Huntington’s disease (HD) is a rare, genetic neurodegenerative disorder often presenting with emotional, cognitive and behavioral abnormalities before manifestation of disease defining motor symptoms. Cognitive impairment is a frequent clinical feature caused by different dementia subtypes. Imaging cortical and subcortical glucose metabolism via 18F-FDG PET/CT can help to discriminate the underlying disease. The patient is a 54-year old man presenting with progressive cognitive impairment and mild orofacial dyskinesia. 18F-FDG PET/CT of the brain revealed a severe bilateral hypometabolism in the striatum. Following imaging Huntington’s disease was suspected and a molecular genetic testing confirmed the diagnosis. Huntington’s disease is a rare but important differential diagnosis of cognitive impairment, especially before motor symptoms are manifest. 18F-FDG PET is capable to show early striatal dysfunction in HD even when structural imaging is normal. We conclude that, in cases with negative family history the HD characteristic metabolic pattern can lead to the diagnosis when no other dementia-suspected changes are present.
The ageing of an organism is associated with certain features of functional decline that can be assessed at the cellular level (e.g., reduced telomere length, loss of proteostasis, etc.), but also at the organismic level. Frailty is an independent syndrome that involves increased multidimensional age-related deficits, heightens vulnerability to stressors, and involves physical deficits in mainly the locomotor/muscular capacity, but also in physical appearance and cognition. For sporadic Alzheimer’s disease, age per se is one of the most relevant risk factors, but frailty has also been associated with this disease. Therefore, we aimed to answer the two following questions within a cross-sectional study: (1) do Alzheimer’s model mice show increased frailty, and (2) what changes of the microbiota occur concerning chronological age or frailty? Indeed, aged 5xFAD mice showed increased frailty compared to wild type littermates. In addition, 5xFAD mice had significantly lower quantities of Bacteroides spp. when only considering frailty, and lower levels of Bacteroidetes in terms of both frailty and chronological age compared to their wild type littermates. Thus, the quality of ageing—as assessed by frailty measures—should be taken into account to unravel potential changes in the gut microbial community in Alzheimer’s disease.
Telepractice is increasingly finding its way into the care of people with dementia. Web-based delivery of speech and language therapy (SLT) is feasible and has the potential to improve communication in people with dementia-related speech disorders. Although experts are discussing the strengths and weaknesses of telepractice, a precise analysis of the differences between analogue and digital communication for this heterogeneous group of patients is still missing. The three current single cases investigated verbal and nonverbal aspects of communication in a face-to-face (F2F) and digital setting through a qualitative research design. Using the scenario-test (ST) in person and via big blue button (BBB; video conferencing system), several decisive factors were detected, influencing the effectiveness of communication in a F2F compared to a digital setting. The most important results of the qualitative content analysis are described for each case individually. Additionally, the influence of person-related factors, such as age, diagnosis, presence of depression, and level of education, is presented. Perceptual, executive, and affective disorders, as well as aids of relatives, are considered separately. The results indicate that executive functions, affects, and perceptual deficits need to be taken into account if telepractice is to be applied. Age, education, and distinct forms of dementia might be decisive for successful telepractice as well.
Background: Due to communication problems, people with primary progressive aphasia (PwPPA) are often affected in their self-image and experience a reduced quality of life (QoL). Biographic-narrative therapy is an effective approach to improve QoL in post-stroke aphasia. This study describes how PwPPA experienced their participation in the biographic-narrative intervention called Cope PPA. Methods: The intervention comprised a combination of five individual and seven group therapy sessions as well as the use of music and art therapy elements. Inclusion criteria were a capacity to give consent and sufficient visual/auditory abilities of PwPPA. Exclusion criteria were the presence of severe depression (MADRS > 35) or severe cognitive deficits (MMST < 10). After the therapy, PwPPA and their family members took part in half-hour semi-structured interviews. Interviews were analysed according to the reflexive thematic analysis by Braun and Clarke. Results: The qualitative analysis was based on a data set of 34 interviews. A total of six themes were identified: (1) Participation required adherence; (2) Materials were considered remarkable; (3) Storytelling was conducted in an aphasia-free area; (4) Group participation created a sense of belonging; (5) Experiences encouraged self-reflection and (6) Coping is lengthy and ongoing. Conclusions: The findings of our reflexive thematic analysis suggest that PwPPA experienced the intervention as meaningful. Some PwPPA described the effects of our intervention on their self-image. Others emphasised that coping with their condition was an ongoing process requiring continuous support.