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Neurology
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
This is a group of disorders characterised by frontal/temporal lobe atrophy, often with young onset (<65 years). There are three main syndromes: frontotemporal dementia – personality change/behavioural symptoms.semantic dementia – loss of semantic memory, i.e. conceptual knowledge.progressive non-fluent aphasia – impaired speech production.Other causes of dementia – it is important to rule out ‘reversible’ causesMetabolic: Vitamin B12/folate deficiency, hypothyroidism, Wilson’s diseaseInflammation/infection: HIV, multiple sclerosis, cerebral vasculitis, SLE, neurosyphilisDegenerative: Huntington’s disease, progressive supranuclear palsy, CJD Alcohol/toxicStructural: chronic subdural haematoma, tumours
Dementia
Published in Rachael E. Docking, Jennifer Stock, International Handbook of Positive Aging, 2017
Frontotemporal dementia is the impact of pathological changes in the front and temporal lobes of the brain, sometimes referred to as Pick’s disease or frontal lobe dementia. Frontotemporal dementia is more common in people under the age of 65 (Mercy et al., 2008). There are three clinical presentations of frontotemporal dementia: Frontotemporal behaviour variant – particularly noticeable are changes in the person’s personality and behaviour, such as reward-seeking behaviours including overeating and alcohol use (Perry et al., 2014).Progressive non-fluent aphasia – the main impact is on a person’s speech, which becomes slow and hesitant, with errors in grammar and understanding complex sentences.Semantic dementia – the development of problems understanding the meaning of words, and finding the right word, which progresses to difficultly recognising familiar people or objects (Alzheimer’s Society, 2013a).
Frontotemporal Dementia
Published in Marc E. Agronin, Alzheimer's Disease and Other Dementias, 2014
Semantic dementia (SD) is associated with degeneration in bilateral middle and inferior regions of the temporal lobes and is characterized by progressive impairment in semantic memory; namely, knowledge of particular words and objects and their meanings. Afflicted individuals initially retain sufficient language skills to carry out a conversation but with progressive loss of word comprehension as well as lack of insight into their language dysfunction. They demonstrate a fluent aphasia in which specific words are often substituted for more general ones (e.g., “animal” used instead of the specific animal intended), and the overall word content lacks meaning (Cardarelli, Kertesz, & Knebl, 2010). There is also lack of recognition or agnosia for well-known faces and objects and their function. Individuals with SD may demonstrate behavioral problems seen in FvFTD but with more compulsive behaviors, preoccupations with ideas, emotional disturbances (e.g., depression, anxiety, irritability, overreactivity to pain), and loss of empathy and sympathy (Boxer & Miller, 2005; McMurtrey et al., 2006).
A randomized crossover single-case series comparing blocked versus random treatment for anomia
Published in Neuropsychological Rehabilitation, 2023
Victoria A. Diedrichs, Jennifer P. Lundine, Deena Schwen Blackett, Alexandra Zezinka Durfee, Xueliang Jeff Pan, Stacy M. Harnish
Semantic dementia, also known as the semantic variant of primary progressive aphasia, is a neurodegenerative condition primarily affecting semantic knowledge and leading to anomia, among other deficits (Gorno-Tempini et al., 2011). For a review of word-retrieval treatments in semantic dementia and other forms of primary progressive aphasia, see Jokel et al. (2014). In an anomia treatment study for three participants with semantic dementia, Hoffman et al. (2015) found that introducing variability into the naming treatment by altering the presentation sequence led to greater retention of trained items at follow-up for two participants, despite a similar benefit of the variable and fixed-order presentation sequences during training. Although this study was not structured to specifically assess blocked and random practice schedules, the results support the notion that a random schedule may facilitate maintenance due to greater and variable spacing between trial items, requiring repeated lexical retrieval. However, a study that directly assessed the benefit of spaced retrieval on naming in a single participant found no benefit of spacing recall intervals (Bier et al., 2009).
No negative impact of word retraining on vocabulary use or clarity of communication in semantic dementia
Published in Neuropsychological Rehabilitation, 2023
Sharon A. Savage, Leonie F. Lampe, Lyndsey Nickels
The aim of this study was to examine data from a series of participants with semantic dementia (n = 9) who had previously taken part in a tailored word retraining programme to determine: Is there an increase in misuse (overgeneralization) of words following word retraining that is not attributable to perseverative or repetitive responding?Do the types of naming errors change from pre- to post-training for items that are not correctly named?Is there a reduction in the clarity of communication when an individual does not retrieve the target word (e.g., because the individual now produces an incorrect word in lieu of providing an accurate description)?
Training flexible conceptual retrieval in post-stroke aphasia
Published in Neuropsychological Rehabilitation, 2022
Sara Stampacchia, Glyn P. Hallam, Hannah E. Thompson, Upasana Nathaniel, Lucilla Lanzoni, Jonathan Smallwood, Matthew A. Lambon Ralph, Elizabeth Jefferies
Degradation of heteromodal concepts, in contrast, results in multimodal semantic impairment, affecting both verbal and non-verbal stimuli. Atrophy of the ventrolateral anterior temporal lobes (seen in semantic dementia, SD) leads to progressive degradation of semantic knowledge. SD patients show loss of specific and less familiar items first and consistent performance across different tasks probing the same concepts (Jefferies & Lambon Ralph, 2006; Lambon Ralph et al., 2010). These patients show some benefits in training studies focussed on relearning conceptual distinctions as long as the training is continued, potentially reflecting the fact that the anterior temporal lobes (ATLs) can support patterns of relearning despite degradation (Bier et al., 2009; Heredia et al., 2009; Hoffman et al., 2015; Jokel et al., 2006; Jokel et al., 2010; Mayberry, Sage, Ehsan, et al., 2011; Reilly et al., 2010; Savage et al., 2013).