Progressive non_fluent Aphasia (PNFA) or Primary Progressive Aphasia (PPA) by
Dr Jonathan Rohrer & Dr. Jason Warren, Dementia Research Center
PNFA is caused by degeneration (loss) of brain cells mainly affecting the parts of the brain (the frontal and temporal lobes on the left side) that control speech.
Symptoms
The first symptoms are usually difficulties producing spoken language. The term ‘aphasia’ means problems with language. These may include:
- Slow, hesitant speech that is effortful to produce.
- Difficulty finding the right word to say.
- Pronouncing words incorrectly.
- lsquo;Telegraphic’ speech, missing out small words such as ‘the’, ‘and’, ‘or’ etc.
- Saying yes when meaning no and vice versa.
- Problems reading or writing.
Comprehension of what other people are saying is affected to a more variable extent and may be completely normal in the earlier stages of the illness. Non-language functions such as memory, calculation and personality are not usually affected early in the illness. Although the course of the disease varies considerably from person to person, it usually extends over a number of years. Swallowing and other motor functions may become affected as the disease progresses.
Medical tests
Language ability, memory and other aspects of thinking (cognitive functions) will be assessed, initially by a doctor, and often followed by a more detailed assessment by a psychologist. Brain scans can show the loss of brain cells in PNFA (shrinkage of the affected parts of the brain – normally the left frontal and temporal lobes) but there is no single test that can specifically diagnose PNFA with complete reliability during a person’s lifetime. It can therefore be confused with other disorders in which there are problems with language (for example, stroke). Your doctor will often arrange blood tests or other tests (often including a brain scan and EEG or electroencephalogram, and sometimes a lumbar puncture) to help confirm the clinical diagnosis and rule out diseases that can produce similar symptoms to PNFA.
Treatment
Unfortunately, there are no medications presently available which can treat the disorder or slow its progression. Treatment therefore focuses on helping people to manage their symptoms and treating problems such as mood changes that may contribute to the difficulties that people experience. Speech and language therapy concentrating on developing strategies for communication can be useful. This may include the use of communication aids (either simple communication books or for some people electronic devices).
Prognosis
Language problems deteriorate over time – there is a slow but progressive decline in the amount of speech, finding the right words, and ability to speak clearly. Problems are often confined to speech for the first few years of the illness but this does vary between people. Comprehension of speech and movements of the mouth and face and swallowing may become affected. Later in the illness non-language functions can also be affected including changes in personality, and difficulties performing familiar actions. People can have problems carrying out their normal daily living activities and managing their affairs, and they will need extra care and support.
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