Characteristics and Management of Primary Progressive Aphasia Primary progressive aphasia (PPA) was first proposed as a distinct clinical entity by Mesulam (1982) . Mesulam distinguished this disorder from other degenerative neurologic conditions such as Alzheimer Disease (AD) by its gradual progression of language dysfunction in the absence of more widespread cognitive or behavioral disturbances. Mesulam and Weintraub ... Article
Article  |   October 01, 1999
Characteristics and Management of Primary Progressive Aphasia
Author Affiliations & Notes
  • Margaret A. Rogers
    Department of Speech and Hearing Sciences, University of Washington, Seattle, WA
  • Nancy B. Alarcon
    Department of Speech and Hearing Sciences, University of Washington, Seattle, WA
Article Information
Articles
Article   |   October 01, 1999
Characteristics and Management of Primary Progressive Aphasia
SIG 2 Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders, October 1999, Vol. 9, 12-26. doi:10.1044/nnsld9.4.12
SIG 2 Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders, October 1999, Vol. 9, 12-26. doi:10.1044/nnsld9.4.12
Primary progressive aphasia (PPA) was first proposed as a distinct clinical entity by Mesulam (1982) . Mesulam distinguished this disorder from other degenerative neurologic conditions such as Alzheimer Disease (AD) by its gradual progression of language dysfunction in the absence of more widespread cognitive or behavioral disturbances. Mesulam and Weintraub (1992)  further refined this definition by suggesting that the progressive deterioration of speech and language functions associated with PPA should occur for at least two years in the absence of generalized cognitive or behavioral disturbances. After a 2-year history of isolated language symptoms, some proportion (perhaps as much as 50%) of individuals diagnosed with PPA eventually exhibit more widespread cognitive involvement consistent with a diagnosis of dementia. Black (1996)  stated that over 160 cases presenting with progressive aphasia as the predominant symptom have been reported since Wechsler’s patient described in 1977, but reports of cases that meet the diagnostic criteria of Mesulam and Weintraub (1992)  can be found in articles published even earlier (e.g., Rosenfeld’s case from 1909 as cited by Luzzati & Poeck, 1991). While estimates of the prevalence, incidence, and other epidemiological information have not yet been established, it is generally acknowledged that patients presenting with isolated deterioration of language function for a period of more than 2 years are relatively rare, especially compared to the prevalence of AD or dementia in general. Given the rarity of this disorder, gleaning information about trends in this population is difficult and has been limited to estimates based on published reports. Due to a lack of epidemiological information, it is difficult to provide prognostic information to individuals diagnosed with PPA. In this current review of the literature, 57 articles were identified that contained reports of 147 cases of patients with relatively isolated deterioration of speech and language for at least 2 years. The purpose of this review was to obtain specific information concerning the variety of speech and language symptoms present in this population and to consider the relationship of speech and language dysfunction to eventual cognitive status, duration of isolated language symptoms, and the presence of concomitant nonlinguistic sequelae. This information is considered in relation to providing proactive management to assist individuals with PPA to communicate as effectively as possible even once their aphasia reaches severe stages.
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