With newer research-based classification systems, the term Vascular Cognitive Impairment (VCI) is now preferred to vascular dementia. VCI is an umbrella term that includes all forms of cognitive deficits ranging from mild cognitive impairment of vascular origin (VaMCI) to vascular dementia (VaD).The new VCI construct takes into account the fact that in addition to single strategic infarcts, multiple infarcts, and leukoaraiosis, there are other mechanisms of cerebrovascular disease such as chronic hypoperfusion that might account for the pattern of cognitive deficits associated with vascular dementia. The key to defining the spectrum of VCI is neuropsychological testing, bedside or office-based clinical examination, and neuroimaging. The lack of specific cognitive tools that are sufficiently sensitive to detect subtle deficits makes the assessment of cognitive impairment difficult. Prospective cross-sectional and longitudinal studies of VCI from different settings are therefore required.Although there have been few published reports, behavioural and psychological symptoms (BPS) are inherently present in VCI from the onset and during the course of the disease. Besides the type of population (i.e. clinical, community or nursing-home settings), the definition of VCI/VaD and the instruments used, and differences in the prevalence and pattern of BPS between various studies, could be due to other, often unconsidered, factors such as gender, age, education, use of medication and VCI/ /VaD severity.
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.
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