Primary Progressive Aphasia

DisclaimerThis page is not here to be used as a self-diagnosis, self-identification, or promotion - it's simply here to be informational. Whether or not one self-diagnoses, self-identifies, and/or seeks a professional diagnosis is up to them.

**Primary Progressive Aphasia (PPA)** affects the ability to communicate. One may have trouble expressing their thoughts and understanding or finding words. Individuals can lose the ability to speak and write, eventually they are not able to understand written or spoken language. Most individuals with PPA maintain the ability to take care of themselves, pursue hobbies and, in some instances, remain employed.

PPA is a specific type of a more general condition called frontotemporal dementia.

## Signs & Symptoms

Symptoms begin gradually, often before the age of 65, and get worse over time. This may start as minor speech problems until someone struggles with judgement and thinking.

The initial symptoms may include:

* Slowed speech
* Word-finding hesitations
* Sentences with abnormal word order in speech or written language
* Substitution of words, i.e. "table" instead of "chair"
* Mispronounced or incomprehensible words
* Difficulty understanding the meaning of words
* Difficulty following a conversation despite normal hearing
* Forgetting the names of familiar objects
* Inability to remember names of individuals, even though the individual is recognized
* New impairments in spelling

### Diagnosis

PPA is diagnosed when three criteria are met:

* There is a gradual impairment of language, not just speech
* The language problem is initially the only impairment
* The underlying cause is a neurodegenerative disease

## Types of PPA

Symptoms vary based on which part of the brain's language areas are involved. The condition has three types, each with different symptoms.

### Semantic Variant Primary Progressive Aphasia

Semantic variant primary progressive aphasia symptoms include:

* Trouble understanding spoken or written language
* Trouble understanding the meaning of words
* Not being able to name objects
* Trouble formulating sentences

### Logopenic Variant Primary Progressive Aphasia

Logopenic variant primary progressive aphasia symptoms include:

* Trouble understanding spoken language, especially longer sentences
* Pausing and hesitancy during speech while searching for words
* Not being able to repeat phrases or sentences

### Nonfluent-Agrammatic Variant Primary Progressive Aphasia

Nonfluent-agrammatic variant primary progressive aphasia symptoms include:

* Poor grammar in written and spoken language
* Trouble understanding complex sentences
* Using grammar incorrectly
* May have problems speaking

## Causes

Primary progressive aphasia is caused by a shrinking of certain areas of the brain, known as lobes. The frontal, temporal and parietal lobes are affected. The damage is mainly centered around the left side of the brain.

Atrophy, the shrinking of areas of the brain, is associated with the presence of certain proteins in the brain.

Risk factors for primary progressive aphasia include:

* Learning disabilities
* Certain gene changes; rare gene changes have been linked to primary progressive aphasia

PPA is the result of a neurodegenerative disease, such as Alzheimer's disease.

## Treatment

Unlike individuals who develop aphasia from head injury or stroke, those with PPA do not typically improve with time, but a speech therapist may be helpful. Non-verbal techniques for communication, such as gesturing or pointing to pictures, may help as well. There is no cure and no way to stop its progression. One could also take medications approved for Alzheimer's disease if that is the underlying cause of their PPA.

## References

https://www.mayoclinic.org/diseases-conditions/primary-progressive-aphasia/symptoms-causes/syc-20350499

https://www.aphasia.org/aphasia-resources/primary-progressive-aphasia/

https://memory.ucsf.edu/dementia/primary-progressive-aphasia

https://my.clevelandclinic.org/health/diseases/17387-primary-progressive-aphasia-ppa

https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/symptoms-causes.html

https://rarediseases.info.nih.gov/diseases/8541/primary-progressive-aphasia

https://www.theaftd.org/what-is-ftd/primary-progressive-aphasia/