[Primary progressive aphasia: its clinical variability: an analysis of 15 cases]

Abstract

Introduction: Primary progressive aphasia (PPA) represents a clinical syndrome linked to multiple degenerative diseases. The diagnosis of PPA is made when language is the only area of salient and progressive dysfunction for at least the first two years of the disease.

Aim: To evaluate the neuropsychological, neuropsychiatric and language characteristics of the PPA.

Patients and methods: 15 patients with PPA underwent language, neuropsychological and neuropsychiatric evaluation, magnetic resonance imaging, computerized tomography and single photon emission computerized tomography.

Results: We observed a clear distinction between the oral expression patterns; the patients were classificated by type of aphasia. The most common sign of PPA was a word finding deficit, also known as anomia. Seven aphasia type Broca, four sensorial transcortical aphasia, two aphasia type Wernicke and two anomic aphasia were found in our patients. Depression, apathy, anxiety and irritability were the most prevalent neuropsychiatric sign.

Conclusions: PPA is a language-based syndrome, that include fluent (normal articulation, flow and number of words per utterance) and nonfluent form of aphasia. It has been considered a cognitive term, however, PPA is associated with high prevalence of psychiatric manifestations. More research it will be necessary to evaluate the prognostic value of them. The slow and progressive deterioration of language provides an interesting model to understand the mechanisms and biological bases involved in the linguistic process.