Rare Disease Report: Monty Python Alum Terry Jones has Rare Disease

Terry Jones of Monty Python’s Flying Circus has been diagnosed with Primary Progressive Aphasia (PPA), a type of Frontotemporal Dementia.

The announcement came from his representative in response to being invited to accept a British Academy Cymru Award on October 2, 2016 in Wales.

Jones was part of the revolutionary television show Monty Python’s Flying Circus from 1969 to 1974. He also directed or co-directed many of Monty Python Films, including Monty Python and the Holy Grail (1975), Monty Python’s Life of Brian, and Monty Python’s Meaning of Life.

What is Primary Progressive Aphasia

Primary Progressive Aphasia (PPA) is a neurological syndrome in which language abilities become progressively impaired.

Unlike language problems that occur in a stroke, the impairments seen in primary progressive aphasia are irreversible.

The 3 types of PPA are:

Semantic PPA:  The person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

Agrammatic PPA or progressive nonfluent aphasia: The person has trouble linking nouns with verbs correctly.

logopenic PPA: The person has difficulty finding the right words during conversation but can understand words and sentences.

In addition to losing the ability to speak, persons with PPA also progressively  lose their ability to read and write.

PPA is a rare disease with a typical onset between the ages of 50 – 70 years. Terry Jones is 74 years old.

There is currently no treatment to delay the progression of PPA but they can be given speech therapy to improve language skills and provide alternative ways to communicate.

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