Understanding Primary Progressive
Primary progressive aphasia is a rare neurological
syndrome in which language capabilities become slowly and
progressively impaired. This information sheet was prepared
by the NAA to help explain the condition of Primary Progressive
Aphasia to the general public.
What is aphasia?
Aphasia is an acquired disorder caused
by brain damage which affects a person's ability to communicate.
The principal signs of aphasia are impairments in the ability
to express oneself when speaking, trouble understanding speech,
and difficulty with reading and writing. Aphasia is most often
the result of stroke or head injury but can also occur in other
neurological disorders, such as brain tumor or Alzheimer's disease.
The effects of aphasia differ from person to person and can
sometimes be eased by speech therapy. Strategies to communicate
non-verbally (without words) may also be helpful to the person
What is primary
The syndrome of primary progressive
aphasia has been defined by Mesulam and colleagues as a progressive
disorder of language, with preservation of other mental functions
and of activities of daily living, for at least two years. Primary
progressive aphasia is not Alzheimer's disease. Most people
with primary progressive aphasia maintain ability to take care
of themselves, pursue hobbies, and, in some instances, remain
employed. The problem is a disorder of language, and signs and
symptoms of other clinical syndromes are not found through tests
routinely used to determine the presence of other conditions.
Although primary progressive aphasia
may take a number of forms, it commonly appears initially
as a disorder of speaking (an articulatory problem), progressing
to nearly total inability to speak in its most severe stage,
while comprehension remains relatively preserved. A less common
variety begins with impaired word finding and progressive
deterioration of naming and comprehension, with relatively
However, other neurological disorders exist
in which progressive deterioration of language is only one
component of a broad, progressive decline of mental functions,
including memory, attention, visuospatial skills, reasoning,
and the carrying out of complex motor activities.These diseases,
such as Alzheimer's disease, Pick's disease, and Creutzfeld-Jakob
disease, should be excluded by appropriate neurologic examinations,
when a person experiences progressive language decline.
Is there any assistance for people
with primary progressive aphasia?
People with primary progressive aphasia
are fighting against a condition in which they will continue
to lose their ability to speak, read, write, and/or understand
what they hear. Usually, people with aphasia that results from
stroke or head injury will experience improvement over time,
often aided by speech therapy. This is not the case for people
with primary progressive aphasia, although they may benefit
during the course of their illness by acquiring new communication
strategies from speech-language pathologists. Some families
have also learned new strategies through participation in Aphasia
cards and other materials available from the National Aphasia
Association can aid in communicating the person's condition
to others. Some communication-assistive devices may also be
helpful. (See NAA's information sheet on technology resources.)
Non-verbal techniques for communicating, such as gesturing,
pointing to pictures, etc., may help people with primary progressive
aphasia express themselves.
Getting additional information
None of the information provided on
this fact sheet is intended as medical advice. If you believe
you or a relative or friend may have primary progressive aphasia,
you should consult a physician.
For individuals who have access to a medical library or the
internet, the following papers discuss primary progressive
Other papers relating to very technical
medical issues in primary progressive aphasia are also available.
Some are listed in Medline. For information on searching through
the medical literature, see NAA fact sheet Information about
aphasia rehabilitation research.
- Mesulam M-M. Slowly progressive aphasia
without dementia. Annals of Neurology, 11:592-598, 1982.
- Weintraub S, Rubin NP, Mesulam M-M. Primary
progressive aphasia: longitudinal course, neurological profile,
and language features. Archives of Neurology, 47:1329-1335,
- Kertesz A, Hudson L, MacKenzie IRA, Munoz
DG. The pathology and nosology of primary progressive aphasia.
Neurology, 44:2065-2072, 1994.
- Kirshner II, Baker M. Syndromes of language
dissolution in aging and dementia. Comprehensive Therapy,
- Mesulam M-M, Johnson N, Grujic Z, Weintraub
S. Apolipoprotein E genotypes in primary progressive aphasia.
Neurology, 49:51-55, 1997.
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