NMDA receptor antibody encephalitis presenting as Transient Epileptic Amnesia

Transient Epileptic Amnesia (TEA) is a subtype of temporal lobe epilepsy, typically presenting in a person's early 60s, and of unknown aetiology. Encephalitis caused by antibodies to NMDA receptors (NMDARE) has not previously been documented in TEA. We describe a 47-year-old male who satisfied criteria for TEA, but given his atypical symptoms, was also screened for autoimmune epilepsy. High levels of serum NMDAR antibodies were found, suggesting NMDARE. Immunosuppressive treatment gradually eliminated the NMDA receptor antibodies. Our case extends the clinical spectrum associated with neuronal cell-surface autoantibodies to include atypical cases of TEA.This article is freely available via Open Access. Click on the Publisher URL to access the full text

The relationship between response consistency in picture naming and storage impairment in people with semantic variant Primary Progressive Aphasia

This is the author accepted manuscript. The final version is available from the American Psychological Association via the DOI in this record.Objective. The progressive loss of stored knowledge about word meanings in semantic variant Primary Progressive Aphasia (svPPA) has been attributed to an amodal “storage” deficit of the semantic system. Performance consistency has been proposed to be a key characteristic of storage deficits but has not been examined in close detail and larger participant cohorts. Methods: We assessed whether 10 people with svPPA showed consistency in picture naming across three closely consecutive sessions. We examined item-by-item consistency of naming accuracy and specific error types, while controlling for the effects of variables such as word frequency, familiarity and age of acquisition. Results: Participants were very consistent in their accurate and inaccurate responses over and above any effects of the word-related variables. Analyses of error types that compared consistency of semantic errors, correct responses and other error types (e.g., phonologically related errors, unrelated errors) revealed lower consistency. Conclusions: Our findings support the assumption that semantic features constituting semantic representations of objects are progressively lost in people with svPPA and are therefore consistently unavailable during naming. Variability in the production of error types remains when distinctive features of an object are lost resulting in the selection of semantically or visually similar items, or in the failure to select an item and the production of a no-response. The assessment of performance consistency sheds light on the underlying impairment of people with semantic deficits (semantic storage versus access deficit). This can support the choice of an appropriate treatment technique aiming to maintain, or re-learn semantic information

A complete sample of quasars from the 7C redshift survey

We present details of a new sample of radio-loud quasars drawn from 0.013 sr of the 7C Redshift Survey. This sample is small (21 quasars) but complete in that every object with an unresolved nucleus and/or broad emission lines with S(151MHz) > 0.5 Jy has been discovered. The dependence of the quasar fraction with redshift and radio luminosity is investigated, providing new evidence supporting the unification of radio-loud quasars and powerful radio galaxies. This 7C sample is compared with optically-selected quasars, in order to determine whether there are systematic biases in the different selection techniques. There are no lightly reddened (Av approx. 1) quasars in our sample amongst the 14 with z < 2. The discovery of a reddened quasar at z = 2.034 and its implications are discussed. A tight correlation between radio luminosity and optical/near infrared continuum luminosity for a subset of the sample is also found.Comment: 6 pages Latex, To appear in the "Cosmology with the New Radio Surveys" Conference - Tenerife 13-15 January 199

Measuring Awareness in People With Dementia: Results of a Systematic Scoping Review

This is the final version. Available on open access from SAGE Publications via the DOI in this recordBackground: Awareness of the diagnosis or related changes in functioning varies in people with dementia (PwD), with implications for the well-being of PwD and their carers. Measuring awareness in a clinical setting could facilitate tailored support and optimize involvement in personal health and care decisions. This scoping review aimed to identify validated methods of assessing awareness in dementia and appraise their clinical utility. Method: A systematic search was conducted of English-language publications that measured awareness in PwD, in 6 electronic databases. Search terms included dement*, Alzheimer*, Pick disease, and awareness, unawareness, anosognosia, insight, denial, metacognit*, or discrepanc*. Results: We screened 30,634 articles, finding 345 articles that met our inclusion criteria. We identified 76 measures, most commonly using a discrepancy questionnaire comparing evaluations of function by PwD and an informant. There were 30 awareness measures developed and validated for use in dementia populations but few designed for general clinical use. Conclusions: Although we found a range of clinical indications for measuring awareness, there were few studies investigating clinical applications and few tools designed for clinical purposes. Further investigation and development of a person-centered tool could facilitate health and care choices in mild-to-moderate dementia.Alzheimer’s Societ

Non-primary progressive language impairment in neurodegenerative conditions: protocol for a scoping review

BACKGROUND: Progressive language difficulties arise in many neurodegenerative conditions, causing significant impact upon patients and families. This occurs most obviously in primary progressive aphasia (PPA) but can also occur within other forms of progressive disease. In these cases, language decline may be significant, but as they are not the presenting or dominant symptom, may be overlooked in favour of more prominent cognitive, behaviour or motor deficits. To date, there has been no systematic investigation into non-primary progressive aphasia. This scoping review aims to describe the currently reported language impairments found in non-language-led dementias and identify their clinical relevance, defined as the impact on everyday living. It also seeks to identify the reported interventions for language impairment in this patient group to-date. METHOD: We will conduct a scoping review of published studies that have assessed and/or treated aphasia in people diagnosed with a neurodegenerative condition other than primary progressive aphasia. The systematic search will include the electronic databases PubMed, MEDLINE, OVID-EMBASE, PsycINFO, and speechBITE, using search terms for specific non-language-led dementia subtypes. Findings will be mapped and described according to the type of language difficulties identified and rehabilitation approaches employed. Intervention studies will be evaluated for their methodological rigour using validated scales. DISCUSSION: This scoping review will provide an overview of the types of aphasia found in neurodegenerative conditions where language dysfunction is not the primary focus. Current treatment approaches (and gaps in the provision of treatment) will be identified

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

Semantic variant primary progressive aphasia: Practical recommendations for treatment from 20 years of behavioural research

People with semantic variant primary progressive aphasia (svPPA) present with a char-acteristic progressive breakdown of semantic knowledge. There are currently no pharmacological interventions to cure or slow svPPA, but promising behavioural approaches are increasingly reported. This article offers an overview of the last two decades of research into interventions to support language in people with svPPA including recommendations for clinical practice and future research based on the best available evidence. We offer a lay summary in English, Spanish and French for education and dissemination purposes. This paper discusses the implications of right-versus left-predominant atrophy in svPPA, which naming therapies offer the best outcomes and how to capitalise on preserved long-term memory systems. Current knowledge regarding the maintenance and generalisation of language therapy gains is described in detail along with the development of compensatory approaches and educational and support group programmes. It is concluded that there is evidence to support an integrative framework of treatment and care as best practice for svPPA. Such an approach should combine rehabilitation interventions addressing the language impairment, compensatory approaches to support activities of daily living and provision of education and support within the context of dementia

Reading behaviour project report 2016 : ‘Digital Magpies’ - the academic reading habits of undergraduate students

Engagement with the Library is essential in delivering a successful student experience. A previous Library Impact Project found that students who use the Library more tend to achieve better academic results. However, analytics from library systems indicate declining book borrowing and electronic resource usage. Are students really reading less? Student academic reading patterns have adapted to the increase in digital resources. As a result they may be “reading smarter’’. Feedback indicates they expect to find and use information quickly, synthesising information from a variety of sources. As part of a commitment to improve retention and completion figures, the Library has initiated a Reading Behaviours project at the University of Salford, focused on the reading habits of undergraduate students. It explores what motivates their academic reading; whether reading patterns vary according to purpose or source, academic discipline, status, or age and what this means for our role in helping students to find resources. In light of findings, how should we tailor classroom training, e-learning and collaboration with academics to support the student journey? Several key issues have emerged during this project:1. Synthesizing information for an academic purpose2. Embedding information literacy as a flexible learning habit3. Supporting students as they adapt to new learning context

Clinical outcomes in transient epileptic amnesia: A 10-year follow-up cohort study of 47 cases

This is the final version. Available on open access from Wiley via the DOI in this recordOBJECTIVE: Transient epileptic amnesia (TEA) is a form of adult-onset epilepsy where presenting features are well described, but little is known regarding prognosis. This study aimed to elucidate the long-term prognosis of TEA regarding seizure control, memory, medical comorbidities, and life expectancy. METHODS: Up-to-date clinical information was collected for 47 people diagnosed with TEA who had joined the The Impairment of Memory in Epilepsy (TIME) study 10 years earlier. At entry to the study, information about comorbid conditions was systematically collected. Details regarding subsequent diagnoses, seizure activity, changes to treatment, or reports of cognitive impairment were obtained through the family doctor. The variables of interest were compared with UK population data. RESULTS: Mortality in the cohort was 21 of 47 (45%), with an average age at death of 82.5 years. Seizures remained well controlled for the majority but medications required adjustments in dose and type for some (28%). A small number (three cases) remained seizure-free without medication. History of cardiovascular disorders was frequent (78.7%), typically involving hypertension (55.3%). Autoimmune disorders (25.5%), cancer (23.4%), and depression (21.3%) were also commonly reported. Although persisting memory problems were often noted, dementia was diagnosed in seven cases (14.9%). Life expectancy and comorbidities in TEA did not differ from available population norms. SIGNIFICANCE: Results suggest that life expectancy is not reduced in TEA. Although TEA does not appear to be a self-limiting form of epilepsy, seizures are typically well controlled via medication. Because adjustments to medication may be required, even after long periods of stability, ongoing medical monitoring is recommended. Comorbid vascular disorders are frequent but appear similar to general population estimates. Monitoring mood may be important, given that people with chronic conditions are often vulnerable to depression. Because of persisting memory difficulties, the development of effective memory interventions for people with TEA is warranted.Dunhill Medical TrustMedical Research Council (MRC)Alzheimer's Societ