FACTSHEET 4: What is primary progressive aphasia?

Frontotemporal dementia or FTD is a progressive disorder of the brain. It can affect behaviour, language skills and movement.

Primary progressive aphasia or PPA is one of the subtypes of FTD. The two main subtypes of PPA are called:

  • Semantic dementia or SD
  • Progressive nonfluent aphasia or PNFA

A third subtype has been more recently described and is called:

  • Logopenic aphasia or LPA

These are also sometimes called the semantic, nonfluent and logopenic variants of PPA:

  • svPPA
  • nfvPPA
  • lvPPA

In all of the types of PPA impairment of language skills is the main problem initially.

Symptoms of SD include:

  • Difficulty finding the right word
  • Losing understanding of what words mean
  • Talking about things in a vague manner
  • Difficulty understanding what other people are saying
  • Problems with reading
  • Problems with spelling

See FACTSHEET 5 for more details

Symptoms of PNFA include:

  • Slow, hesitant speech
  • Difficulty finding the right word to say
  • Pronouncing words incorrectly
  • ‘Telegraphic’ speech
  • Producing the wrong grammar
  • Saying the opposite word to the one they mean to say
  • Problems with reading
  • Problems with spelling

See FACTSHEET 6 for more details

Symptoms of LPA include:

  • Difficulty finding the right word to say
  • Pausing in the middle of sentences
  • Difficulty repeating sentences

LPA is usually felt to be an unusual form of Alzheimer’s disease, and is sometimes called the language variant of Alzheimer’s disease.

In all of the different forms of PPA other non-language symptoms occur later on, including:

  • Changes in behaviour
  • Problems with planning and problem solving
  • Difficulty remembering things

Physical problems occur in some people similar to the symptoms of Parkinson’s disease. Very rarely people with PPA may develop motor neurone disease.

Does PPA run in families?

In some cases PPA can be a genetic disorder and run in families. This is most likely for PNFA.

Mutations in either the progranulin or C9ORF72 gene can cause PPA.

See FACTSHEET 2 for more details about familial FTD.

How is PPA diagnosed?

Usually a diagnosis is made by a specialist rather than a GP. There is no single test that will make a diagnosis. A series of tests are usually performed including a scan of the brain.

In PPA the scan may show loss of cells mainly on the left side of the brain. This can either be at the front of the brain in an area called the frontal lobe, or in the middle of the brain in an area called the temporal lobe.

See FACTSHEET 11 for more details.

Is there a treatment for the nonfluent variant of PPA?

There is currently no cure for PPA but there are some important things which can help when caring for someone – see FACTSHEET 12 for more details.

The PPA Support Group has been set up to help people with PPA and their carers in the UK. It meets a number of times a year. More information can be found at www.ucl.ac.uk/drc/support-groups.

For a list of PPA support groups in the US and Canada see www.ppaconnection.org.