Stephanie Fowler

1.
Primary progressive aphasia (PPA) is a form of aphasia that is gradually progressive. Primary
progressive aphasia can affect syntax, object naming, and word comprehension. To be diagnosed with
PPA, language must be the only affected area in the initial phase; no other concomitant symptoms
may be present (Chapey, 2008). There may be deficits in activities of daily living, but only those that
are associated with language, such as talking on the phone. Present progressive aphasia cannot be
diagnosed when there are changes in mood, behavior, or memory abilities that are not directly related
to the language problem. In addition, no visual problems should be present. (Gorno-Tempini, 2011)
There are three different types of primary progressive aphasia; semantic, nonfluent/agrammatic, and logopenic. Patients with semantic PPA will display difficulties with singleword comprehension, especially words that are not used in high frequencies. They will also have word
finding difficulties, impaired knowledge of objects, and may have dyslexia or dysgraphia. Patients
with this form of PPA will still be able to repeat and their syntax and motor speech will not be
affected (Gorno-Tempini, 2011). This type of primary progressive aphasia will show atrophy or
abnormalities in blood flow in the left temporal lobe with more posterior abnormalities than the other
forms of PPA (Chapey, 2008). The next type is the non-fluent/agrammatic form of PPA. This form
will present with agrammatisms, which could be in the form of deleting closed class words, incorrect
syntax, etc. Speech output may require a lot of effort for this form of PPA. A patient with this
diagnosis will display difficulty with complex sentences in terms of syntax. They may show
difficulties with passive sentences, embedded clauses, and negation. They may also show speech
sound errors and distortions in spontaneous speech. Their single word comprehension and knowledge
of objects will be unaffected (Gorno-Tempini, 2011). With non-fluent PPA, Chapey reports that
neuroimaging will show atrophy or metabolic dysfunction in the left inferior front cortex and
perisylvian area (Chapey, 2008). The last form of PPA is logopenic. Characteristics of this form of
PPA include deficits in repetition of phrases and sentences, speech sound errors in spontaneous speech
and naming tasks, and difficulties with word retrieval. This type of primary progressive aphasia will
not present with agrammatism. There may be a slowing or decrease in verbal output (Chapey, 2008).
The ability to comprehend single words and object knowledge is largely unaffected. Mesulam reports
that errors in spontaneous speech are usually phonologic in nature (Gorno-Tempini, 2011). This
type of PPA will have localized damage to the inferior parietal lobe (Chapey, 2008).
The etiologies for primary progressive aphasia remain unknown. Mesulam suggests that there
is a genetic component to this diagnosis. He reports that PPA can be inherited in an autosomal
dominant manner; the majority of these patients have mutations in the progranulin (GRN) gene
(Gorno-Tempini, 2011). However, this has not been studied in much detail. Neurological imaging has
revealed damage and atrophy to the left perisylvian, anterior temporal lobe, and inferior parietal lobe.
There can be some damage in the right hemisphere, but the left usually is more affected. Due to this,
primary progressive aphasia should be referred to as an asymmetric disease and not a focal one
(Chapey, 2008). As stated previously, this is a progressive disease. In the initial phase of PPA,
language is the only area affected. Patients may have difficulty with word finding at this stage. The
average onset for primary progressive aphasia is approximately 60 years. Deficits in language can
remain as the only symptoms for years after onset and diagnosis. A majority of patients will
eventually experience symptoms in areas outside of language, such as mood, memory, or personality
(Chapey, 2008). As the disease progresses into the phase where these other areas are affected, the
diagnosis will change from PPA to aphasia in conjunction with the other disorder, or just the other
disorder by itself (e.g., dementia) (Chapey, 2008).

This type of aphasia has some similarities and differences to the more commonly seen types of
aphasias. The major difference between aphasia and primary progressive aphasia is that typical
aphasia is a sudden onset due to stroke, tumor, etc. Another difference is that patients with primary
progressive aphasia are aware of their deficits, where many traditional aphasics are not. As previously
mentioned, the onset of primary progressive aphasia is insidious and gradual (Sapolsky 2011). There
are many similarities in the way the language deficits can present themselves. Both primary
progressive aphasia and typical aphasia can have fluent and non-fluent classifications. They can both
present with agrammtism, word finding difficulties, difficulty with comprehending complex
sentences, etc. They also both present with damage to the left hemisphere language areas.
2.
The Progressive Aphasia Severity Scale (PASS) differs from standardized assessments such as
the BDAE and WAB, because it is not a formal assessment. The PASS is a rating scale where
different areas are presented, such as fluency, syntax, single-word comprehension, etc. There are
rating areas from 0-3, ranging from normal to severe impairments. Each subset and severity class
gives example characteristics that would assign a patient that rating (Sapolsky, 2010). The WAB and
BDAE are both standardized assessments that have normed samples and yield raw scores that can be
converted into percentile rankings. The PASS will just give you a general rating that is largely
subjective.
To assess this patient, I would first get a patient intake form with client information. This
would include information about how long these symptoms have been occurring and what areas they
feel they are demonstrating difficulties with. One of the unique aspects of this type of aphasia is that
the patient is largely aware of the deficits and will be better equipped to explain what is happening.
This will tremendously help the clinician and the course of therapy.
I would administer the Boston Diagnostic Aphasia Examination. While this patient does not
present with the typical aphasia that this test was designed for, it assesses many areas that the patient
may exhibit deficits in. By using a comprehensive assessment like the BDAE, it will give insight in
what specific areas this patient is displaying difficulties. Subtests that require picture description will
give pertinent information about syntax and word finding abilities. The subtest portions that require
picture naming tasks will give information regarding word finding and object knowledge. Other
subtests will give important information about the patients ability to comprehend different
complexities of sentences, repetition abilities, and single word comprehension. All areas necessary to
identify which type of PPA is present will be assessed in the BDAE. The results of this test would be
utilized to determine which type of primary progressive aphasia the patient is presenting with by
analyzing the patients performance on various subtests and language tasks.


3.
The patients adenocarcinoma that has spread to a cyst in the brain has contributed to the
aphasia. This mass occurs in the left fronto-parietal region of the brain and the secondary mass effect
is likely the cause of the aphasia. The patient received radiation for the adenocarcinoma, which has
caused a change in the white matter of the brain. The change in this tissue will hinder the ability for it
to function. The patient has also been experiencing seizures and states of unconsciousness, which may
contribute to the presenting aphasia. The patient also reports that she was hospitalized for a fall and
head trauma in the past, which may have been a contributing factor.
Due to the fact that the MRI showed the cyst on the left fronto-parietal lobe which is in close
proximity to Brocas area, I would expect to see deficits primarily in expressive language. The patient

will likely omit closed class words like articles, grammatical markers, and prepositions. She will show
difficulty utilizing verbs. The patient will likely be unable to put sentences together. Content words
will remain. All areas and modalities will be affected, but expressive language will be the primary
deficit. I would expect these language symptoms due to the lesion around Brocas area; however, a
full assessment of the patient is needed to see what symptoms she is presenting with.

References
Chapey, R. (2008). Primary Progressive Aphasia and Apraxia of Speech. In Language intervention
strategies in aphasia and related neurogenic communication disorders (5th ed., pp. 543-560).
Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins.
Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. F.,
Grossman, M. (2011).
Classification of primary progressive aphasia and its variants.
Neurology, 76(11), 10061014.
doi:10.1212/WNL.0b013e31821103e6
Sapolsky, D., Bakkour, A., Negreira, A., Nalipinski, P., Weintraub, S., Mesulam, M.-M.,
Dickerson, B. C.
(2010). Cortical neuroanatomic correlates of symptom severity in primary
progressive aphasia. Neurology, 75(4), 358366. doi:10.1212/WNL.0b013e3181ea15e8
Sapolsky, D., Domoto-Reilly, K., Negreira, A., Brickhouse, M., McGinnis, S., & Dickerson, B.
(2011). Monitoring progression of primary progressive aphasia: Current approaches and future
directions.
Neurodegenerative Dis. Manag, 2011;1:43-5