Profile of linguistic and cognitive deficits in persons with a semantic variant of a primary progressive aphasia

Abstract

Primary progressive aphasia (PPA) includes a group of neurodegenerative disorders that are characterized by progressive deterioration of language functions, while other cognitive functions, at least at the onset of the disease, are relatively spared. There are three basic subtypes of PPA: the nonfluent progressive aphasia (nvPPA), the semantic variant of a primary progressive aphasia (svPPA), and the logopenic progressive aphasia (lvPPA). The semantic variant of a PPA can also be found in the literature under the term of semantic dementia. It is clinically manifested by progressive deterioration of semantic knowledge, fluent aphasia, impaired naming and comprehension, prosopagnosia and surface dyslexia and dysgraphia (in languages with irregular orthography). As the disease progresses, other cognitive changes can be observed. The main cause of the disorder is progressive bilateral atrophy of the anterior temporal lobes, which is more manifested in the left hemisphere. The literature is modest in terms of the use of specific treatment methods in the rehabilitation of these patients. Since speech and language disorders are the most conspicuous symptom, at least at the beginning of the disease, the role of speech therapists in the assessment and restitution of speechlanguage and communication skills is also indisputable.

Primarna progresivna afazija (PPA) obuhvata grupu neurodegenerativnih poremećaja, koje karakteriše progresivno propadanje jezičkih funkcija, dok su druge kognitivne funkcije, barem na početku bolesti, relativno pošteđene. Izdvojene su tri osnovne varijante PPA: nefluentna progresivna afazija (nvPPA), semantička varijanta primarne progresivne afazije (svPPA) i logopenična progresivna afazija (lvPPA). Semantička varijanta PPA može se pronaći u literaturi i pod nazivom semantička demencija. Klinički se ispoljava progresivnim propadanjem semantičkog znanja, fluentnom afazijom, oštećenim imenovanjem i razumevanjem, prozopagnozijom i površinskom disleksijom i disgrafijom (u jezicima s nepravilnom ortografijom). S progresijom bolesti, uočavaju se i druge promene na kognitivnom planu. Osnovni uzrok ove bolesti je progresivna obostrana atrofija prednjih temporalnih režnjeva, koja je izraženija u levoj hemisferi. Nema dovoljno podataka u literaturi o korišćenju specifičnih metoda tretmana u rehabilitaciji ovih bolesnika. S obzirom na to da su govorno-jezički poremećaji najupadljiviji simptom, bar na početku bolesti, nesporna je i uloga logopeda u proceni i restituciji govorno-jezičkih i komunikativnih sposobnosti.