Down Syndrome Daily: dementia

Showing posts with label dementia. Show all posts

Thursday, October 2, 2014

Dementia in those with Down syndrome now twice as likely

by Christina Finn from thejournal.ie:

THE PREVALENCE OF dementia among people with Down syndrome has almost doubled, according to a new study by Trinity College’s School of Nursing and Midwifery.
Dementia has increased from 15.8% to 29.9%, according to the second wave of the Intellectual Disability Supplement to The Irish Longitudinal Study on Ageing (TILDA).
This is a much higher level than those seen in the general population.

Intellectual disability
Not only has the prevalence almost doubled, but the study, which is the first one in the world to include people with a intellectual disability (ID) into a long term ageing study, shows that the average age of the onset of dementia for people with Down syndrome is 55 years old, with some cases presenting in their 40s.
By comparison, the majority with dementia in the general population are over 65 years of age.
Osteoporosis has also doubled since the first wave of the report three years ago. It has doubled from 8.1% to 16.4% for those with an ID. The study showed that nearly 70% who took part in the health assessment study had poor bone health, which the report states indicates a high level of under diagnosing.
There was also a 50% increase in cataracts.
Other findings show that those with an ID are less like to suffer from other conditions.
Rates of hypertension were more than 50% lower in the ID group (17.5%) than those in the general population (37%).

UC San Diego Launches Unprecedented Down Syndrome Study

from UC San Diego:
To many, Down syndrome (DS) is a childhood condition. But improved health care means that individuals with DS now routinely reach age 50 or 60 years of age, sometimes beyond. However, if they live long enough, people with Down syndrome are almost certain to develop Alzheimer’s disease (AD).
Risk estimates vary, but the National Down Syndrome Society says that nearly 25 percent of individuals with DS over the age of 35 show signs of Alzheimer’s-type dementia, a percentage that dramatically increases with age. Almost all develop dementia by the age of 60.
“The more we learn about Down syndrome and Alzheimer’s disease, the more we realize these conditions – one seen at birth, the other quite late in life – are two sides of the same coin,” said William C. Mobley, MD, PhD, professor and chair of the Department of Neurosciences at UC San Diego School of Medicine. “Autopsies of DS and AD brains reveal virtually identical pathologies – the same telltale amyloid plaques and neurofibrillary tangles.”
Under the auspices of the Alzheimer’s Disease Cooperative Study (ADCS), based at the University of California, San Diego School of Medicine, a new clinical study called the Down Syndrome Biomarker Initiative (DSBI) was launched in March 2013. According to the study’s director, Michael Rafii, MD, PhD – medical director of the ADCS – its aim is to discover indicators of Alzheimer’s and study progression of the disease, with the ultimate goal of better understanding brain aging and AD in adults with Down syndrome.
The three-year pilot study has enrolled 12 participants, aged 30 to 60 years of age. Study participants will be screened for various biomarkers of AD, using tests that include three types of brain scans, retinal amyloid imaging and blood tests, among others.
“Findings to date using MRI and amyloid PET scans indicate that individuals with Down syndrome show the same brain patterns as those in the general population with the earliest stages of the memory-robbing disease, called prodromal AD,” said Rafii. He added that indications of increased brain amyloid deposition – the insoluble protein aggregates found in the brains of patients with AD that are thought to be an underlying cause of the disease – is similar in individuals with DS and those in the general population with AD.
People with amyloid deposition in the brain experience progressive cognitive deterioration. Brain atrophy – shrinking of the brain’s hippocampus – caused by the amyloid buildup, affects routine functional abilities, ultimately leading to complete physical disability.
“By understanding the progression of the disease in people with Down syndrome and those in the general population, we hope discoveries can be made in each group that can be shared between both populations,” said Rafii.
The design of the DSBI pilot study is patterned after the Alzheimer’s Disease Neuroimaging Initiative (ADNI), which began in 2004 to establish neuroimaging and biomarker measures of AD. ADNI tracked the changes taking place in the brains of 800 older people, either free of symptoms or diagnosed with late-stage mild cognitive disorder and early Alzheimer’s disease.
“Our aim is for the Down Syndrome Biomarker Initiative to mirror ADNI’s successes,” Rafii said. “ADNI has helped the international Alzheimer’s research community learn significant lessons about the pathology and biomarkers of AD, which in turn has driven new ways of looking at the disease and new studies that we hope will lead to viable treatments. We are confident we can do the same thing for Down syndrome.”
The 12-subject pilot study at UC San Diego is funded by Janssen Research & Development, LLC. The research is projected to expand into a five-year, 1,000-subject international study.

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Media Contact: Debra Kain, 619-543-6163, ddkain@ucsd.edu; Jeffree Itrich, ADCS, 858-246-1317, jitrich@ucsd.edu

Thursday, August 22, 2013

The link between Alzheimer's disease and Down syndrome

by Maureen Wallace from She Know's: Parenting:
Throughout my life, I've feared I would one day develop Alzheimer's disease. I never expected I'd have a child with Down syndrome whose chances of developing Alzheimer's (AD) surpassed mine.

Individuals with Down syndrome have increased risk of developing Alzheimer's disease and related dementia. The idea terrifies me. If I'm worried I won't be able to take care of myself in my old age, what makes me think I will be able to care for my son?
My 3-year-old son, Charlie, has Down syndrome (Ds), which means while most people have 46 chromosomes, he has 47 — thanks to a third copy of the 21st chromosome.
The 21st chromosome. Remember that.

Ninth Annual Trisomy 21 Symposium

Saturday, March 16, 2013

Down syndrome may hold key to new Alzheimer's treatments

by Julie Steenhuysen from Reuters and The Chicago Tribune:
In a new lead on Alzheimer's research, Johnson & Johnson is bankrolling a three-year pilot study of people with Down syndrome to identify the early changes that herald dementia, which afflicts up to 75 percent of adults with the condition.

The aim is to generate support for a much bigger, public-private partnership funded by drugmakers, advocates and government agencies that will study at least 1,000 people with Down syndrome, tracking them from an early age and eventually testing treatments to keep dementia from developing.
"The study we're proposing would provide insight into treating Alzheimer's, but it might help individuals with Down syndrome as well," said Dr. Husseini Manji, J&J's global head of neuroscience drug development.

Experts in Down syndrome and Alzheimer's who gathered in Chicago for a workshop on the idea at the Alzheimer's Association offices this month say it may offer the best scientific model yet for testing drugs to prevent the degenerative brain disease.

The industry has been repeatedly stung by the failure of experimental Alzheimer's treatments, including recent trials of the J&J and Pfizer Inc therapy bapineuzumab. As a result, companies and researchers are looking for ways to test Alzheimer's drugs earlier, before people's brains become too damaged to benefit.
Studies are already planned to enroll people who carry genetic mutations that ensure they will develop Alzheimer's at an early age. One trial backed by the U.S. Department of Health & Human Services will test a drug from Roche Holding AG's Genentech unit called crenezumab in an extended family from Colombia who carry a mutation that causes them to develop Alzheimer's in their 30s.

Only a few hundred families in the world carry these genes, and there is some worry that drugs tested in people with genetic mutations that cause early-onset Alzheimer's may work differently in people who develop the more common late-onset Alzheimer's, which develops after age 65.

AN IMPORTANT DIFFERENCE

The dementia that develops in people with Down syndrome may bear a stronger resemblance to the disease in the broader population because it differs from other forms of early-onset Alzheimer's, researchers say.

People with Down syndrome inherit a third copy of chromosome 21, giving them an extra helping of a gene that makes amyloid precursor protein, or APP, which is linked with the development of plaques in the brains of Alzheimer's patients.

Most early-onset Alzheimer's is caused by mutations in the APP gene or in one of two genes known as presenilin 1 or presenilin 2. People with Down syndrome appear to develop dementia because of their extra copy of an otherwise normal APP gene.

"There is a possibility that the Down syndrome population mimics (late-onset) Alzheimer's disease a little more closely," said Manji, co-author of a commentary this month in Nature Reviews Drug Discovery that laid out plans for the study.
Dementia starts much earlier in people with Down syndrome, who develop brain plaques and tangles by age 30 and signs of dementia by age 40.
The number of potential Down syndrome patients exceeds those with the genetic mutation. There are some 400,000 people in the United States and 6 million people worldwide with Down syndrome.
"These diseases almost certainly have common features," said Dr. William Mobley of the Down Syndrome Center for Research and Treatment at the University of California, San Diego.

While all of the similarities are not yet clear, studies in mice with Down syndrome show that just eliminating just the extra copy of the gene for APP can keep brain cells from dying, he said.

Mobley's center will run the 12-patient pilot study, which aims to lay the foundation for the larger project, dubbed the Down Syndrome Biomarker Initiative.

The larger trial would be patterned after two successful studies: the Alzheimer's Disease Neuroimaging Initiative, a public-private partnership that helped identify biomarkers linked with Alzheimer's, and a breast cancer trial known as I-SPY that pioneered adaptive trial design, in which researchers use biomarkers to match the right drug to the right patient.

What is not yet known is how many parents of people with Down syndrome would be willing to sign up their adult children for such trials. Michelle Whitten of the Global Down Syndrome Foundation thinks many will be.www.globaldownsyndrome.org/

Friday, February 17, 2012

Down syndrome stem cells used to model Alzheimer’s

The scientists used skin cells donated from healthy volunteers and those with Down’s syndrome and turned them into stem cells. These stem cells were then used to generate networks of functioning nerve cells in the lab, which resemble the complex wiring of cells in the human cerebral cortex. The cortex, which makes up over three quarters of the brain, houses many of the nerve cells involved in memory and thinking and suffers particular damage during Alzheimer’s.

People with Down’s syndrome have an extra copy of chromosome 21, a segment of DNA that carries a gene responsible for producing the Alzheimer’s protein amyloid. Due to this extra version of the gene, people with Down’s syndrome have a much higher incidence of Alzheimer’s than the rest of the population. By generating nerve cells from skin cells of people with Down’s syndrome, the scientists could observe the disease process over a period of weeks and compare this to those cells derived from healthy volunteers.

Dr Rick Livesey, who led the study at the Wellcome Trust and Cancer Research UK Gurdon Institute at the University of Cambridge, said: “One of the biggest challenges facing dementia researchers at the moment is a lack of good ways to track the disease over time. By using stem cells donated from people with Down’s syndrome – who are much more likely to develop Alzheimer’s – we have been able to track how the disease develops over a shorter time period than has been possible in the past.”

Within 28 days, the nerve cells made from people with Down’s syndrome showed more than double the amount of the Alzheimer’s protein amyloid than those from healthy volunteers and this built up into amyloid plaques within two months. The scientists also observed that a protein called tau became abnormally altered and distributed in the cells- one of the common later-stage characteristics of the disease.

Dr Livesey added: “What is promising about this stem cell technique is that we can create functioning human cortex cells in a dish, allowing us to more closely model what is happening in our brains. Not only this, but our new model shows many of the characteristic features of human Alzheimer’s disease and will allow us to test new treatments more easily.”

Dr Simon Ridley, Head of Research at Alzheimer’s Research UK, the UK’s leading dementia research charity, welcomed the findings. He said: “We are pleased to have contributed funding towards this study and we hope it can be used to unravel some of the remaining questions about how Alzheimer’s progresses.

Modelling a complex disease like Alzheimer’s is a big challenge, but innovative approaches like this can improve our understanding. As the stem cells in this study were donated by people with Down’s syndrome, they differ genetically to the rest of the population, but could still offer valuable insight into the disease processes in Alzheimer’s.

“Increasing our understanding of dementia is essential not only for people with Down’s syndrome, but for the 820,000 people across the UK living with the condition. It is essential that we improve the models that we have for understanding dementia, but this can only be done through research. As dementia research is so desperately underfunded, we must invest now if we are to find the answers that are so urgently needed.”

from the University of Cambridge

Wednesday, February 8, 2012

brain imaging study to explain connection between Down syndrome and Alzheimer's

from Varsity Online:

The University has unveiled an ambitious £1 million brain imaging study this week, which is investigating why people with Down syndrome are at such a high risk of developing Alzheimer’s in later life. With the exception of a few rare familial links to the devastating disease, Down syndrome is the only disorder known to correlate so clearly with dementia.

Professor Tony Holland, of the Cambridge Intellectual and Developmental Disabilities Research Group, the organization charged with leading the research, has stated that almost 100% of people with Down syndrome with go on to develop dementia. Even more worryingly for these sufferers and their families is Professor Holland’s assertion that symptoms of dementia often manifest themselves up to 40 years earlier in those who have Down syndrome.

Recent medical advances may mean that those with Down syndrome are living longer and better lives, but this increase in life expectancy is leaving more and more sufferers at the risk of developing dementia, a consequence that Professor Holland described as a ‘poisoned chalice’ for patients. He is now urging those with Down syndrome to come forward as volunteers for this groundbreaking project.

The four-year study, funded through the Medical Research Council in partnership with the Down Syndrome Association intends to examine the role of beta amyloid in the development of Alzheimer’s. Scientists are already aware that people with Down syndrome have more amyloid in their brains and hope that their findings will shed light on the causes of this specific form of dementia for the population as a whole.

Across the UK, 700,000 people are diagnosed with dementia annually at a cost of £17 billion. Dementia is commonly associated with memory loss but is also responsible for changes in mood as well as communication and reasoning problems. The degenerative nature of the disease means that sufferers will inevitably end up needing round the clock care.

With the UK population set to rise to 70 million by 2027, it seems this funding boost has come at just the right time.

Friday, January 13, 2012

Patients with Down Syndrome Not Benefitted by Alzheimer's Drug

from Third Age:

Patients with both Down syndrome and Alzheimer’s disease are not benefitted by a popular drug used to treat cognitive decline, a new study shows. According to HealthDay News, researchers from King’s College London found that the brain function of people older than 40 years with Down syndrome was not helped by taking memantine.

The results came as disappointing to researchers, who had been excited about the positive results found in mice with Down syndrome.

To test the effectiveness of memantine, 88 people with Down syndrome received the drug for one year, while another 85 people received a placebo. Some study participants had Alzheimer’s while some did not.

Overall brain function declined in both groups regardless of whether or not they were taking the drug.
In fact, memantine was not only ineffective, it was dangerous. Eleven percent of people in the group that took the medication experienced serious adverse side effects, compared to just seven percent of people in the placebo group. Five people from the memantine group eventually died of these complications.

Still, researchers were pleased that their work would contribute to the ongoing field of study involving Down syndrome and Alzheimer’s. According to HealthDay, the issue is particularly important as nearly 40 percent of people with Down syndrome will be diagnosed with dementia once they pass the age of 60.

“Memantine is not an effective treatment in this group of patients,” said study author Clive Ballard. “We believe that this robust finding will have implications for clinical practice and research strategy in the future. Specifically, therapies that are beneficial for people with Alzheimer’s disease are not necessarily effective for the treatment of cognitive impairment or dementia in the context of Down syndrome.”

Tuesday, September 6, 2011

Down syndrome and Alzheimer's

from newstimes.com:

Dear Readers,

I would like to stray from our regular pattern of answering one of your emails or letters. The reason for this is a particular topic which many of you brought up during a recent lecture given by geriatrician Marina Blagodatny and psychiatrist Neil P. Dolan at the Trumbull Marriott.

The topic in question was dementia, specifically Alzheimer's dementia in people with Down syndrome. I hope that you do not mind my taking the opportunity to write about it some more.

Let's start with the definition of Down syndrome. It is a genetic disorder affecting one in 733 live births and accounts for about 15 percent of intellectual disabilitiy cases.

Symptoms of the learning problems associated with the syndrome range from mild to moderate, as does the patient's functional capacity. The cause of this disorder is, most of the time, an extra chromosome -- or genetic "building block" -- in our body. Chromosome 21 is the one affected. Normally a person has two copies of each of the chromosomes.

In at least 90 percent of Down syndrome cases, a person has three copies of Chromosome 21. This is not an illness one inherits from a parent, at least not in a majority of cases. The most commonly known risk factor is late maternal age. By age 45, a woman's risk of having a baby with this disorder is one in 35.

Life expectancy of patients with Down syndrome has increased dramatically in the last century. From roughly 10 years in the 1920s, it is now quite normal for those with Down syndrome to enjoy life way beyond age 50.

Unfortunately, there are many problems these patients and their families have to struggle with throughout their lives. People born with the syndrome age faster, and are prone to developing dementia.

It is known that 25 percent of Down syndrome patients have dementia by age 35. They are three to five more times more likely to get dementia as compared to patients the same age not born with the genetic disorder.

What is most perplexing is the fact that, although virtually all adult patients with Down syndrome have specific changes in the brain characteristic of dementia, not all of them have the symptoms.

For those of you who are more scientifically inclined, the changes are called neurofibrillary tangles and amyloid plaques, as well as loss of nerve cells in certain parts of the brain.

It must be clarified that people with other forms of intellectual disability not related to Down syndrome have the same risk and rate for dementia diagnosis as the non-affected population.

Scientists still do not fully understand why this is the case, other than that the Chromosome 21 abnormalities must play a significant role. Alzheimer's disease is the most common form of dementia. It is a progressive memory disorder that affects our ability to reason, remember, use language and think.

The disease can attack quickly and lead to demise in only a few years, or linger over 10 to 15 years, affecting not only the patient but the whole family. Approximately 4 million Americans are affected today by Alzheimer's. Presentation of the disease in patients with Down syndrome is quite different from that in other dementia patients.

Loss of language skills and the ability for self-care are quite common early. Seizures happen often in individuals who did not have them before. Incontinence, personality changes and sleep disruptions are also seen.

Since the mental abilities of patients with Down syndrome vary greatly to begin with, diagnosis of dementia is challenging. Unfortunately, the majority of patients are diagnosed late in the process of the illness. Standardized tests used in non-Down syndrome individuals do not apply to this group.

It is crucial for a specialist involved to have some idea of the person's cognitive baseline. Many advocate for the universal screening of Down syndrome individuals around age 30. There is a questionnaire called "The Dementia Questionnaire for Mentally Retarded Persons," which can help, as well as a few other available tools. As a rule, a specialized dementia center is the best place to follow a patient with Down syndrome and Alzheimer's. The input from the caregivers and the primary care physician are considered absolutely necessary for the proper diagnosis and the meaningful follow-up.

One of the important goals of early diagnosis is to rule out other reasons for cognitive decline and behavioral problems that can be potentially curable. Thyroid problems, excessive medication use, severe depression, and unrecognized hearing and vision problems may all present as similar or identical to Alzheimer's. Even simple infection can trick us.

The treatment of Alzheimer's dementia in Down syndrome patients is uncharted waters. Only one out of four drugs available has been tested in these patients, and no drug is specifically approved by the Food and Drug Administration. Trying them, however, may prove very beneficial, as they may help with behaviors and stabilize functional abilities.

Other medications, like antidepressants, mood stabilizers and anti-anxiety medications, may also play a role when used judiciously and closely supervised and monitored.

There is yet another reality of this particular scenario. Many primary caregivers of Down syndrome patients in their 40s and 50s can be their aging parents and/or siblings. Not only do they have to face their own fears about forgetfulness and their own memory lapses, they are often unable to meaningfully plan ahead.

Legal issues are important, as well as financial ones. There has to be a plan of care in case of a caregiver's illness or, as horrible as it is to think about, death.

I strongly recommend that all involved use their local Alzheimer's Association, as well as local Down syndrome support groups and the National Down Syndrome Society. The help of local religious organizations also can be priceless. Government support is available, albeit limited. All of us professionals involved in diagnosis and treatment of dementia are more than willing to help.

Dr. Beata Skudlarska is a Bridgeport geriatrician. Send questions to Bridgeport Hospital Center for Geriatrics, 95 Armory Road, Stratford CT 06614 or geriatricmd@aol.com.

Thursday, June 16, 2011

More People with Down syndrome developing dementia

from channel4.com:

People with Down's syndrome are living longer than ever before. Since the 1980s their life expectancy has doubled and many now live into their 60s.

But for the estimated 60,000 people with Down's syndrome in the UK this development is coupled with the startling knowledge that people with Down's are significantly more at risk of developing dementia than in the rest of us. Not only that but they also develop it at a much younger age - 30 to 40 years earlier than the general population.

Charlene is just 29 and was diagnosed with dementia six years ago. Her mum has witnessed her change from a fun-loving, karaoke-singing young person to "an old lady". Previously unaware of the high prevalence of dementia in people with Down's, it was a shock for the family to learn that Charlene was developing the disease in her early 20s.

Charlene is unusually young to start developing dementia, but studies do show that the risk increases dramatically with age. By 50, half of people with Down's are at risk of developing dementia, and this, in turn, is presenting a huge burden to families and services.

With an aging Down's population, Diana Kerr, a leading expert in the dual diagnosis at University of Edinburgh, worries that local authorities are not ready to meet the increasing demands of this population - partly because of a false perception that people with Down's don't live long enough.

Often changes in personality are the first sign of dementia, and a diagnosis is crucial for accessing the right services. It also means the family or carers can begin to change their responses and environment to make life more manageable for the person with Down's.

In Bradford, Nicole has Down's syndrome and lives with her sister Jackie. Nicole's changing behaviour is causing the family and her daycare service great strain. At 52 years of age there is a high probability this change in her character is due to the onset of dementia.

Recently has she been allocated a social worker to help begin the diagnostic process with Bradford District Care Trust. Dr Baylis is part of a multi-disciplinary team which will try to diagnose Nicole. She has other health conditions that could mimic dementia-like symptoms, so a clear diagnosis takes some time while other problems are ruled out.

But Diane Kerr insists that many options for carers are not costly and that simply training families and staff "who are supporting people with dementia to get into their world, instead of expecting them to get into our world," could greatly improve the situation for all concerned.

However, the default care option for many people with Down's no longer able to remain at home is to be placed in a care home for the elderly. Diana Kerr's findings warn that this is generally not the best solution. It may be cheaper than specialist dementia care for those with a learning disability, but there is often a lack of understanding among staff about the unique care needs involved.

Down's syndrome facts
- Around one in every 1000 babies born in the UK will have Down's syndrome.
- There are 60,000 people in the UK with the condition. 
- Although the chance of a baby having Down's syndrome is higher for older mothers, more babies with Down's syndrome are born to younger women. 
- Down's syndrome is caused by the presence of an extra chromosome in a baby's cells. It occurs by chance at conception and is irreversible. 
- Down's syndrome is not a disease. People with Down's syndrome are not ill and do not "suffer" from the condition.
- People with the syndrome will have a degree of learning difficulty. However, most people with Down's syndrome will walk and talk and many will read and write, go to ordinary schools and lead fulfilling, semi-independent lives.
- Today the average life expectancy for a person with Down's syndrome is between 50 and 60. A considerable number of people with Down's syndrome live into their 60's.

Information by The Down's Syndrome Association

For Elaine in Cardiff this is something that she worries about daily. She cared for her daughter Mandy until dementia rapidly took hold in her 40s, when the burden on Elaine became too great. She ended up in hospital and Mandy was placed in a local care home.

Elaine visits the care home daily, and pushes to make sure that Mandy receives as much support as she can get. Where she thinks there is a shortfall in the available support, Elaine tries to fill it herself - such as trips to the physiotherapist so Mandy can stand for just a short time every week. Nationally, there are few facilities that cater specifically for those with Down's syndrome and dementia. As more and more people develop dementia, experts warn that, due to a lack of resources, more will end up in homes for the elderly, like Mandy.

While families and services provide varied support to people with Down's syndrome, scientists at Cambridge University are seeking treatments that could prevent future generations getting dementia at all. Professor Tony Holland has recently received funding from the Medical Research Council for a four-year study into a protein called amyloid.

'If we do not do something about the needs of people with Down's as they get older and have developed dementia, they will go back into the institutions that we have spent the last 20, 30 years trying to get them out of.'

People with Down's have unusually high levels of amyloid in the brain, and Professor Holland wants to find out if this is the driving force behind dementia in those with Down's syndrome.

If the study concludes that this protein is the key factor, the next step will be to find treatments that reduce the amount of amyloid being deposited in the brain - and thus attempt to prevent the onset of dementia in the Down's population.

Furthermore, it might offer key insights into the role of amyloid in Alzheimer's disease in the general population.

However until a medical breakthrough can defeat dementia, Diana Kerr fears that recent improvements in quality of life for people with Down's could quickly regress.

"If we do not do something about the needs of people with Down's as they get older and have developed dementia, they will go back into the institutions that we have spent the last 20, 30 years trying to get them out of - long-stay units where they are left in a bed, where they can restrain people with chemicals so they don't have to tie them down.

"And we've gone back 30 years. You know that thing about the mark of a civilised society is the way we support vulnerable people? Well, that would be a pretty big indictment."

Wednesday, June 15, 2011

Diagnosing Dementia In Adults With Down Syndrome

from ivanhoe.com:

A unique brain scanner is used to allow the assessment of amyloid plaques and neurofibrillary tangles- the hallmarks of Alzheimer's disease- in adults with Down syndrome. This finding may offer a new clinical tool to help diagnose dementia in adults with Down syndrome.

Adults with this disorder develop Alzheimer's-like plaque and tangle deposits early, often before the age of 40. Previously, the only way to physically detect these abnormal proteins in this population was through an autopsy.

Over the last decade, methods for identifying and imaging the neuropathology of Alzheimer's disease in living patients have been developed. UCLA researchers have created a chemical marker called FDDNP that binds to both plaque and tangle deposits, which can then be viewed through a positron emission tomography (PET) brain scan, providing a "window into the brain." Using this method, researchers are able to pinpoint where in the brain these abnormal protein deposits are accumulating.

"Early detection can also lead to earlier interventions and treatments, often before symptoms begin."
For this study, researchers intravenously administered FDDNP and then performed PET brain scans on 19 non-demented adults with Down syndrome (average age 37), 10 healthy controls (average age 43) and 10 patients with Alzheimer's disease (average age 66).

The results showed significantly higher binding levels of FDDNP in participants with Down syndrome in all brain regions, when compared to healthy controls. Compared with Alzheimer's disease patients, subjects with Down syndrome showed significantly higher binding levels in the parietal and frontal regions — areas involved in memory, behavior and reasoning.

"The higher level of plaques and tangles may be reflecting the early and extensive accumulation of these deposits seen in individuals with Down syndrome," Dr. Small said.

The researchers also discovered significant associations between increased age in those with Down syndrome and higher FDDNP binding values in the parietal, lateral temporal and frontal regions.

"This is one of the first times we've been able to visualize the neuropathology occurring in the living brains of adults with Down syndrome," study author Dr. Jorge R. Barrio, a professor of molecular and medical pharmacology at the David Geffen School of Medicine at UCLA who holds UCLA's Plott Chair in Gerontology, was quoted as saying.

"The age-related patterns and regional distribution of the plaques and tangles were consistent with the types of deposits that could only be identified previously through an autopsy."
The areas of accumulation were consistent with earlier autopsy study findings, which had shown that while plaque and tangle pathologies are the same in both Down syndrome and Alzheimer's disease, the deposit patterns are different.

Autopsy studies have also shown that all adults with Down syndrome eventually develop these accumulations of amyloid plaques and tau tangles. But rather than experiencing memory decline and other cognitive losses, as is common with Alzheimer's, aging Down syndrome patients tend to develop behavioral problems.

"We found that the behavioral changes in the subjects with Down syndrome correlated with neurological changes in related areas of the brain consistent with the level of FDDNP binding levels to the abnormal proteins," Dr. Small said.

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