Frontotemporal Dementia is a neurological disorder that affects the frontal and temporal lobes of the brain. These lobes control the personality, behavior, movement and language of an individual. In frontotemporal dementia, the frontal and temporal lobes shrink, which causes problems in communication, behavior, and emotions.¹ 

Primary progressive aphasia is one of the variants of frontotemporal dementia. It mainly affects an individual's ability to communicate. The condition slowly progresses over time. 

What is primary progressive aphasia?

Primary progressive aphasia is a neurological condition that gradually interferes with speech and language controlling centers of your brain. This leads to difficulty in speaking and understanding conversations. 

Primary progressive aphasia usually affects people before the age of 65. It progresses slowly and worsens over time. It occurs due to underlying neurodegenerative disorders like frontotemporal dementia and Alzheimer's disease.²

Causes of primary progressive aphasia 

Primary progressive aphasia occurs due to degeneration of areas of the brain that control language and speech. This is most commonly affects the left side of the brain. Areas of the brain mainly affected are the frontal, temporal, and parietal lobes. These areas of the brain atrophy or shrink. Genetic and environmental factors may be responsible for this condition.³

Known causes include:⁴

• The most common causes of primary progressive aphasia are neurodegenerative disorders like frontotemporal dementia and Alzheimer's disease
• Family history
• Genetic mutations
• Learning disabilities 

Types of primary progressive aphasia

People with primary progressive aphasia experience symptoms affecting speech or language depending on the part of the brain affected. Based on the symptoms produced, there are 3 types of primary progressive aphasia: 

1. Semantic type

Patients experience difficulty when naming people and objects or while remembering words or facts

2. Non-affluent type

These patients face trouble when pronouncing words

3. Logopenic type

Patients feel difficulty finding words⁴

Signs and symptoms of primary progressive aphasia

Primary progressive aphasia usually develops between 50 to 70 years of age. Most people with primary progressive aphasia find difficulty in speech and expression, though their memory seems to remain intact during the initial 2 years. In later stages, it affects thinking and judgment.

Common symptoms experienced are:

• Pausing while speaking
• Difficulty finding words or names of objects 
• Trouble forming sentences
• Slow speech
• Grammar mistakes
• Difficulty understanding written language
• Difficulty understanding speech
• Complete loss of language skills^2,4

Symptoms experienced vary in different types of primary progressive aphasia.

Semantic type

• Difficulty understanding speech or written language, especially single words
• Inability to name objects
• Difficulty understanding the meaning of words
• Difficulty forming sentences

Logopenic type

• Difficulty understanding speech, especially long sentences
• Inability to repeat sentences and phrases
• Inability to find words and pausing during speech

Nonfluent agrammatic type

• Difficulty understanding complex sentences
• Incorrect grammar in speech and written language
• Errors in speech sounds called aphasia
• Problems while speaking²

Diagnosis

Diagnosis involves:

1. Examination of the medical history of the patient
2. Examination of the family history of the patient
3. Clinical presentation of symptoms 

Speech and language symptoms are evaluated. Diagnosis is made when the patient has a progressive loss of speech and communication due to neurodegenerative diseases affecting the brain. Speech-language pathologists play an important role in the evaluation of symptoms of primary progressive aphasia.⁵ 

4. Neurological exam 

This includes a speech-language evaluation and neuropsychological evaluation. These tests measure language comprehension and speech skills, and the ability to recognize and name objects and recall facts.

5. Blood tests

Blood is tested for infections and disease conditions. Genetic changes are also determined.

6. Cognitive tests and brain scans like CT scan, MRI, PET imaging

MRI detects the shrinking of specific areas in the brain and also detects strokes or tumors that affect the functioning of the brain. PET scan also records the brain function.⁶

Treatment

There is no permanent cure for primary progressive aphasia. However, certain therapies can help manage the condition and improve communication.

These include:

1. Speech and language therapy

Speech and language pathologists mainly focus on improving lost language skills. They help in the management of primary progressive aphasia and also prevent the condition from worsening. 

2. Physical and occupational therapy

Physical and occupational therapists help to manage the symptoms that affect movement and balance. 

3. Cognitive therapy

The patient's thinking and behavioral skills can be maintained through cognitive therapy. 

4. Sign language and other ways of communication

5. Medications for anxiety and depression 

These medicines are known as Selective Serotonin Reuptake Inhibitors

6. Medication for the treatment of Alzheimer's disease ⁴

Risk factors 

1. Learning disabilities

Learning disabilities during childhood, like dyslexia, serve as a risk factor for primary progressive aphasia.

2. Gene mutations

Genetic changes and a family history of primary progressive aphasia can predispose one to the condition.²

How can we prevent primary progressive aphasia?

Some changes in your lifestyle help to lower the risk of dementia. These include:

• Eating a balanced diet that is healthy and nutritious
• Maintaining ideal weight for your body and age
• Regular exercises to increase cardiac activity and cardiac strength 
• Reduced alcohol intake
• Quitting smoking
• Maintaining good cardiac health
• Avoid damage to your head by taking precautions, especially while driving⁴

Summary

Primary progressive aphasia is a neurological disorder that mainly damages speech and language-controlling regions of your brain. People aged between 50 and 70 are most commonly affected. The condition progresses over time. It occurs due to environmental or genetic factors. An underlying neurodegenerative disorder is often responsible for primary progressive aphasia. Although there is no permanent cure for this neurological condition, speech and language therapies, along with physical and occupational therapists, help to maintain the language and communication skills for as long as possible. Consult your healthcare provider and seek timely treatment if you experience any related symptoms.

FAQs

What happens when you have primary progressive aphasia?

People with primary progressive aphasia experience an inability to communicate and a loss of language skills over time. They eventually require support in their day-to-day activities.

How do you diagnose primary progressive aphasia?

Primary progressive aphasia is diagnosed when you experience progressive loss of speech and communication skills due to underlying neurodegenerative disorders. Speech-language pathologists and CT and MRI scanning play important roles in the diagnosis of primary progressive aphasia.

Is primary progressive aphasia completely curable?

There is no permanent cure for primary progressive aphasia. However, some treatments help to slow down the progression of the disease. These mainly include speech, language, occupational and physical therapies.