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Archive for September, 2009

Isobel Jeffrey The AlzheimerA 13-year-old girl is suffering from Alzheimer’s-like dementia after developing an extremely rare form of the disorder.

Isobel Jeffery displays the same symptoms as some victims in their 70s, and can no longer feed or dress herself or walk or talk properly. She becomes easily confused and suffers memory loss, nightmares and hallucinations. Isobel was diagnosed with early onset dementia aged nine after she began to slur her words, lose the ability to swallow and became unsteady on her feet.

She now needs 24-hour care and will never develop the basic day-to-day skills to look after herself.

Child dementia is extremely rare and affects about one in 12million – about 500 worldwide. It is sometimes treatable, but although there are 100 different types of dementia, including Alzheimer’s, doctors say Isobel’s condition has not responded to any medication.

Her mother and full-time carer, Nicola, 39, says her daughter has lost understanding of the world around her as her mind has gradually shut down. But she described her as a ‘rare cookie’ who retains a sense of humour and goes about her life with enthusiasm.

The mother of two from Exeter said: “When she was nine she suddenly started slurring her words and was less clear in her talking. It sounded like she was drunk.”

“Now it is a relentless loss of skills and mobility. We’ve been told that she will slowly deteriorate. She has undergone extensive tests but the outcome is always the same.”

“The prognosis is she will get gradually worse.” She added: “The cruel thing about dementia is that she is semi-aware of the fact that she is losing her skills.”

“Despite all this she is one of the most vibrant people I have ever known, with a wicked sense of humour and enthusiasm about life.”

After her diagnosis Isobel gradually lost the ability to concentrate and her conversation became ‘fixed’ and ‘rigid’.

Doctors have said that the condition will eventually rob her of her ability to walk, speak and even communicate with her family at all. She has been given just ten to 15 years to live – meaning she could be dead before her 30th birthday.

Dementia is a degenerative and progressive disease which can affect all areas of mental and physical functions, not just memory. Diagnosis before the age of 65 is considered as early onset. For Isobel it has meant learning difficulties, impaired memory and sensory processing problems.

Isobel lives with her mother, father Keven, 39, a nuclear safety engineer, and sister, Katie, eight.

Mrs Jeffery said: “Two years ago we took the painful decision to have an operation to enable her to be tube fed directly into her stomach because she was no longer able to swallow.”

“We don’t know why it is happening. Izzie effectively has Alzheimer’s although she is only 13. But she is a rare cookie and really has made every attempt to live her life to the full.”

Terry Roberts, of the Alzheimer’s Society, said: “This is a very sad state of affairs which re-emphasizes how important support is for the young person and the carers.”

Mrs Jeffery is taking part in a four-day, 370-mile bike ride from London to Paris to raise awareness of her daughter’s condition and funds for research. It begins on September 16.

To sponsor her, visit http://www.justgiving.com/nicolajeffery2.

(Source: The Daily Mail, September 12, 2009 – http://www.dailymail.co.uk/health/article-1212904/The-girl-13-whos-dementia-nine.html?printingPage=true)

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Doctors have identified the following list of conditions can cause dementia or dementia-like symptoms. Many of these conditions are reversible with appropriate treatment.

(1) Reactions to Medications.
Medications can sometimes lead to reactions or side effects that mimic dementia. These dementia-like effects can occur in reaction to just one drug or they can result from drug interactions. They may have a rapid onset or they may develop slowly over time.

(2) Metabolic Problems and Endocrine Abnormalities
Thyroid problems can lead to apathy, depression, or dementia. Hypoglycemia, a condition in which there is not enough sugar in the bloodstream, can cause confusion or personality changes. Too little or too much sodium or calcium can also trigger mental changes. Some people have an impaired ability to absorb vitamin B12, which creates a condition called pernicious anemia that can cause personality changes, irritability, or depression. Tests can determine if any of these problems are present.

(3) Nutritional Deficiencies
Deficiencies of thiamine (vitamin B1) frequently result from chronic alcoholism and can seriously impair mental abilities, in particular memories of recent events. Severe deficiency of vitamin B6 can cause a neurological illness called pellagra that may include dementia. Deficiencies of vitamin B12 also have been linked to dementia in some cases. Dehydration can also cause mental impairment that can resemble dementia.

(4) Infections
Many infections can cause neurological symptoms, including confusion or delirium, due to fever or other side effects of the body’s fight to overcome the infection. Meningitis and encephalitis, which are infections of the brain or the membrane that covers it, can cause confusion, sudden severe dementia, withdrawal from social interaction, impaired judgment, or memory loss. Untreated syphilis also can damage the nervous system and cause dementia. In rare cases, Lyme disease can cause memory or thinking difficulties. People in the advanced stages of AIDS also may develop a form of dementia. People with compromised immune systems, such as those with leukemia and AIDS, may also develop an infection called progressive multifocal leukoencephalopathy (PML). PML is caused by a common human polyomavirus, JC virus, and leads to damage or destruction of the myelin sheath that covers nerve cells. PML can lead to confusion, difficulty with thinking or speaking, and other mental problems.

(5) Subdural Hematomas
Subdural hematomas, or bleeding between the brain’s surface and its outer covering (the dura), can cause dementia-like symptoms and changes in mental function.

(6) Poisoning
Exposure to lead, other heavy metals, or other poisonous substances can lead to symptoms of dementia. These symptoms may or may not resolve after treatment, depending on how badly the brain is damaged. People who have abused substances such as alcohol and recreational drugs sometimes display signs of dementia even after the substance abuse has ended. This condition is known as substance-induced persisting dementia.

(7) Brain Tumours
In rare cases, people with brain tumours may develop dementia because of damage to their brains. Symptoms may include changes in personality, psychotic episodes, or problems with speech, language, thinking, and memory.

(8) Anoxia
Anoxia and a related term, hypoxia, are often used interchangeably to describe a state in which there is a diminished supply of oxygen to an organ’s tissues. Anoxia may be caused by many different problems, including heart attack, heart surgery, severe asthma, smoke or carbon monoxide inhalation, high-altitude exposure, strangulation, or an overdose of anesthesia. In severe cases of anoxia the patient may be in a stupor or a coma for periods ranging from hours to days, weeks, or months. Recovery depends on the severity of the oxygen deprivation. As recovery proceeds, a variety of psychological and neurological abnormalities, such as dementia or psychosis, may occur. The person also may experience confusion, personality changes, hallucinations, or memory loss.

(9) Heart and Lung Problems
The brain requires a high level of oxygen in order to carry out its normal functions. Therefore, problems such as chronic lung disease or heart problems that prevent the brain from receiving adequate oxygen can starve brain cells and lead to the symptoms of dementia.

(B) WHAT CONDITIONS ARE NOT DEMENTIA?

(1) Mild Cognitive Impairment (MCI)
Some people develop cognitive and memory problems that are not severe enough to be diagnosed as dementia but are more pronounced than the cognitive changes associated with normal aging. This condition is called mild cognitive impairment. Although many patients with this condition later develop dementia, some do not. Many researchers are studying mild cognitive impairment to find ways to treat it or prevent it from progressing to dementia.

(2) Age-Related Cognitive Decline
As people age, they usually experience slower information processing and mild memory impairment. In addition, their brains frequently decrease in volume and some nerve cells, or neurons, are lost. These changes, called age-related cognitive decline, are normal and are not considered signs of dementia.

(3) Depression
People with depression are frequently passive or unresponsive, and they may appear slow, confused, or forgetful. Other emotional problems can also cause symptoms that sometimes mimic dementia.

(4) Delirium
Delirium is characterized by confusion and rapidly altering mental states. The person may also be disoriented, drowsy, or incoherent, and may exhibit personality changes. Delirium is usually caused by a treatable physical or psychiatric illness, such as poisoning or infections. Patients with delirium often, though not always, make a full recovery after their underlying illness is treated.

(Source: U.S. National Institutes of Health.)

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Cleaner Teeth cleaner BrainA major research grant will help scientists study the links between memory and oral health in a bid to establish control over Alzheimer’s disease. The news of a $1.3 million study to be carried out on thousands of participants has been welcomed by the UK’s leading oral health charity – the British Dental Health Foundation.

Research has long associated oral health with overall health problems, including dementia – though no studies have made clear how the state of the teeth and mouth affect mental function.

Last year researchers found a link between mild memory loss and gum disease* and the seven-figure grant will enable examination of medical records of thousands of Americans to further pursue the link.

Foundation chief executive Dr Nigel Carter welcomed the news saying: “Oral health and gum disease in particular has been increasingly linked to overall health through studies such as this exciting piece of research.

“The recent review of NHS dentistry suggested the government is committed to preventive care.

“It would be a great boost if scientists could prove preventive treatment could not only protect our teeth but also help prevent chronic diseases such as Alzheimer’s.

“We will watch developments with interest and urge the public to make sure they look after their oral health with simple steps which can reduce the risk of gum disease.

“Minimizing the risks caused by poor oral hygiene is as simple as brushing teeth twice a day with a fluoride toothpaste and visiting the dentist regularly for professional check-ups.”

The US studies will be led by Dr Bei Wu of the University of North Carolina and Dr Richard Crout of the University of West Virginia, who will continue with a program testing oral health and memory in 273 people aged 70.

The new funding will establish larger studies looking for links between oral health and brain function over time, while scientists will also seek to establish a link between improved cognitive function and better oral hygiene through intervention to improve oral health.

In an interview this week Dr Crout, who has predicted that dentists may in future be in a position to administer memory tests on older patients, said: “to have overall good general health you need to have good oral health.”

(Source: http://www.alzheimersweekly.com/Prevention/cleaner-teeth-cleaner-brain-a592.html)

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THE MOMENT OF TRUTH

He placed the cold stethoscope back into his starched white coat. “I’ll be back shortly,” he said as he exited the exam room.

Once in the narrow corridor, he slowed. His patient, Richard, not only had developed enlarged neck nodes despite radiation therapy to that area, but now had new ones in his axillary and groin regions. Amid 20 minutes of cheerful conversation, it took seconds to discover that Richard was a terminal patient. Yet, he had left him in the room the way he had done it so many times before: the reassuring smile and the gentle closing of the door. He heard Richard’s muffled voice, “I think Carol wants to speak with you.”

He reached the waiting room, took a deep breath, and entered. There was nothing in the room to suggest that it was a physician’s office. The chairs were natural wicker with floral upholstery; several original Calder paintings hung on the walls, and an antique table sat in the middle of the room covered with Gourmet, Arts & Antiques, and Esquire magazines. Only the sliding opaque glass panel shielding the receptionist suggested that an entirely different world existed behind this facade.

She was sitting in the corner. He was glad that it was lunch time and the room was empty, given that she tended to be so emotional when they discussed her husband’s condition. She was 10 years younger than her husband and very much an artist: black hair pulled back in a bun and long gold earrings. Her mouth was a striking red against the absence of all other makeup, her blouse was a colorful South American weave, and her skirt full-length denim. He looked for sandals, but she wore simple, flat black shoes.

He was not certain if any of her paintings had ever sold, but in the years he had been caring for her husband, she was always between paintings; abstracts, she had told him, though she had never shown him any of her work, not even a photograph. He knew that she detested the Calders in the waiting room.

She immediately got up and kissed him on the cheek. “Thanks for coming out to talk with me,” she said, as if it were the first time. She was tall, and her eyes were level with his; green eyes that seemed to penetrate through his professional veneer.

“Did he ask you to come out to see me? You know how he likes to have everything told only to him; then he can pass out selected bits of information to everyone else.”

“Well, he did say that you might want to see me. He’s dressing now.”

“You are going to tell him, aren’t you?” she asked, squeezing his arm.

“Tell him what?” he asked, feigning innocence. How could she know what he had only discovered minutes ago?

“That he is dying.”

He stepped back suddenly, as if the words had struck him physically. He quickly recovered. “He’s been slowly dying for 2 years from his lymphoma-why tell him now? Besides, surely he knows.”

“If he knows, why is he doing crazy things? Since he completed his radiation last week, he has arranged for a cruise to the Caribbean next month, and he is out looking to buy a new house-after 25 years, he wants to move.”

He was puzzled by her surprise: didn’t she understand that that’s how the dying behave?

“That’s not unusual behavior,” he said, trying to be reassuring.

“Really? What about the fact that he wants to write several books; one’s a new translation of Thucydides and the other is a historical novel of a Greek boy in an Odysseus-like adventure. He’s already working overtime in his library, and yesterday he asked his agent for an advance on his novel. Tell him, John. Please tell him.”

He liked it when she used his first name. He managed to smile. “Look, he got through radiation; he may have another 6 months.”

“He’s acting like he has another 10 years.” She released his arm and slumped into her chair. She was crying. He hesitated. Then he sat down beside her and gently put his arm around her, comforting her. He could feel her shaking, and tears ran down her cheeks. He gave her his handkerchief. She took it and wiped her face. The tears continued.

“He might as well see me cry. He never has, you know. I’ve been afraid to do it in front of him. And he never says anything about not making it—not even a doubt. He directs all of his energy into his next project and the next . . . I can’t stand it.”

She jumped up and was standing over him. “Please tell him, please.”

He stood up slowly. He was pulled between her plight and her husband’s immutable will to live. The truth would crush his hope and plunge him into depression.

She handed him back his handkerchief. She had stopped crying.

“Please,” she said.

“All right, all right. I’ll go in and tell him,” he said as he closed the door behind him, not looking back. An avalanche of thoughts tumbled over him, his gait slowing to a shuffle: how had this journey from life to death suddenly gone awry? He had been there for Richard all this time, as a physician and an intellectual peer, and now what?

When he reached the door only the image of her pleading, tears streaming down her cheeks, remained. He turned the knob and entered.

He found Richard sitting in the large chair in the examining room, completely dressed: a loose tweed jacket, button-down shirt, gray trousers, and cordovan loafers. His hair had regrown into tight browncurls. His eyes were also brown, always with an intense stare. He still looked well despite all his previous treatment. However, anyone who knew him could appreciate that his usual full, youthful face was now more gaunt and his muscles were losing tone. Still, sitting there, he looked the full professor of Greek at the university.

“Did you satisfy Carol’s queries? She’s really been on edge lately,” Richard said nonchalantly.

“I did my best,” he said, trying to match Richard’s demeanor. He could feel his voice tremble. He wondered if Richard could sense his ambivalence.

He was always amazed that someone of Richard’s intellect could ask so little about his illness-the diagnosis, yes, and the treatment, of course, but never the ultimate prognosis. He had seen patients with much less education come into his office and ask more probing questions; they would even bring in pertinent Internet articles about new treatments. Richard was not indifferent. He was intense about everything, but his illness was just one of the many facets of his life that he was dealing with at the time. Actually, talking with Richard about anything non medical was the most exciting conversation he could anticipate on any day. He always looked forward to talking with him. Not today. Not now.

He had learned through years of caring for cancer patients never to present the brutal facts. Besides, in Richard’s case, there was initially plenty of optimism; at the onset, he had a 50% chance of cure. He tolerated immunochemotherapy despite nausea and vomiting, bouts of hair loss, and episodes of infection requiring admissions to the hospital. He took leave of absence from the university, and when his disease was in remission, he immediately returned to work. He was never jubilant about his remission, and when he relapsed, he barely expressed any emotion.

A month ago, he had asked Richard’s wife over the phone, “What did he say to you after I told him that he had relapsed?” At that time, Richard’s enlarged neck nodes had recurred despite immunochemotherap.

“Nothing, nothing. He said it was unfortunate, but you thought that local radiation would help.”

“I told him radiation was only for relief of symptoms. His neck was painful.”

“He never said that.”

“Maybe he’s protecting you,” he had told her.

“I doubt that. He never protects my feelings when it comes to anything.”

“There isn’t any option now but radiation.”

“Tell that to Richard, not me,” she said as she hung up.

The memory of that phone call stuck with him now as he turned and looked out the window. The sky was a bright blue. The silence was palpable. He had to tell Richard his life was ending. He needed a cue.

He turned back to Richard. “Carol says you’re planning to resume writing.”

“Not resume, continue. I have two manuscripts that I’ve been working on for a while.” He seemed genuinely excited. “You know that I’ve always been interested in Thucydides. I’ve decided to do my own translation. I’ve been working on it. I could probably finish it in the next few years. There are actually full paragraphs that have different meanings than was originally thought. It’s tedious but full of potential.”

He could see Richard in the classroom, pacing, exciting the students with his belief in the yet undiscovered.

“And I always wanted to do a historical novel,” he continued.

“About the Greeks; I’ll use Odysseus as my main character: the ultimate physical specimen against the unknown.”

“Are you going to take any more disability leave from the university to work with your books?”

“No, I’ll be working and writing.”

He sat down on the stool opposite Richard. “How do you see the situation, Richard?” He gripped the stool tightly.

“How do you mean?”

The intercom buzzed. A rasping voice announced: “The pathologist is on line two; he needs to talk with you.”

“Tell him I’m doing a procedure, and I’ll call him back,” he yelled, twisting his head toward the intercom.

He turned back to Richard. “Well, now that you’ve finished your radiation treatment, what do you think will happen?”

Richard got up deliberately and turned to face him. There were tears in his eyes. “I’m going to die, you fool.” He was suddenly out the door.

Through the smoked glass of the waiting room he could see Richard and his wife embrace. Then they were gone.

The intercom buzzed again. “Doctor, you have another call on line four.” He stared out the window. A few clouds had appeared, but the sky was still blue.

“Doctor, you have a call on line four. Did you hear me?”

He did not move. Outside, he heard a motor start. Perhaps it was Richard and his wife. He rose slowly to answer the phone. His legs were weary.

(Source: By Michael J. Messer – The author(s) indicated no potential conflicts of interest – link at: http://jco.ascopubs.org/cgi/reprint/27/22/3723)

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Could lifestyle factors and behaviours affect the chances that a breast cancer survivor would develop cancer in the other breast?

A study analyzed data on 1,091 women, 40 to 79 years old, who had been treated successfully for breast cancer, including 365 who subsequently developed a new cancer in their other breast.

Women who were considered obese (with a body mass index of 30 or higher) when first diagnosed were 40% more likely than non-obese women to develop a second cancer. Those who consumed one or more alcoholic drinks a day were 70% more likely than no-drinkers to have a second cancer. The likelihood was about doubled among women who smoked, compared with those who had never smoked. Those who smoked and also had one or more alcoholic drinks daily had a seven-fold greater risk for cancer in other breast than did women who smoked or drank less.

Who may be affected? Breast cancer survivors, who have a considerably greater chance of developing a tumor in their other breast than the average woman who does of getting an initial breast cancer diagnosis.

Some of the lifestyle data came from the women’s responses to questionnaires. The first breast cancer for all participants was oestrogen receptor-positive; whether the findings would apply to women with oestrogen receptor-negative tumors was not tested.

(Source: Journal of Clinical Oncology, September 8, 2009)

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Two new blood tests could help doctors detect colon and stomach cancers simply, cheaply and early without the need for invasive procedures or unpleasant examinations, researchers said on Monday.

The tests, one developed by the Belgian biotech firm OncoMethylome and another by scientists in Germany, use blood samples to detect specific genetic signals of the disease and could help predict whether it is likely to spread.

Ernst Kuipers, a specialist in bowel cancer at Rotterdam’s Erasmus University, who was not involved in the research, said the new tests marked a promising advance in the field of developing more convenient screening.

“The blood sample can be taken by nurses or primary care doctors without the need for special equipment or training,” Joost Louwagie of OncoMethylome said.

Ulrike Stein, who presented her findings with Louwagie’s at the ECCO-ESMO European cancer congress in Berlin, said hers was the first test to be able to detect signals of a specific gene, called S100A4 and known to be linked to cancer, in the blood.

Stein’s test finds various types of cancer, including colorectal and gastric cancers, and had also shown potential in identifying patients whose cancer was likely to spread.

“Cancer patients have significantly higher levels of this S100A4 gene than people without cancer,” she said. “Being able to detect this gene in the blood of the patient, you can monitor the disease course and you can continue to monitor it over several years and throughout various treatments.”

Colorectal cancer effects around one in every 17 people and is the second leading cause of cancer death in the United States and Europe, where a total of 560,000 people develop the disease each year, and 250,000 die from it.

Deaths can be reduced if the cancer is diagnosed early, when it is most treatable.

Although current tests such as a colonoscopy internal examination or the analysis of stool samples are effective, they can be invasive, expensive and unpleasant.

Stein and colleagues from the ECRC Charite University of Medicine and the Max-Delbrueck-Center for Molecular Medicine in Berlin looked at daily blood samples from 185 colon cancer patients, 190 with rectal cancer and 91 gastric cancer patients. They also analyzed blood from 51 tumor-free volunteers.

They found a signal of the gene at significantly higher levels in those with the cancers. There were even higher levels in patients whose cancer had spread.

Louwagie’s team collected blood before surgery from 193 patients known to have colorectal cancer, and from 688 people being screened using a colonoscopy internal examination.

They looked for two so-called methylation genes, SYNE1 and FOXE1, known to be linked to the formation of tumors, and found high levels in colorectal cancer patients, Louwagie said.

OncoMethylome said in August it was in advanced talks with several large companies over licensing rights to its colorectal test.

(Source: Reuters, September 21, 2009)

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Problems carrying out daily chores or enjoying hobbies could predict which people with “mild cognitive impairment” will progress more quickly to Alzheimer’s dementia, U.S. researchers report.

According to the Alzheimer’s Association, mild cognitive impairment (MCI) is “a condition in which a person has problems with memory, language, or another mental function severe enough to be noticeable to other people and to show up on tests, but not serious enough to interfere with daily life.” This type of mental state is considered a risk factor for dementia.

In fact, some studies have found that about 10 percent to 15 percent of those with MCI will progress to dementia each year, according to background information in the new study.

Reporting in the September issue of the Archives of Neurology, the researchers sought to determine if there were telltale signs within MCI that might spot those people who would progress more rapidly to full-blown dementia. To do so, they collected data on 111 people with mild cognitive impairment, then evaluated these individuals using brain scans and cognition tests.

Over the next two years of follow-up, 28 people did go on to develop dementia.

“On their own, the tests did not predict which patients went on to develop dementia,” said lead researcher Sarah Tomaszewski Farias, an Associate Professor of Neurology at the University of California, Davis.

“However, level of daily function was a key predictor,” Farias said.

“So, if an older adult is starting to display problems in daily life, such as problems shopping independently, problems managing their own finances, problems performing household chores, and problems maintaining their hobbies, they are more likely to develop a dementia within several years,” she said.

Farias cautioned that the study involved people visiting a clinic because they were already having memory and other problems, so the implications could be different among the general population of older adults.

“If you look at individuals in the community, you see a much slower progression to dementia in those with some mild cognitive impairment,” she said. “The time to develop dementia once someone has mild cognitive impairment is probably slower in the general population of older adults than we had previously thought.”

Still, any kind of early warning is helpful, and Farias believes health-care providers should ask patients and those who know them well – a spouse or adult child – about how they are doing in their daily lives.

“It is important to keep in mind that sometimes individuals themselves lack awareness of some of these problems. So it is important, if at all possible, to get feedback from individuals who are familiar with how the older adult is functioning in their daily life,” she added.

“If there is evidence or suspicion that an older adult has some mild cognitive or memory problems, and it is starting to interfere with their ability to do daily activities, there is a higher likelihood this individual is developing a dementia and they should be closely monitored,” Farias said.

Greg M. Cole, a Neuroscientist at the Greater Los Angeles VA Healthcare System and Associate Director of the Alzheimer’s Center at UCLA David Geffen School of Medicine, said it is crucial to be able to identify people with early Alzheimer’s disease, “if we want to test methods of preventing it.”

“This study illustrates the difficulties in early diagnosis of Alzheimer’s disease in aging people with mild problems with memory and cognition,” Cole said.

“In my view, because memory and cognitive performance vary widely in our population no matter what age, the best indications of ongoing decline are going to be seen against past individual performance rather than some cross-sectional ‘normalized’ standard,” he said.

Dr. Ronald C. Petersen, Director of the Alzheimer’s Disease Research Center at the Mayo Clinic in Rochester, Minn., agreed that, despite the lack of effective treatments, spotting Alzheimer’s disease early remains important.

“If people in the family start to recognize a change in memory/learning patterns, that might be sufficient to identify someone who could develop Alzheimer’s disease,” Peterson said. “Don’t wait until the person is having trouble driving, is having trouble paying their bills or having trouble functioning in the community – that’s dementia,” he said. “This study tells us that we can identify important symptoms earlier and it may be worthwhile doing so.”

(Source: HealthDay, September 17, 2009)

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