Aphasia, primary progressive

Primary Progressive Aphasia Primary Progressive Aphasia (PPA) is a neurological syndrome characterized by a gradual decline in language capabilities. It is a type of neurodegenerative disease that primarily affects the language-dominant hemisphere of the brain, typically the left hemisphere. Unlike other forms of aphasia, which may result from a stroke or brain injury, PPA is progressive and degenerative.

Clinical Features

PPA is marked by a progressive loss of language skills, including speaking, understanding, reading, and writing. The condition is divided into three main variants:

• Nonfluent/Agrammatic Variant (nfvPPA): Characterized by effortful, halting speech and agrammatism. Patients may have difficulty forming complete sentences and may omit small words.
• Semantic Variant (svPPA): Marked by a loss of word meaning, leading to difficulties in naming objects and understanding words. Patients may speak fluently but with little meaningful content.
• Logopenic Variant (lvPPA): Characterized by difficulty finding words and constructing sentences, with frequent pauses in speech. Patients may have trouble repeating phrases or sentences.

Diagnosis

Diagnosis of PPA involves a combination of clinical evaluation, neuropsychological testing, and neuroimaging. MRI and PET scans are often used to assess brain atrophy and metabolic activity. A detailed history and examination by a neurologist or speech-language pathologist are crucial.

Pathophysiology

The underlying cause of PPA is the degeneration of brain tissue in areas responsible for language. This degeneration is often associated with abnormal protein deposits, such as tau protein or TDP-43. The specific pathology can vary between the different variants of PPA.

Management

There is currently no cure for PPA, but management focuses on maintaining communication skills and quality of life. Speech therapy is a key component of treatment, tailored to the individual's specific language deficits. Supportive therapies and assistive communication devices may also be beneficial.

Research and Future Directions

Ongoing research aims to better understand the genetic and molecular basis of PPA, as well as to develop targeted therapies. Clinical trials are exploring the use of medications and interventions that may slow the progression of the disease.

See Also

• Aphasia
• Neurodegenerative disease
• Speech-language pathology

External Links

• [National Aphasia Association](https://www.aphasia.org/)
• [Alzheimer's Association](https://www.alz.org/)

NIH genetic and rare disease info

• NIH info on Aphasia, primary progressive

Aphasia, primary progressive is a rare disease.