social support network. Connecting with an Aphasia Community Group may also provide you with insights and strategies as to how to better manage your condition, and to optimize your communication and interactions with those around you, as you will be exposed to people who are faced with similar problems in day-to-day life.
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    Carry information about your diagnosis with you.[17] As their condition progresses, some people carry ID cards with them and/or papers with information about their diagnosis that they can give to others. This way, if communication is a significant challenge, it can help others to understand and hence smooth the interaction as much as possible.

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Categories: Neurological Disorders