PPA; Semantic Dementia; Aphasia, Primary Progressive; Primary Progressive Aphasia Syndrome

Primary Progressive Aphasia (PPA) is a rare type of dementia characterized by slow and gradual loss of language (aphasia). It affects the language and the person’s ability to retain general world knowledge (semantic dementia) and eventually progresses to amnesia. PPA is a subdivision of Pick disease. Recent studies have concluded that individuals with PPA have a specific combination of prion gene variants, although this is not the primary cause of the disorder.

Diagnostic testing:

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  Clinical exam;

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  Speech/language evaluation that examines word retrieval, sentence formulation, and auditory comprehension skills; and

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  MRI or CT scan of the brain demonstrating atrophy of the brain language areas or of the frontal and temporal lobes.

Exam: PPA symptoms vary, depending on which portion of the brain's language area is involved. The condition has three types, which cause different symptoms. The three types of PPA are:

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  Semantic variant;

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  Logopenic variant; and

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  Nonfluent-agrammatic variant.

Symptoms of semantic variant PPA include:

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  Having difficulty comprehending spoken or written language, particularly single words;

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  Having difficulty comprehending word meanings; and

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  Having difficulty naming objects.

Symptoms of logopenic variant PPA include:

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  Having difficulty retrieving words;

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  Frequently pausing in speech while searching for words; and

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  Having difficulty repeating phrases or sentences.

Symptoms of nonfluent-agrammatic variant PPA include:

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  Having difficulty forming words;

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  Being hesitant and halting in speech;

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  Making errors in speech sounds;

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  Having difficulty understanding sentences; and

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  Using grammar incorrectly.

ICD-9: 784.3

Symptoms of PPA begin gradually, usually before the age of 65 years, and worsen over time. Individuals with PPA may become mute and eventually lose the ability to understand spoken or written language within 10 years of diagnosis.

There is currently no effective treatment that can cure or slow the progression of PPA.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical examination that describes diagnostic features of the impairment;

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  Documentation of a clinically appropriate longitudinal medical history, speech-language assessment, neurological findings, and neuroimaging consistent with the diagnosis of PPA;

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  Speech/language assessment can determine the ability to express and comprehend oral and written messages:

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    A valid Total Language standard score that is at least 2 1/2 standard deviations below the mean on a comprehensive language test, such as the Oral and Written Language Scales, or

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    An Aphasia Quotient that is in the “severe” range on an aphasia battery such as the Boston Diagnostic Aphasia Examination or Western Aphasia Battery;

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  If the administration of a comprehensive language test or aphasia battery is not possible or speech-language testing is not available, a descriptive statement may be used. This statement should indicate that the claimant is either unable to follow a simple two-step direction without prompting or visual cues, or to spontaneously produce single words or two-word phrases, or both; and

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  Brain imaging (MRI or CT scan) documenting atrophy of fronto-temporal regions is necessary.

Suggested Listings for Evaluation:

REMARKS