Background And Purpose: Nonfluent variant primary progressive aphasia (nfvPPA) is a neurodegenerative disorder characterized by the progressive deterioration of language functions that typically appears with atrophy predominating in the left peri-insular region (left-nfvPPA) on imaging. While both left-dominant and right-dominant presentations have been reported in semantic variant primary progressive aphasia, the other language presentation of frontotemporal dementia, no case series of nfvPPA with predominantly right-sided atrophy of the peri-insular region (right-nfvPPA) have been reported previously. This study explored whether such entities exist and what their clinical features might be.
View Article and Find Full Text PDFAlzheimers Dement (Amst)
November 2024
Introduction: This study investigated the changes in functional capacity with disease progression in a well-characterised cohort of patients diagnosed with frontotemporal dementia (FTD) and Alzheimer's disease (AD) presentations.
Methods: We recruited 126 behavioural variant FTD (bvFTD), 40 progressive nonfluent aphasia (PNFA), 64 semantic dementia (SD), 45 logopenic progressive aphasia (LPA), and 115 AD patients. Functional capacity was measured annually over ∼7 years using the Disability Assessment for Dementia.
Purpose: This paper introduces a practice framework for individualised positive behaviour support (PBS). The framework incorporates existing function-based PBS principles and integrates contemporary research and Australian legislation to frame practice elements through a human rights lens. It is designed to support people with disability of varied aetiologies across the lifespan in various settings (e.
View Article and Find Full Text PDFIntroduction: Mounting evidence indicates distinct memory profiles among the primary progressive aphasia (PPA) variants. Neuropsychological tests reveal disproportionate memory impairments in the logopenic variant PPA (lv-PPA) relative to the non-fluent variant PPA (nfv-PPA) and semantic variant PPA (sv-PPA). The real-world experience of day-to-day memory disturbances in PPA, however, remains poorly understood.
View Article and Find Full Text PDFObjective: This study investigated the visuospatial working memory profiles of behavioural variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD) using a novel computerised test of visuospatial working memory: the Box Task.
Methods: Twenty-eight bvFTD and 28 AD patients, as well as 32 age-matched control participants were recruited. All participants completed the Box Task and conventional neuropsychological tests of working memory, episodic memory, and visuospatial function.
The delivery of a dementia diagnosis, the information provided, and the practical advice and support arranged can have a long-lasting impact on patients and their families and deserves attention equal to that given to the assessment and investigation process. Patients and their families need a constructive yet sensitive conversation about the nature and cause of their difficulties, communicated in plain language, and tailored to their main concerns and needs. This conversation should lead to the provision of high-quality, easily accessible information.
View Article and Find Full Text PDFImpaired verbal 'phonological' short-term memory is considered a cardinal feature of the logopenic variant of primary progressive aphasia (lv-PPA) and is assumed to underpin most of the language deficits in this syndrome. Clinically, examination of verbal short-term memory in individuals presenting with PPA is common practice and serves two objectives: (i) to help understand the possible mechanisms underlying the patient's language profile and (ii) to help differentiate lv-PPA from other PPA variants or from other dementia syndromes. Distinction between lv-PPA and the non-fluent variant of PPA (nfv-PPA), however, can be especially challenging due to overlapping language profiles and comparable psychometric performances on verbal short-term memory tests.
View Article and Find Full Text PDFObjectives: Almost 10% of people with dementia experience a younger-onset of disease (before 65 years). Changes in behaviour are common, as are delays in diagnosis and limited access to appropriate support and services. This study aimed to explore the specific behaviour support needs of families living with younger-onset dementia.
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