Forgetting a word is something that happens to everyone at some point. However, if you're in the later decades of your life, this might be a sign of primary progressive aphasia (PPA). This is a neurological condition that affects your language abilities. But how do you know if you have it?

PPA is diagnosed by evaluating specific speech and language symptoms, through imaging scans, and reviewing family history. There isn’t a specific cure for this condition, but there are some treatments available that can improve the quality of life for those affected, including speech therapy and medications to alleviate certain symptoms.^1,2

This article aims to explain the diagnosis and treatment methods for primary progressive aphasia. If you’re interested in learning more, keep reading!

Understanding primary progressive aphasia

Primary progressive aphasia is a neurodegenerative disorder that affects your ability to find and/or remember words and use and understand speech. This is a rare type of dementia that worsens your language skills over time and it usually appears during your 50s or your 60s.^1,2 

The impairment of your communicative skills happens as a result of deterioration in the frontal and temporal areas on the left side of the brain. This decline goes on for approximately two years before other cognitive impairments appear.²

There are 3 variants of PPA, each with their symptoms and causes:^2,3

• Nonfluent/Agrammatic PPA (nfaPPA): Characterised by struggling with speaking fluently, producing sounds, and using complex grammar
• Semantic Variant (svPPA): Involves progressively forgetting or losing the meaning of words
• Logopenic Variant (lvPPA): Exhibits difficulty finding words while speaking

Diagnosing PPA

Getting an accurate and early diagnosis of PPA is important to receive the appropriate help. 

Clinical assessment

Your general practitioner (GP) or neurologist will conduct a clinical assessment, looking to confirm specific signs related to your language production, like grammatical mistakes, mispronounced sounds, frequent pauses while speaking, and difficulty understanding speech or words.^2,3 They will also analyse your previous or regular verbal skills, along with factors such as your education, bilingualism, occupation, or any learning disabilities. Additionally, your health provider will also evaluate your medical and family history to see if you have an increased risk of PPA.

Speech-language and neuropsychological assessment

Speech-language specialists will evaluate you with different tests to determine specific impairments. They will assess several language domains like semantic, phonological, and syntactic processing, written language, and motor speech. The assessments for this include:²

• Repetition: Repeat words or sentences
• Word and sentence understanding: Tasks like following commands, matching words to pictures, etc
• Spontaneous speech: Answer open-ended questions
• Confrontation naming: Seeing an image of an object and labelling it
• Semantic processing: Match semantically related words 
• Motor speech: Oral diadochokinesis (the ability to rapidly repeat sequences of syllabus), producing single syllables, multisyllabic words, and sentences
• Reading
• Spelling 

Your health professional might also suggest a neuropsychological evaluation to assess your memory, capacity for learning, arithmetic calculation, executive function, and visuospatial ability (how you mentally represent, visualise and manipulate objects).²

Imaging scans

Conducting brain imaging, like MRI scans, can confirm the PPA diagnosis and rule out other conditions with similar symptoms. In the case of this disorder, the scan will show atrophy in the left side of your brain, particularly a thinning in the cortical region with wider sulci.² 

Biomarkers

Brain atrophy or shrinkage is associated with certain proteins which are responsible for reducing the activity of the affected brain areas. For instance, the presence of the biomarker beta-amyloid can be an indicator of lvPPA. Another protein, tau, is associated with the symptoms in nfvPPA as well as lvPPA.² 

Genetic studies

Your health provider might use genetic studies as an additional test for a differential diagnosis. Certain genes could be a risk factor or indicative of primary progressive aphasia. This is often used when the other diagnostic tools are inconclusive. Your GP will collect your DNA from your saliva or blood, and laboratory results will provide the necessary information after some time.^2,3 

Treatment approaches for PPA

While there isn’t a specific treatment that cures primary progressive aphasia, there are some therapies that can improve your quality of life. Treatment for this condition should involve a multidisciplinary approach, which focuses on helping you regain speech capabilities, informing you about PPA, helping you maintain autonomy, and ensuring you can engage in social and work activities.

Speech therapy

Speech therapy might help you maintain your language abilities for as long as possible when you have PPA. Word retrieval interventions--where you are given an image and you use reading and repetition of the word–appear to have a positive outcome in patients with PPA. However, this requires constant practice. Other techniques involve improving fluency with activities like repeating or rehearsing a script, or practising daily conversation with the help of signs or interpretations.⁴

Activities where you practise spelling and repeat words verbally also appear to improve both written and spoken construction.¹ 

Cognitive interventions 

Transcranial direct current stimulation (tDCS) is an intervention that uses small electrical signals to stimulate the areas of your brain that affect your speech. tDCS can improve the functioning of these regions, resulting in improved speech performance that can last up to 5 hours.¹ 

Cognitive therapy can help you deal with the negative emotions and frustration that come with losing the ability to communicate. Furthermore, this can also educate your close ones about your condition, and give them the necessary tools to support you. 

Medications

Sometimes, if the underlying cause of PPA is Alzheimer’s disease (AD), your GP might prescribe you cholinesterase inhibitors and/or memantine to treat the associated symptoms. Moreover, you may also be prescribed selective serotonin reuptake inhibitors to help manage feelings of anxiety and depression that PPA might cause, as well as to enhance brain stimulation.¹

FAQs

Does aphasia get worse over time?

Unfortunately, as it’s a progressive disease, it gets worse over time. However, early diagnosis and appropriate treatment, such as speech or language therapy, can help slow down the decline and improve quality of life. 

How quickly does primary progressive aphasia progress?

This will depend on the individual, and factors such as the timing of treatment and presence of any underlying conditions. On average, PPA progresses over a period of 3 to 15 years. 

Can I reduce my risk of primary progressive aphasia?

There are currently no known methods of preventing PPA. However, there are some lifestyle changes that you could adopt like eating a balanced, nutritious diet, rich in fruits, vegetables and whole grains, reducing your alcohol consumption, exercising regularly, and maintaining overall wellness.

How can I help my family members with PPA?

Educate yourself about the condition, and treat them with respect and dignity. Be patient with them and give them time to speak and articulate, without rushing them. Use simple and clear sentences at a slow pace. You can also have visual aids, like cards with images, to assist in communication. Finally, you can also join support groups, where you can share experiences and get advice from people in a similar situation.

Summary

Primary progressive aphasia is a neurodegenerative condition which affects your speech. It’s considered a rare type of dementia that progressively declines over time. Depending on the specific type of PPA, individuals may have difficulty understanding words and sentences, speaking fluently, using complex grammar, or finding the right words. Having an early diagnosis is crucial for finding adequate solutions to manage the condition effectively. 

Diagnosing PPA involves several methods, including clinical assessments focusing on your symptoms and family history. Brain imaging, genetic studies and biomarker tests ensure a differential diagnosis, as sometimes this could be confused with dementia or Alzheimer’s disease. 

While there is no cure for PPA, there are several therapies available that can improve quality of life. For instance, speech therapy can help maintain language skills for as long as possible, whilst cognitive therapy can help manage negative emotions and educate patients about the condition. Medications may also be prescribed to deal with certain symptoms. 

More research is needed to develop better treatments that can improve the outcomes and quality of life for people affected by PPA. As scientific advancements continue, there is hope for more effective solutions in the future.