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Primary Progressive Aphasia (PPA): Causes - Symptoms- Diagnosis -Treatment

 What is primary progressive aphasia (PPA)?

Primary Progressive brain disease (PPA) could be a medicine syndrome during which language capabilities become slowly and more and more impaired. Unlike alternative types of brain disease that result from stroke or brain injury, PPA is caused by neurodegenerative diseases, like Alzheimer’s illness or Frontotemporal body part Degeneration. PPA results from deterioration of brain tissue vital for speech and language. Though the primary symptoms are issues with speech and language, alternative issues related to the underlying illness, like cognitive state, usually occur later.


PPA unremarkably begins as a refined disorder of language, attending to an almost total inability to talk, in its most severe stage. The kind or pattern of the language deficit could take issue from patient to patient. The initial language disturbance is also sensory aphasia (i.e., the person could have a traditional or maybe inflated rate of word production) or non-fluent brain disease (speech becomes exhausting and also the person produces fewer words). A less common selection begins with impaired word-finding and progressive deterioration of naming and comprehension, with comparatively preserved articulation.

What is primary progressive aphasia (PPA)?
 progressive aphasia (PPA)


As with brain disease that results from stroke or brain trauma, the manifestations of PPA depend upon what elements of the left brain are comparatively additional broken at any given purpose within the sickness. The person could or might not have issues understanding speech. Eventually, the majority of patients become mute and unable to know spoken or communication, notwithstanding their behavior looks otherwise traditional.


Signs and symptoms of alternative clinical syndromes aren't found through tests accustomed to confirm the presence of alternative conditions. PPA isn't Alzheimer’s illness. The majority with PPA maintain the ability to require care of themselves, pursue hobbies, and, in some instances, stay used.

  1. Brain

  2. Cerebral hemispheres

  3. Diencephalon or interbrain

  4. Thalamus

  5. Hypothalamus

  6. Midbrain

  7. Cerebellum

medical terms

  • Primary progressive aphasia (PPA) is a neurological disorder that affects a person’s ability to communicate. It is a form of dementia that specifically affects language-based functions in the brain, such as speaking, reading, and writing. It begins with mild symptoms like difficulty finding words and remembering names, but can eventually lead to significant impairments in comprehension, conversation and communication with others. This disorder is a progressive condition, meaning it gradually worsens over time.

  • Primary progressive aphasia (PPA) is a rare, debilitating neurological disorder which affects the ability of an individual to communicate verbally or through writing. It is caused by degeneration of the language centers of the brain, most commonly the left hemisphere. Over time, the patient gradually loses their ability to speak, read and write, and eventually other cognitive functions such as memory and reasoning are affected. As the disease progresses, patients become unable to carry out even daily activities such as making a phone call or preparing meals.

Symptoms Primary Progressive Aphasia

Early-stage symptoms include:

  • Slowing down, pausing, or stopping of speech

  • Word-finding difficulty

  • Written or spoken sentences with abnormal word order

  • Substitution of words

  • Mispronouncing words

  • Talking around a word

  • Using abnormally short phrases

  • Trouble understanding conversation

  • Trouble understanding simple words

  • Difficulty writing

  • Difficulty reading

  • Problems with spelling

  • Problems with arithmetic

  • Inability to remember familiar objects or how they are used

  • Mistakenly writing words that sound alike

  • Substituting a similar letter sound – for example, “t” for a “d”

  • Substituting a non-word that sounds similar to a real word – for example, telephone for telephone

Primary progressive encephalopathy symptoms vary, counting on that portion of the brain's language area units are concerned. The condition has 3 varieties that cause completely different symptoms.

Semantic variant primary progressive aphasia

Signs and symptoms include:

  • Difficulty comprehending spoken or written language, particularly single words

  • Trouble comprehending word meanings

  • Struggling to name objects

Logopenic variant primary progressive aphasia

Signs and symptoms include:

  • Difficulty retrieving words and word substitutions

  • Frequently pausing in speech while searching for words

  • Difficulty repeating phrases or sentences

Nonfluent-agrammatic variant primary progressive aphasia

Signs and symptoms include:

  • Poor grammar in written and spoken form

  • Trouble understanding complex sentences

  • Using grammar incorrectly

  • May be accompanied by speaking problems such as errors in speech sounds (known as apraxia of speech)

Causes Primary Progressive Aphasia

Primary progressive brain disorder is caused by a shrinking (atrophy) of sure sections (lobes) of the brain chargeable for speech and language. During this case, the frontal, temporal or membrane bone lobes, totally on the left facet of the brain, square measure affected.

Atrophy is related to the presence of abnormal proteins, and brain activity or operation in affected areas can be reduced.

Risk factors Primary Progressive Aphasia 

Risk factors for primary progressive aphasia include:

  • Learning disabilities. If you had a childhood learning disability, particularly developmental dyslexia, you might be at somewhat higher risk of primary progressive aphasia.

  • Certain gene mutations. Rare gene mutations have been linked to the disorder. If other members of your family have had primary progressive aphasia, you might be more likely to develop it.

Complications Primary Progressive Aphasia

People with primary progressive brain disorder eventually lose the flexibility to talk and write, and to grasp written and speech. Some individuals develop substantial problems forming sounds to talk (a drawback known as encephalopathy of speech), even once their ability to put in writing and comprehend don't seem to be considerably impaired.

As the unwellness progresses, different mental skills, like memory, will become impaired. Some individuals develop different neurologic symptoms like issues with movement. With these complications, the affected person eventually can facilitate every day care.

People with primary progressive {aphasia|brain disorder|encephalopathy|brain unwellness} also can develop depression or activity or social issues because the disease progresses. different issues would possibly embrace dull emotions like unconcern, poor judgment or inappropriate social behavior.

Is primary progressive aphasia a form of dementia?

An estimated 5-10% of people with dementia have primary progressive aphasia also known as PPA It is a rare form of frontotemporal dementia that causes changes in language ability such as loss of grammar or the ability to communicate thoughts and ideas Primary progressive aphasia usually begins in middle age but can begin at any age after 60. People with this condition can understand what others are saying but have difficulty expressing themselves through speaking or writing. In most cases speech becomes slurred and repetitive; eventually one stops speaking altogether. Writing gradually develops incorrect sentence structure and vocabulary errors Signs may include trouble finding.

Is primary progressive aphasia fluent?

Primary progressive aphasia is the result of damage to your brain's language-processing centers As such it makes it difficult for you to understand and express yourself using language Like all forms of dementia this condition progresses over time The symptoms are different for each individual but most people with primary progressive aphasia can initially continue to work and maintain social relationships Communication becomes increasingly more difficult as the disease progresses and eventually becomes completely impossible.

How common is primary progressive aphasia?

Primary progressive aphasia is the most common form of dementia affecting about 5 percent of people diagnosed with Alzheimer's disease It occurs in 10 percent to 20 percent of those diagnosed with frontal lobe syndrome one type of frontotemporal degeneration Another 15 to 25 percent are diagnosed with another type called semantic dementia Also referred to as "word-finding" problems these symptoms greatly affect language skills and become gradually worse over time.

How does primary progressive MS start?

With primary progressive MS (PPMS) the disease begins with a few isolated symptoms and slowly worsens The onset of PPMS is usually much later in life when compared to relapsing-remitting MS (RRMS) which typically develops earlier in adulthood It’s not clear exactly what causes PPMS but it is believed that genetics plays a role; in many people with the disease family members have had MS as well.

Diagnosis Primary Progressive Aphasia

To diagnose primary progressive encephalopathy, your doctor can review your symptoms and order tests.

Worsening communication problems while not vital changes in thinking and behavior for a year or 2 may be a hallmark of primary progressive encephalopathy.

Neurological examination

Doctors would possibly conduct a medical specialty examination, a speech-language analysis and a psychological science analysis. Tests can test your speech, language comprehension and skills, recognition and naming of objects, recall, and alternative factors.

  1. Mental And Psychological Examination

  • Blood tests

Doctors would possibly order blood tests to see for infections or explore alternative medical conditions. Genetic tests will confirm if you have got genetic mutations related to primary progressive encephalopathy or alternative medicine conditions.

  1. Blood analysis

  2. Blood count

  3. Blood typing

  • Brain scans

Magnetic resonance imaging (MRI) scans will facilitate diagnose primary progressive encephalopathy, sight shrinking of sure spaces of the brain and show that area of the brain could be affected. Tomography scans may sight strokes, tumors or alternative conditions that have an effect on brain performance.

  1. X-ray

  2. (computed tomography) scans(CT)

  3. magnetic resonance imaging (MRI)

A single-photon emission computed tomography (SPECT) scan, that creates a 3D image of your brain, or a antielectron emission pictorial representation (PET) scan, that shows brain performance, will uncover blood flow or aldohexose metabolism abnormalities.

  1. Cerebral angiography

  2. Brain scanning

  3. Echoencephalography

  4. Magnetoencephalography

  5. Pneumoencephalography

Treatment Primary Progressive Aphasia

Primary progressive encephalopathy cannot be cured, and there aren't any medications to treat it. However, some therapies may facilitate, improve or maintain your ability to speak and manage your condition.

  1. Rehabilitation of The Brain and Nerves
  2. Child medical and psychological care
  3. Rehabilitation of the tongue and pronunciation : Oral muscle rehabilitation

Speech and language therapy

Working with a speech-language diagnostician, focusing totally on ways that to form up for lost language skills, will be useful. Though speech and language medical aid cannot stop the progression of the condition, it will assist you manage your condition and should slow the progression of some symptoms.

Coping and support

Losing the ability to communicate can be stressful and frustrating, both for the person with primary progressive aphasia and friends and family. If you're a caregiver of someone with primary progressive aphasia, taking these steps can help everyone cope:

  • Learn all you can about the condition.

  • Have the person with the condition carry an identification card and other materials that can help explain the syndrome to others.

  • Give the person time to talk.

  • Speak slowly in simple, adult sentences and listen carefully.

  • Take care of your personal needs — get enough rest and make time for social activities.

Family members eventually might have to think about long-run care choices for the person with primary progressive encephalopathy. They will additionally ought to set up the person's finances and facilitate building legal choices to arrange for more-serious stages of the condition.

Support teams could also be accessible for caregivers and other people with primary progressive encephalopathy or connected conditions. raise your welfare worker or different members of your treatment team regarding community resources or support teams.

Preparing for your appointment

If you are experiencing symptoms, you would possibly begin by seeing your medical care supplier. He or she might refer you to a doctor trained in brain and system conditions (neurologist) or a speech-language diagnostician.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

  • Your symptoms, including any that seem unrelated to the reason for your appointment

  • Key personal information, including major stresses, recent life changes and family medical history

  • All medications, vitamins and supplements you take, including doses

  • Questions to ask your doctor

Take a family member or friend along, if possible, to help with communication and to help you remember the information you receive.

For primary progressive aphasia, some questions to ask your doctor include:

  • What's likely causing my symptoms?

  • Other than the most likely cause, what are other possible causes?

  • What tests do I need?

  • Is my condition likely temporary or chronic?

  • What's the best course of action?

  • What are the alternatives to the primary approach you're suggesting?

  • I have other health conditions. How can I best manage them together?

  • Are there restrictions I need to follow?

  • Should I see a specialist?

  • Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

What happens during your appointment will vary depending on the type of doctor you see. Your doctor may ask:

  • When did your symptoms begin?

  • Have your symptoms been continuous or occasional?

  • How severe are your symptoms?

  • What, if anything, seems to improve your symptoms?

  • What, if anything, appears to worsen your symptoms?

General summary

  1. Primary progressive aphasia (PPA) is a neurological disorder that typically affects older adults. It is a form of dementia that impacts the language areas of the brain, resulting in difficulty understanding and expressing language. People with PPA have a gradual onset of subtle language deficits, often beginning with difficulty with word-finding and naming. As the condition progresses, individuals have increasingly more difficulties with speaking, understanding, reading, and writing.

  2. Primary progressive aphasia may progress quickly and steadily but it can progress slowly as well If the disorder progresses quickly symptoms can become severe within two to four years of onset However about two-thirds of patients have a more gradual decline where individuals experience problems with language for several years before difficulties in other cognitive functions appear.

Primary Progressive Aphasia (PPA): Causes - Symptoms- Diagnosis -Treatment

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