Primary progressive aphasia

Main article: Aphasia

Primary progressive aphasia
Classification and external resources
Specialty
OMIM	607485
Patient UK	Primary progressive aphasia
MeSH	D018888

Primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired while other mental functions remain intact. It was first described as a distinct syndrome by M.-Marsel Mesulam in 1982. Primary Progressive Aphasias have a clinical and pathological overlap with the Frontotemporal Lobar Degeneration (FTLD) spectrum of disorders and Alzheimer's disease.

Classification

Three classifications of primary progressive aphasia have been described. In the classical Mesulam criteria for primary progressive aphasia, there are 2 variants: a non-fluent type Progressive Nonfluent Aphasia (PNFA) and a fluent type Semantic Dementia (SD). A third variant of primary progressive aphasia, Logopenic Progressive Aphasia (LPA) is an atypical form of Alzheimer's disease.

Diagnostic criteria

The following diagnosis criteria were defined by Mesulam

• Gradual impairment of object naming, syntax and word-processing
• Premorbid language function is usually intact
• Acalculia: inability to perform simple mathematical calculations

• Ideomotor Apraxia: loss of the ability to execute or carry out learned purposeful movements

Risk Factors

There are no known environmental risk factors for the progressive aphasias. However, one observational, retrospective study suggested that vasectomy could be a risk factor for PPA in men. These results have yet to be replicated or demonstrated by prospective studies.

PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration, including PPA, are at slightly greater risk of developing PPA or another form of the condition.

Treatment

There is no approved treatment. But speech therapy can assist an individual with strategies to overcome difficulties. ″There are three very broad categories of therapy interventions for aphasia: restorative therapy approaches, compensatory therapy approaches, and social therapy approaches. Rapid and sustained improvement in speech and dementia in a patient with primary progressive aphasia utilizing off-label perispinal etanercept, an anti-TNF treatment strategy also used for Alzheimer's, has been reported. A video depicting the patient's improvement was published in conjunction with the print article. These findings have not been independently replicated and remain controversial.

See also

• Anomic aphasia
• Aphasiology
• Apraxia of speech
• Speech and Language Pathology
• Speech disorder

• Transcortical sensory aphasia

External links

• FAQ on PPA from IMPPACT, the International PPA Connection
• PPA information from the UCSF Memory and Aging Center
• Northwestern Cognitive Neurology and Alzheimer's Disease Center