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STUART J. KANTER, DO RONAN M. FACTORA, MD* THEODORE T. SUH, MD, PhD, MHSc†
CME
CREDIT
Section of Geriatric Medicine, Department of
General Internal Medicine, Cleveland Clinic
Section of Geriatric Medicine, Department of
General Internal Medicine, Cleveland Clinic
Section of Geriatric Medicine, Department of General Internal
Medicine, Cleveland Clinic
■ WHAT IS PPA?
*Dr. Factora has indicated that he owns stock in the Pfizer corporation and has received
honoraria from Pfizer for teaching and speaking.
†Dr. Suh has indicated that he has received honoraria from the Pfizer corporation for teaching PPA is clinically defined as a progressive lan-
and speaking. guage deficit that leads to the dissolution of
TA B L E 1
Cognitive domains affected by PPA,
Alzheimer disease, and vascular dementia
LANGUAGE MEMORY MEMORY EXECUTIVE
EXPRESSION REGISTRATION RECOGNITION FUNCTION
almost all language functions, while other cog- 6th decade, and it arises only rarely after age
nitive functions remain relatively preserved 75. Patients experience isolated language
until late in the course of the disease. problems for a median of 4.5 years (range
Although controversy exists regarding the 1–15) before seeking medical attention.4
nomenclature of PPA, several subtypes have PPA is primarily a sporadic disorder, but it
been recognized. PPA can be either fluent or can be inherited in an autosomal-dominant
nonfluent, and with or without repetition or manner. Familial PPA has been described, and
impairments in comprehension, reading, or the disease has been linked to chromosomal
writing.1 PPA in which speech is nonfluent locus 17q21.4 Early studies of apolipoprotein E
and agrammatical is known as progressive non- genotyping in PPA do not indicate a predispo-
fluent aphasia. PPA with fluent speech but poor sition with the E4 allele, which has been
comprehension is called semantic dementia. observed in Alzheimer disease.5
PPA with fluent aphasia is also called logopenic Neuropathologic studies demonstrate
progressive aphasia. changes consistent with Alzheimer disease in
She states Depending on the type of aphasia, specif- 30% of PPA cases. In about 70%, the micro-
ic areas of the brain are affected.2 Progressive scopic findings include focal spongiform
‘I know what I nonfluent aphasia is associated predominantly changes and focal neuronal degeneration,
want to say with atrophy of both frontal lobes. Difficulty resembling the neuropathological findings
but I can’t in naming and understanding words is associ- found in frontal lobe dementia of the non-
ated with involvement of the left parietotem- Alzheimer type. An additional 20% of
say it’ poral lobe. Difficulty with recognizing faces patients with PPA have Pick disease, charac-
and objects is associated with involvement of terized by tau-positive spherical neuronal
the right temporal lobe. inclusions.3
Errors in speech may be semantic (word
choice) or phonemic (sound-based). Speech ■ PPA VS STROKE, MASS LESION,
may also be described as telegraphic (using ALZHEIMER DISEASE
abnormally short phrases). Examples of
semantic errors in the sentence “I will come The differential diagnosis of PPA includes
my house in your car and drive my car into stroke, mass lesion, and Alzheimer disease.
Chicago” include the inappropriate use of Dominant hemispheric stroke involving
“my” and “into Chicago” instead of “to the language centers produces aphasia similar
Chicago.”3 The sentence “For exercise I like to the language deficits in PPA, but presents
to runt and lit wastes” demonstrates phonemic acutely, making confusion with PPA unlikely.
errors in the substitution of “runt” for “run” A slow-growing mass in the language cen-
and “lit wastes” for “lift weights.” Many of ter could also produce aphasia, but an imaging
these errors can be identified during the study would exclude this possibility.
patient interview. Aphasia is common in Alzheimer disease
Although PPA can appear in people as but is almost invariably is associated with early
young as 35, the mean age of onset is in the memory loss or other cognitive impairment.