Extract from the PPA newsletter issued
by Dr. Nancy Johnson, Northwestern University Medical School, The Cognitive
Neurology and Alzheimer Center, Chicago.
Primary Progressive Aphasia (PPA) is
a disorder caused by degenerative diseases of the brain, in which speech
and language functions deteriorate gradually over a period of years, while
memory and other abilities remain relatively preserved. This form of dementia
tends to occur more often in men than in women.
SYMPTOMS:
Symptoms can begin as early as the 40's,
but can appear any time afterwards. Initially, the symptoms of PPA are
confined exclusively to the area of language functions. The types of language
difficulties that occur differ between patients, but generally involve
things such as the following:
Increased difficulty thinking of words
-
substituting the wrong word (e.g. "school"
for "work")
-
mistakes in pronunciation (e.g., "truss"
for "truck")
-
talking around the word (e.g., (We went
to the place to get bread where you can buy it for the word "bakery")
Problems reading or writing
-
no longer able to write cheques, letters
-
difficulty following written directions,
reading signs
Reduced ability to understand speech
-
trouble following conversations, especially
in larger groups
-
asking for repetition often, misunderstanding
things that are said
Decreased use of language
-
speech may become garbled or empty of
any real information and difficult to comprehend early in the course of
the illness
-
eventually may be unable to use speech
to communicate, becoming mute
Problems in arithmetic and calculations
-
may lose ability to perform even simple
mathematical operations
-
no longer able to handle money
In patients with PPA, you may get the
impression that they can't
remember because they usually can't express
themselves well. However, the fact that their memory system is working
relatively well is apparent from their actions: they are able to perform
activities of daily living independently. They do not get lost. They continue
to perform daily chores and routines.
There have been several PPA patients
whose brains have been studied at autopsy. In most cases (about 60%) the
pathology is "Non-specific Focal Degeneration".
WHAT IS THE COURSE OF PPA?
Symptoms associated with PPA are progressive.
Early in the disease process impairment are confined exclusively to the
area of language. However, as the disease progresses, eventually other
abilities such as memory, attention, personality and behaviour, become
compromised as well. The time frame within which these changes occur varies
greatly between patients. A few cases have been reported in which language
deficits were the only symptoms for at least 10 years. On average, impairments
are confined exclusively to the area of language for approximately 5 years.
In some cases though, deficits in areas other than language may be noticeable
after 2 years. |
