Progression of language and cognitive impairment in patients with the logopenic variant of primary progressive aphasia – case series

Abstract

Aim: The paper presents longitudinal observation of four patients diagnosed with the logopenic variant of primary progressive aphasia (lvPPA). Materials and methods: The results of 3–4 language and cognitive assessments were available for four individuals (three women, one man) with lvPPA. The length of the observation period was 2–4 years. Language evaluation was comprehensive and addressed narrative speech, naming, word and sentence comprehension, repetition, reading and writing. Neuropsychological examination, whenever feasible, assessed visuospatial function, praxis, memory and executive functions. Results: Anomia was not an isolated symptom at the time of lvPPA diagnosis. Rapid deterioration of both spoken and written communication was observed. Reading aloud single words and high-frequency word comprehension were preserved longer than other linguistic competences. The narrative speech was progressively impoverished in terms of idea density, manifested by a reduced use of nouns and verbs in particular and an increased use of pronouns. At the advanced stage of the disease, the idea density was very low. There was marked deterioration of visuospatial functions, praxis and episodic memory in all patients. Progression to full-blown dementia was observed in all patients. Conclusions: The progression of linguistic and cognitive symptoms in lvPPA, albeit slightly heterogeneous, is relatively fast. The patients usually reach dementia stage within 1–2 years of the diagnosis and sometimes even earlier than 2 years of the declared symptom onset. When planning long-term management of lvPPA cases, rapid deterioration and progression to full-blown dementia within a relatively short time should be considered.