Monty Python’s Terry Jones Diagnosed with Rare Form of Dementia

Actor/director Terry Jones, 74, has been diagnosed with a rare form of dementia, called Primary Progressive Aphasia. Jones is best known as one of the members of Monty Python, which  also included Terry Gilliam, John Cleese, Michael Palin and Graham Chapman(d. 1989). Jones directed some of the group’s best-known films, including “The Meaning of Life,” “Life of Brian” and “Monty Python and the Holy Grail.”
A statement from the British Academy of Film and Television Arts (BAFTA) said:

“Terry has been diagnosed with Primary Progressive Aphasia, a variant of Frontotemporal Dementia. This illness affects his ability to communicate and he is no longer able to give interviews.”

The academy also announced that it will present its Special Award for Outstanding Contribution to Film and Television to Jones. Jones’ representative said of the award, “Terry is proud and honoured to be recognised in this way and is looking forward to the celebrations.”

Here is Terry playing Prince Herbert, in Monty Python and the Holy Grail:

What is Primary Progressive Aphasia?

Primary progressive aphasia (PPA) is a disorder characterized by language disturbance, including difficulty making or understanding speech (aphasia). In the early stages, PPA often causes difficulty with naming, word finding, or word comprehension. In later stages, affected people often become mute and lose their ability to communicate. PPA may sometimes be the initial manifestation of another neurodegenerative disease, such as frontotemporal dementia or Alzheimer disease.  In some cases, PPA is caused by mutations in the GRN gene and is inherited in an autosomal dominant manner.

Signs and symptoms of primary progressive aphasia (PPA) usually begin gradually and vary based on the type of PPA present. In addition to speech problems, reading and writing also are usually affected. Symptoms generally worsen over time. Affected people may become mute and eventually lose the ability to understand written or spoken language. Although additional cognitive (thought) symptoms may emerge later in the course of the illness, deficits must be generally confined to the area of language for at least a couple of years to fulfill a diagnosis of PPA.

PPA can be classified into three distinct types which include progressive non-fluent aphasia (PNFA), semantic dementia (SD), and the logopenic variant (LPA).

People with the semantic dementia type may have difficulty with:

  • understanding spoken or written language, particularly single words
  • understanding word meanings
  • naming objects

People with the lopogenic type may have:

  • difficulty retrieving words in speech
  • frequent pauses in speech while searching for words
  • slow speech
  • difficulty repeating phrases or sentences

People with the progressive non-fluent type may have:

  • difficulty speaking
  • hesitant, halting speech
  • errors in speech sounds
  • difficulty understanding sentences
  • difficulty using grammar correctly

Some people with PPA are ultimately diagnosed with other neurodegenerative diseases.

Neurodegenerative  diseases include

There is limited information in the medical literature about the long-term outlook (prognosis) for people with primary progressive aphasia (PPA) and what ultimately causes death in affected people. PPA may be the form of onset of any of a number of different neurodegenerative diseases, as mentioned above. Prognosis may therefore vary depending on whether another neurodegenerative disease develops, and what that disease is.

While the age of onset of PPA tends to fall between the ages of 50 and 70 years, some people may become affected before or after this age range. The condition tends to progress, developing into mutism in many cases. Loss of independence occurs at a later stage than in other types of dementia (at 7 years in 50% of all affected people, according to one author). Survival typically ranges from about 7 to 10 years, although recent studies suggest that the course of PPA may be more gradual.  In some people, language deficiency remains the predominant symptom, or even the only one. Others may develop additional problems including cognitive, behavioral, or extrapyramidal deficits (such as Parkinsonism). Alzheimer disease (AD) is found in many autopsies, but it is not known whether AD can cause PPA in its early stages or if two neurodegenerative diseases are coexisting as a result of shared causes.

Source: Genetic and Rare Diseases Information Center

Michele R. Berman, M.D. was Clinical Director of The Pediatric Center, a private practice on Capitol Hill in Washington, D.C. from 1988-2000, and was named Outstanding Washington Physician by Washingtonian Magazine in 1999. She was a medical internet pioneer having established one of the first medical practice websites in 1997. Dr. Berman also authored a monthly column for Washington Parent Magazine.

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