Treatment for spoken and written word retrieval in the semantic variant of primary progressive aphasia.

This study investigated whether a treatment programme for spoken word retrieval, supplemented with written naming, was beneficial for an individual with right-hemisphere dominant semantic variant of PPA (svPPA). Assessment and treatment were delivered remotely through Skype. Treatment consisted of two phases of lexical retrieval therapy (Repetition and Reading in the Presence of a Picture: RRIPP), with and without written responses (Phases 1 and 2 respectively), and a third treatment phase based on the procedures of Conceptual Enrichment (COEN) therapy. The first two phases of treatment resulted in short-lasting improvements in spoken and written word retrieval, with greater improvement in Phase 2 when written production was also required. Both treatment phases resulted in gains only for treated items, but generalised to different depictions to those treated. However, Phase 2 also resulted in significant improvement of treated items on a comprehension task. COEN treatment did not result in significant gains in word retrieval or comprehension. This study reinforces the value of a simple lexical retrieval treatment delivered remotely. It adds to the current evidence that anomia in svPPA can be responsive to treatment, but also shows that challenges remain regarding maintenance effects and the generalisation of treatment effects to connected speech.

The tailored activity program (TAP) to address behavioral disturbances in frontotemporal dementia: a feasibility and pilot study.

PURPOSE: To explore the feasibility of implementing the Tailored Activity Program with a cohort of people with frontotemporal dementia and their carers (dyads). METHODS: The Tailored Activity Program is an occupational therapy based intervention that involves working collaboratively with family carers and prescribes personalized activities for behavioral management in people with dementia. Twenty dyads randomized into the study (Tailored Activity Program: n = 9; Control: n = 11) were assessed at baseline and 4-months. Qualitative analyzes evaluated feasibility and acceptability of the program for the frontotemporal dementia cohort, and quantitative analyzes (linear mixed model analyzes, Spearman's rho correlations) measured the impact of the program on the dyads. RESULTS: The Tailored Activity Program was an acceptable intervention for the frontotemporal dementia dyads. Qualitative analyses identified five themes: "carer perceived benefits", "carer readiness to change", "strategies used by carer to engage person with dementia", "barriers to the Tailored Activity Program uptake/implementation", and "person with dementia engagement". Quantitative outcomes showed an overall reduction of behavioral symptoms (F18.34 = 8.073, p = 0.011) and maintenance of functional performance in the person with dementia (F18.03 = 0.375, p = 0.548). CONCLUSIONS: This study demonstrates the potential for using an activity-based intervention such as the Tailored Activity Program in frontotemporal dementia. Service providers should recognize that while people with frontotemporal dementia present with challenging issues, tailored therapies may support their function and reduce their behavioral symptoms. Implications for rehabilitation The Tailored Activity Program is an occupational therapy based intervention that involves prescribing personalized activities for behavioral management in dementia. The Tailored Activity Program is an acceptable and feasible intervention approach to address some of the unique behavioral and functional impairments inherent in frontotemporal dementia.

Cognitive telerehabilitation in mild cognitive impairment, Alzheimer's disease and frontotemporal dementia: A systematic review.

INTRODUCTION: Given the limited effectiveness of pharmacological treatments, non-pharmacological interventions in neurodegenerative diseases have gained increasing attention in recent years and telerehabilitation has been proposed as a cognitive rehabilitation strategy. The purpose of this systematic review is to examine the evidence for the efficacy of cognitive telerehabilitation interventions compared with face-to-face rehabilitation in patients with mild cognitive impairment, Alzheimer's disease and frontotemporal dementia. METHODS: In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a systematic search of the Medline database was conducted. Out of 14 articles assessed for eligibility, five studies were identified, three in participants with mild cognitive impairment or Alzheimer's disease, two in patients with primary progressive aphasia. RESULTS: The Physiotherapy Evidence Database scale was used to assess the methodological quality of four out of five studies included in this systematic review, with only one report receiving a high-quality rating. Effect-size analysis evidenced positive effects of telerehabilitation interventions, comparable with those reported for face-to-face rehabilitation. DISCUSSION: The available evidence for the effectiveness of cognitive telerehabilitation is limited, and the quality of the evidence needs to be improved. The systematic review provides preliminary evidence suggesting that cognitive telerehabilitation for neurodegenerative disease may have comparable effects as conventional in-person cognitive rehabilitation.

Smartphone for smart living: Using new technologies to cope with everyday limitations in semantic dementia.

New technologies have considerable potential to support people with semantic dementia-a form of progressive aphasia-in their everyday lives, but evidence is still sparse. The first objective of the study was to document day-to-day compensation strategies, including the use of a smartphone, in ND, a 56-year-old man with semantic dementia. The second objective was to explore if, 5 years after receiving his diagnosis, ND could still learn new smartphone functions. Results for objective 1 showed that ND had adopted a large number of compensation mechanisms in his everyday life, and expanded the use of one application he had learned 4 years earlier. Results for objective 2 showed that, with an errorless learning approach, he learnt to effectively use 10 smartphone functions. He was also able to verbalise semantic knowledge about those functions and still used 40% of them in daily life 6 months post-intervention. He particularly appreciated note-taking, and spontaneously expanded his abilities in using this function's features in order to reduce his semantic difficulties. This study shows the potential of new mobile technologies for semantic dementia, how they can be adapted and modified as the disease progresses, and how some patients can creatively use external technological aids.

Successful short-term re-learning and generalisation of concepts in semantic dementia.

Patients with semantic dementia (SD) can rapidly and successfully re-learn word labels during cognitive intervention. This new learning, however, usually remains rigid and context-dependent. Conceptual enrichment (COEN) training is a therapy approach aimed to produce more flexible and generalisable learning in SD. In this study we compare generalisation and maintenance of learning after COEN with performance achieved using a classical naming therapy (NT). The study recruited a 62-year-old woman with SD. An AB1ACAB2 experimental design was implemented, with naming performance assessed at baseline, post- intervention, 3 and 6 weeks after the end of each treatment phase. Three generalisation tasks were also assessed pre- and post-intervention. Naming post-intervention improved significantly following both therapies, however, words trained using COEN therapy showed a significantly greater degree of generalisation than those trained under NT. In addition, only words trained with COEN continued to show significant improvements compared with baseline performance when assessed 6 weeks after practice ceased. It was concluded that therapies based on conceptual enrichment of the semantic network facilitate relearning of words and enhance generalisation in patients with SD.

Relearning knowledge for people in a case of right variant frontotemporal dementia.

Right variant frontotemporal dementia (Rvt-FTD) is a rare variant of FTD that usually presents with a progressive difficulty in recognizing familiar people. We aimed to determine whether rehabilitation of semantic knowledge for people improves recognition by both verbal and visual channels in a patient with Rvt-FTD. Knowledge for 21 famous people was assessed in a patient with Rvt-FTD before and after completing a semantic rehabilitation program. After rehabilitation recognition increased by 95% when presented with the famous people's names and related semantic facts, but only by 28% when presented with their faces. Recognition of people by verbal and visual channels improves differently after semantic knowledge rehabilitation.

How to constrain and maintain a lexicon for the treatment of progressive semantic naming deficits: Principles of item selection for formal semantic therapy.

The progressive degradation of semantic memory is a common feature of many forms of dementia, including Alzheimer's disease and the semantic variant of primary progressive aphasia (svPPA). One of the most functionally debilitating effects of this semantic impairment is the inability to name common people and objects (i.e., anomia). Clinical management of a progressive, semantically based anomia presents extraordinary challenges for neurorehabilitation. Techniques such as errorless learning and spaced-retrieval training show promise for retraining forgotten words. However, we lack complementary detail about what to train (i.e., item selection) and how to flexibly adapt the training to a declining cognitive system. This position paper weighs the relative merits of several treatment rationales (e.g., restore vs. compensate) and advocates for maintenance of known words over reacquisition of forgotten knowledge in the context of semantic treatment paradigms. I propose a system for generating an item pool and outline a set of core principles for training and sustaining a micro-lexicon consisting of approximately 100 words. These principles are informed by lessons learned over the course of a Phase I treatment study targeting language maintenance over a 5-year span in Alzheimer's disease and SvPPA. Finally, I propose a semantic training approach that capitalises on lexical frequency and repeated training on conceptual structure to offset the loss of key vocabulary as disease severity worsens.

Enhancing caregivers' understanding of dementia and tailoring activities in frontotemporal dementia: two case studies.

PURPOSE: The purpose of this study is to describe the intervention process and results of the Tailored Activities Program (TAP) in two people diagnosed with Frontotemporal Dementia (FTD). METHOD: TAP is an occupational therapy (OT) community-based intervention program that prescribes personalised activities to reduce difficult behaviours of dementia. The OT works with carers over a 4-month period (assessment, activity prescription and generalisation of strategies). Study measures were collected (blind researcher) pre- and post-intervention: cognition, functional disability, behavioural symptoms and Caregiver Confidence and Vigilance. RESULTS: A 51-year-old woman with behavioural-variant FTD could consistently engage in more activities post-intervention, with scores indicating improvements to behaviour, function and caregiver confidence. A 63-year-old man with semantic variant FTD engaged well in the prescribed activities, with scores reflecting reduced carer distress regarding challenging behaviours and improved caregiver vigilance. CONCLUSIONS: TAP is efficacious in FTD, allowing for differences in approach for FTD subtype, where behavioural symptoms are very severe and pervasive. IMPLICATIONS FOR REHABILITATION: The Tailored Activities Program is an intervention which can be tailored to account for unique behavioural and language profiles inherent across frontotemporal dementia (FTD) subtypes. Maintaining a flexible approach when applying an intervention in FTD allows for tailoring to individual case variability within FTD subtypes.

The CARE pathway model for dementia: psychosocial and rehabilitative strategies for care in young-onset dementias.

The goal of the Care Pathway Model for Dementia (CARE-D) is to improve quality of life and daily functioning both for individuals diagnosed with dementia and for their families or other caregivers. This is accomplished by developing individualized recommendations focused on a person's strengths and weaknesses as determined by formal neurocognitive and psychosocial evaluations. Careful attention is given to the stage of illness and an individual's stage in life, to connecting families with services that target an individual's cognitive and behavioral symptoms, and to providing education and emotional support specific to symptoms, clinical diagnosis, and prognosis.

Relying on procedural memory to enhance independence in daily living activities: Smartphone use in a case of semantic dementia.

Relying on procedural memory is a promising approach for interventions that address the cognitive difficulties found in semantic dementia. The aim of this study was to determine if procedural memory could be used to optimise learning of relevant smartphone functions in MH, a 55-year-old man with semantic dementia. The impact of learning to use specific smartphone applications, which display concepts and their semantic characteristics, on relearning useful significant concepts, was also explored in MH. This patient, who showed no deficits in procedural learning on a serial reaction time paradigm, was able to learn manipulations related to 15 smartphone functions although, because of his deficit in word comprehension, he generally needed verbal cues to clarify which functions he was asked to perform. Six months after the end of the intervention, he was still using 8 of the 15 functions regularly. However, repeated exposure to concepts through the use of two applications did not improve naming or retrieval of semantic attributes. This study showed the potential of relying on procedural memory to optimise learning of new technologies in the ecological rehabilitation of semantic dementia.

Restoration of conceptual knowledge in a case of semantic dementia.

Patients with semantic dementia (SD) may undergo successful relearning of object names, but these gains are usually restricted to the trained exemplars, demonstrating poor generalization. We hypothesized that generalization could be improved by restoring an item's semantic network through specific strategies that recruit the remaining personal semantic memories (conceptual enrichment therapies). We describe the case of a patient with SD who showed greater generalization of learning following a conceptual enrichment therapy than when learning items in a word-retrieval therapy. Our results suggest that enhancing an item's semantic network connections may result in improved generalization of learning in SD. A learning mechanism in the presence of compromised hippocampi is also discussed.

Giving words new life: generalization of word retraining outcomes in semantic dementia.

BACKGROUND: Anomia is a common and debilitating symptom for many dementia sufferers, but is particularly marked in patients with the semantic variant of primary progressive aphasia, semantic dementia (SD). Recent studies have demonstrated that through cognitive training these patients can re-learn the names of objects, but it remains unclear whether this translates to improved use of these relearned words in contexts other than picture naming. METHODS: Five SD patients completed a 2-month, online word training program and were assessed pre- and post-intervention on picture naming and spoken word-picture-matching plus two novel ecological tasks: video description and responses to verbal requests. RESULTS: All participants showed clear gains in naming the trained pictures (p < 0.001). Importantly, improvements were also observed for four out of the five patients on the video description task. Milder patients also demonstrated improved comprehension of verbal instructions. Severe SD patients showed improvements on matching trained words to pictures. As expected, improvements were not found for untrained items. CONCLUSION: There was clear evidence of generalization especially in patients with milder semantic impairments. Future studies should investigate the utility of this training in other forms of dementia.

Adapting to conversation with semantic dementia: using enactment as a compensatory strategy in everyday social interaction.

BACKGROUND: Studies to date in semantic dementia have examined communication in clinical or experimental settings. There is a paucity of research describing the everyday interactional skills and difficulties seen in this condition. AIMS: To examine the everyday conversation, at home, of an individual with semantic dementia. METHODS & PROCEDURES: A 71-year-old man with semantic dementia and his wife were given a video camera and asked to record natural conversation in the home situation with no researcher present. Recordings were also made in the home environment, with the individual with semantic dementia in conversation with a member of the research team. Conversation analysis was used to transcribe and analyse the data. Recurring features were noted to identify conversational patterns. OUTCOMES & RESULTS: Analysis demonstrated a repeated practice by the speaker with semantic dementia of acting out a diversity of scenes (enactment). As such, the speaker regularly used direct reported speech along with paralinguistic features (such as pitch and loudness) and non-vocal communication (such as body posture, pointing and facial expression) as an adaptive strategy to communicate with others in conversation. CONCLUSIONS & IMPLICATIONS: This case shows that while severe difficulties may be present on neuropsychological assessment, relatively effective communicative strategies may be evident in conversation. A repeated practice of enactment in conversation allowed this individual to act out, or perform what he wanted to say, allowing him to generate a greater level of meaningful communication than his limited vocabulary alone could achieve through describing the events concerned. Such spontaneously acquired adaptive strategies require further attention in both research and clinical settings in semantic dementia and analysis of interaction in this condition, using conversation analysis, may be helpful.

Bringing words back to mind - Improving word production in semantic dementia.

Patients with semantic dementia (SD) have significant impairments in naming and comprehension, but demonstrate relatively intact attention, everyday memory, and visuospatial skills. Given these preserved skills, attempts have been made to help re-build vocabulary in SD patients, with promising results. Such reports, however, are generally based upon only one or two cases and have employed variable retraining methods. It is thus unclear which elements of practice are crucial to success. Over two studies, we assessed four patients undergoing a word training program, who ranged in severity from mild to severe impairments to semantic knowledge. All four participants showed significant improvements in their ability to name trained items, with no changes in untrained items over the same time period. Improvements were evident within 3 weeks of practice, and could be established from a simple, repetitive practice of word-picture pairing, carried out at the participant's home. Strong effect sizes of the treatment were found in patients with severe deficits. Maintenance of learning was observed on some follow-up assessments, although continued practice is likely to be needed to sustain naming performance. Incorporating generation tasks into the practice may be assistive, but was not essential to success. These data support the utility of implementing simple home-practice programs even for patients with significant language deficits.

The Environmental Cleanliness and Clutter Scale (ECCS).

BACKGROUND: Development of a scale to rate the degree and various aspects of uncleanliness will facilitate description and research in cases of severe domestic squalor. METHODS: Discussion with representatives of relevant agencies led to selection of various aspects of domestic uncleanliness and clutter to be assessed when describing cases of squalor. Rooms and areas with varying functions (toilet, kitchen, bedroom, etc.) were all to be rated, along with other indicators of squalor (e.g. odor, vermin). Trial use and further discussion resulted in reduction to ten items, each rated on a four-point scale, with succinct descriptions of anchor-points. Two experienced psychogeriatricians then used this new Environmental Cleanliness and Clutter Scale (ECCS) to rate independently 55 dwellings which had been reported by Aged Care staff as cluttered or unclean, and also rated overall degree of squalor. Reliability and indications of validity were analyzed. RESULTS: The inter-rater reliability for the ten items was considered satisfactory (kappa 0.48, varying from 0.31 to 0.58). Cronbach's alpha for the new scale was 0.94, demonstrating high internal consistency. Each author's ratings of degree of squalor (none to severe) matched well with the other's ratings on the scale; a score of >12 usually indicated moderate or severe squalor. CONCLUSION: The ECCS proved reliable and useful in rating cases where elderly people were living in severe domestic squalor.