Tag Archives: Dementia

Scientists at Mayo Clinic, Jacksonville, Florida, USA have created a novel mouse that exhibits the symptoms and neurodegeneration associated with the most common genetic forms of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), both of which are caused by a mutation in the a gene called C9ORF72. The study was published in the journal Science.

ALS destroys nerves that control essential movements, including speaking, walking, breathing and swallowing. After Alzheimer’s disease, FTD is the most common form of early onset dementia. It is characterized by changes in personality, behavior and language due to loss of neurons in the brain’s frontal and temporal lobes. Patients with mutations in the chromosome 9 open reading frame 72 (C9ORF72) gene have all or some symptoms associated with both disorders.

“Our mouse model exhibits the pathologies and symptoms of ALS and FTD seen in patients with theC9ORF72 mutation,” said the study’s lead author, Leonard Petrucelli, Ph.D., chair and Ralph and Ruth Abrams Professor of the Department of Neuroscience at Mayo Clinic, and a senior author of the study. “These mice could greatly improve our understanding of ALS and FTD and hasten the development of effective treatments.”

To create the model, Ms. Jeannie Chew, a Mayo Graduate School student and member of Dr. Petrucelli’s team, injected the brains of newborn mice with a disease-causing version of the C9ORF72 gene. As the mice aged, they became hyperactive, anxious, and antisocial, in addition to having problems with movement that mirrored patient symptoms. The brains of the mice were smaller than normal and had fewer neurons in areas that controlled the affected behaviors. The scientists also found that the mouse brains had key hallmarks of the disorders, including toxic clusters of ribonucleic acids (RNA) and TDP-43, a protein that has long been known to go awry in the majority of ALS and FTD cases.

“Finding TDP-43 in these mice was unexpected” Dr. Petrucelli said. “We don’t yet know how foci and c9RAN proteins are linked to TDP-43 abnormalities, but with our new animal model, we now have a way to find out.” Dr. Petrucelli and his team think these results are an important step in the development of therapies for these forms of ALS and FTD and other neurodegenerative disorders.

Chew et al. “C9ORF72 Repeat Expansions in Mice Cause TDP-43 Pathology, Neuronal Loss and Behavioral Deficits,” Science, May 14, 2015. DOI: 10.1126/science.aaa9344

Although past research has associated obesity with increased risk of dementia, a new study – deemed the largest ever to assess the link between body mass index and dementia risk – suggests obesity could actually be a protective factor against the condition, while people who are underweight may be at increased risk.

A large retrospective cohort study, published in The Lancet Diabetes and Endocrinology, has revealed a surprising association between being underweight in mid-life and late-life, and increased risk of dementia.

The study assessed the medical records of almost 2 million people in the UK in order to gain a better understanding of how obesity affects dementia risk. The researchers found that, compared with adults who had a healthy BMI (between 20-25 kg/m2), those who were underweight – defined in this study as a BMI less than 20 kg/m2 – during middle age were 34% more likely to be diagnosed with dementia. This increased risk remained for 15 years after adults’ underweight status was recorded.

The team notes that participants with a BMI of less than 18.5 kg/m2 are usually classed as underweight, but the threshold was raised in this study to allow comparisons with past studies, which have defined a BMI of less than 20 kg/m2 as underweight.

The researchers also found that middle-aged adults’ risk of dementia steadily reduced as their BMI increased. Compared with adults who had a healthy BMI, those who were severely obese (BMI greater than 40 kg/m2) were 29% less likely to develop dementia. The team says their results remained even after accounting for factors associated with increased dementia risk, including smoking and alcohol consumption. In addition, the results were not affected by adults’ age at dementia diagnosis or the decade in which they were born, according to the researchers.

Source: Medical News Today

The Dementia Discovery Fund, which is being established by the UK government with initial commitments totalling $100 million, brings together leading pharmaceutical companies, the UK government and Alzheimer’s Research UK to address the rising threat posed by dementia by supporting research into future treatments.

The fund aims to identify and nurture promising new avenues of research from around the world in the field of dementia. It is hoped that by providing critical financial support and expert advice during the early stages of research, the development of innovative new treatments for this disease could be accelerated.

The Dementia Discovery Fund will be structured as a typical venture capital fund, but will be the first to focus solely on dementia research. The Fund will comb the global research community for the most promising early stage research to invest in. A scientific advisory board of representatives from each of the partner organisations will provide expert scientific input during the selection of research programmes, as well as providing ongoing advice during pre-clinical and early clinical development. Partners will then be sought for the progression of promising assets through the clinical development pipeline, the intention being that proceeds from licensing or sale of such programmes will be returned to the Fund and its investors. The Fund will appoint a professional investment manager in due course, which will be responsible for its financial governance and investment decisions.

Source: Reuters

The current policy approach to tackling dementia is socially and economically unsustainable, according to a new OECD report.

Countries need to take action now to improve the lives of people living with dementia and their carers, prioritise public research on dementia, and improve the incentives for private investment in dementia innovation.

Addressing Dementia: The OECD Response says that the human and financial costs of this incurable disease are huge. Nearly 50 million people are living with dementia worldwide, costing societies over half a trillion US dollars each year, roughly equal to the GDP of Switzerland.

Source: OECD

Belgian scientists have completed a study, reprogramming skin cells from three dementia patients into induced pluripotent stem cells (iPSCs) – immature cells that mimic stem cells taken from early-stage embryos. Their findings, which revealed a signalling pathway linked to frontotemporal dementia (FTD), are published in the January 13th 2015 edition of the journal, Stem Cell Reports.

Prof. Philip Van Damme, from the Leuven Research Institute for Neuroscience and Disease in Belgium, said: “Our findings suggest that signalling events required for neurodevelopment may also play major roles in neurodegeneration.

Treatment with a drug that suppressed the pathway, known as “Wnt”, restored the ability of neurons affected by the disease to develop normally. “Targeting such pathways…may result in the creation of novel therapeutic approaches for frontotemporal dementia”, Prof. Van Damme said.

The researchers found that iPSCs derived from the patients’ cells were unable to generate cortical neurons, the cell type most affected by FTD. Cortical neurons are the cells responsible for most of the brain’s complex higher activity enabling thought, perception and voluntary movement.

Co-author Dr Catherine Verfaillie, from the University of Leuven in Belgium commented that IPSC models could now be used to better understand dementia, and in particular FTD, which accounts for about half of dementia cases before the age of 60.

Source: Alzheimer Europe

The Dementia in Europe Yearbook 2014, produced by Alzheimer Europe is a comparative report on care pathways for people with dementia living at home in Europe.

The pathways to get a diagnosis of dementia are complex and are likely to be multifactorial. Many people living with dementia in Europe are still not diagnosed, and often the diagnosis comes too late. Every person with dementia has the right to a high quality, timely diagnosis, if they so wish. There is now clear indication that people can live well with dementia. Nevertheless, without the right support and care this may not be possible. Getting the necessary support and care depends on several factors. Among them, availability and appropriateness are key, as are the informational aspects and the navigability of the complex health and care systems involved in the diagnosis and care of people with dementia.

This comparative report contains information on national policies and practices addressing different aspects of the timely diagnosis of dementia and of the post-diagnostic care and support available to individuals living with dementia in 30 European countries. The report outlines the main similarities and differences in the processes that people need to follow to be diagnosed and to access the support and care in these countries. It also highlights some of the gaps and main challenges that these individuals may experience. In doing so, the report shows that there is not always a single, linear pathway that may suit every person and every country.

The Dementia in Europe Yearbook 2014 is available for free download on the Alzheimer Europe website.

Source: Alzheimer Europe

A new study entitled “Evaluating the relationship between amyloid-β and α-synuclein phosphorylated at Ser129 in dementia with Lewy bodies and Parkinson’s disease” reports a correlation between α-synuclein and amyloid-β in the brain of patients affected by neurodegenerative diseases, such as Parkinson’s disease. The study was published in the journal Alzheimer’s Research &Therapy.

In this study, the authors measured both soluble and insoluble amyloid-β and α-synuclein in human post-mortem brain tissue and analyzed whether there is a relation between the levels of amyloid-β, total α-synuclein, pSer129 α-synuclein and cognitive function ante-mortem in Parkinson’s disease patients. Their main findings were the discovery of a positive correlation between the levels of insoluble pSer129 α-synuclein with insoluble and soluble amyloid-β protein in most brain regions analyzed. The correlation was significantly higher in Parkinson’s disease and dementia with Lewy bodies patients when compared to controls. Additionally, the authors found that the proportion of α-synuclei phosphorylated at Ser129 correlated with ante-mortem mini-mental state examination.

Their findings establish a pathogenic link between the accumulation of amyloid-β and the phosphorylation of Ser129 at α-synuclein, thus increasing disease severity and the probability of developing dementia. The authors highlight that additional studies are required to understand fully how the interaction is maintained.

Source: Alzheimer’s News Today

The first 25 research projects of the Dutch “Delta Plan Dementia” have been announced and will start before the end of the year. The studies vary in focus, from new treatments and prevention to better patient care and support. Results are expected in the coming years.

The 25 studies have received over EUR 21 Million in funding. The Dutch Government was the major contributor. Alzheimer Nederland was the major private investor with a contribution of over EUR 1 Million. Gea Broekema-Prochazka, director of Alzheimer Netherlands said “We expect breakthroughs that are important for the patients that are faced with dementia today and for the patients of the future.”

Source: Alzheimer Europe

The Medical Research Council (MRC) Dementias Research Platform UK (DPUK) is a multi-million pound public-private partnership, developed and led by the MRC, to accelerate progress in, and open up, dementias research. The DPUK’s aims are early detection, improved treatment and ultimately, prevention, of dementias.

The DPUK is creating the world’s largest population study for use in dementias research, bringing together two million participants aged 50 and over, from 22 existing study groups within the UK. Included are people from the general population, people known to be at-risk of developing dementia, and people diagnosed with early-stage dementia.

The DPUK is linking a number of industry partners with a UK-wide network of academic excellence. Six UK and international companies have signed a consortium agreement which will allow shared access to the research resources and the rich and unique data in the DPUK, and to provide a basis for instigating joint studies.

DPUK is being set up for use as a research resource for the entire scientific community. Read more at the link below.

In the UK, to support clinical and care staff, managers and estates colleagues, the King’s Fund has produced a range of resources to enable hospitals, care homes, primary care premises and specialist housing providers to become more dementia friendly.

The work that informed the initial development of the resources, the EHE dementia care programme, was funded by the Department of Health to support the implementation of the National Dementia Strategy and the Prime Minister’s ‘Challenge on Dementia’.

The EHE programme is designed to improve the environment of care for people with dementia. It involved 23 teams from acute, community and mental health NHS trusts who worked on a range of projects across the dementia care pathway and sought to make hospital environments less alienating for people with cognitive problems. Projects have demonstrated that relatively inexpensive interventions, such as changes to lighting, floor coverings and improved way-finding, can have a significant impact. Evaluation has shown that environmental improvements can have a positive effect on reducing falls, violent and aggressive behaviours, and improving staff recruitment and retention. The EHE schemes are already showing that it is possible to improve the quality and outcomes of care for people with dementia as well as improve staff morale and reduce overall costs by making inexpensive changes to the environment of care.

The King’s Fund has also published on Sept. 4th 2014, the final report of the Commission on the Future of the Health and Social Care in England (the Barker report). “A New Settlement for Health and Social Care, relations between health and social services”, makes a plea for “better integration” and warns of “hard choices” ahead.