Primary progressive aphasia

Overview  

Primary progressive aphasia is a rare neurological syndrome that impacts an individual’s communication abilities. It is categorized as a type of frontotemporal dementia, a group of disorders caused by the degeneration of the frontal or temporal lobes of the brain, which are responsible for speech and language functions. Symptoms of primary progressive aphasia develop gradually, usually before the age of 65, and worsen over time. People affected by this condition may gradually lose their ability to speak and write, and eventually struggle to comprehend both written and spoken language. 

Despite the progression of the disease, individuals with primary progressive aphasia can often maintain their independence and engage in daily activities for several years. However, the condition’s slow advancement leads to challenges in communication and language processing, impacting their quality of life.

Primary progressive aphasia has three subtypes, which include: 

• Logopenic Progressive Aphasia: This subtype causes trouble finding the right words and understanding others’ speech. 
• Progressive Non–Fluent Aphasia: People with this subtype experience difficulty using proper grammar and speaking fluently. 
• Semantic Dementia: This subtype leads to problems in naming objects and understanding the meanings of standalone words. 

Symptoms  

The symptoms of primary progressive aphasia differ depending on the specific language areas affected in the brain. This condition is classified into three types, and each type leads to different symptoms. Over time, these symptoms develop gradually and worsen progressively. 

Semantic variant primary progressive aphasia is characterized by the following symptoms: 

• Difficulty understanding spoken or written language, especially individual words. 
• Impaired comprehension of the meanings of words. 
• Inability to name objects. 
• Difficulty in formulating coherent sentences. 

Logopenic variant primary progressive aphasia presents with the following symptoms: 

• Difficulty understanding spoken language, especially long sentences. 
• Pausing and hesitancy during speech, often while searching for words. 
• Inability to repeat phrases or sentences accurately. 

Nonfluent–agrammatic variant primary progressive aphasia is characterized by the following symptoms: 

• Impaired grammar in both written and spoken language. 
• Difficulty comprehending complex sentences. 
• Incorrect use of grammar in communication. 
• Speech difficulties, including errors in speech sounds, known as apraxia of speech. 

If you are concerned about your communication abilities, consult your healthcare provider. If you notice symptoms of primary progressive aphasia in a family member or friend, discuss your concerns with them and offer to accompany them to see a healthcare provider. In the case of sudden changes in speech or communication, call emergency services immediately.  

Causes 

Primary progressive aphasia (PPA) is a condition where certain parts of the brain responsible for speech and language shrink, called atrophy. This atrophy mainly occurs on the left side of the brain. While healthcare providers usually can’t pinpoint a specific risk factor, it is believed that a mix of genetic and environmental factors could lead to PPA. Even if there is no gene mutation, PPA can still have a familial tendency. 

Risk factors 

The risk factors for primary progressive aphasia are as follows: 

• Learning disabilities: Individuals who experienced childhood learning disabilities, such as dyslexia, may face a somewhat higher risk of developing primary progressive aphasia.
• Certain gene changes: Primary progressive aphasia has been associated with rare gene mutations. If other family members have been affected by this condition, there might be an increased likelihood of developing it.