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Carl T. Hall, Chronicle Science Writer Dr. Richard Olney, a San Francisco neurologist and pioneer in ALS research, has launched an ambitious clinical trial testing whether two established drugs used in AIDS and cancer might also help some ALS patients. It's a trial that Olney designed and expected to lead as head of a specialized clinic at UCSF for people with ALS, or amyotrophic lateral sclerosis, popularly known as Lou Gehrig's disease. Those plans have changed. For this trial, Olney is participating not as a doctor, but as one of 60 patients. In fact, he made it a point to be the first one to sign up. He went through the initial round of evaluations last week. He expects to be given either the AIDS drug, a protease inhibitor called ritonavir; or the cancer drug, hydroxyurea; or a placebo, starting in about two weeks. Olney, 56, is regarded to be among the country's top clinical investigators of ALS, having spent most of his career conducting trials and working with patients. In June, he was diagnosed with the same disease he has spent a lifetime studying. It's a twist of fate that has shocked the close-knit ALS world. The case also underscores the challenges posed by one of the deadliest -- and fastest- moving -- disorders of the nervous system. ©2005 San Francisco Chronicle
Carl T. Hall, Chronicle Science Writer Dr. Richard Olney first got interested in the neuromuscular disease known as amyotrophic lateral sclerosis, or ALS, when the daughter of his favorite teacher in junior high school died of it. He soon came to regard ALS, also known as Lou Gehrig's disease, as one of the most compelling -- and justly feared -- disorders of the nervous system. And so, during 25 years as a neurologist, Olney devoted his career to the care of ALS patients, founding a specialized clinic at UCSF. He resigned as clinic director this summer and has stopped treating all his patients with ALS. But he has even more reason these days to be interested in their disease. Now, it is his disease. In June, Olney, 56, regarded as one of the top ALS clinicians in the country, was diagnosed with ALS. ALS is not contagious. He didn't catch it from close contact with a patient. It's just a matter of unhappy fortune -- just one more case in one of the biggest ongoing mysteries of brain research. ©2004 San Francisco Chronicle
Michael Hopkin The British scientist who created Dolly the sheep has applied for a licence to clone human embryos in the search for treatments for motor neuron disease. If approved, the research should give experts a far better picture of what happens to the dying brain cells that characterize this condition. Ian Wilmut, of the Roslin Institute in Edinburgh, and his colleagues submitted their proposal today to the UK Human Fertilisation and Embryology Authority (HFEA). They hope to receive a decision early next year and could potentially begin cloning around April 2005. Motor neuron disease, a range of related conditions including amyotrophic lateral sclerosis, also known as Lou Gehrig's disease, causes around 1,000 deaths in Britain every year. The brain cells that govern movement gradually die off, leaving sufferers paralysed but, usually, with their intellect intact. The causes are still poorly understood, says Wilmut. Around 2% of cases are linked to mutations in a gene called SOD1, although experts are still unclear about how this triggers disease. And a further 8% of patients inherit their disease and therefore presumably have some other genetic predisposition. ©2004 Nature Publishing Group
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 7: Life-Span Development of the Brain and Behavior
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 13: Memory, Learning, and Development
Link ID: 6166 - Posted: 06.24.2010
Chemical cause sought for damage to soldiers' nerves. EMILY SINGER Men who have served in the US military are 60% more likely to develop a fatal muscle-wasting disease than civilians, research suggests. The study hints that all military personnel are at increased risk, not just those who served in the first Gulf War. The nerve disorder, known as amyotrophic lateral sclerosis (ALS) or Lou Gehrig's disease, is a relatively rare disease that causes nerve-cell death and muscle wasting. Studies have suggested that the risk of ALS is raised among Gulf War veterans. So Marc Weisskopf from the Harvard School of Public Health, Boston, and colleagues decided to see if military service in other conflicts had a similar effect. The researchers studied the causes of death of around 400,000 men over a nine-year period. Some 217 veterans developed ALS compared with just 63 civilians, researchers revealed at the American Academy of Neurology meeting in San Francisco this week. © Nature News Service / Macmillan Magazines Ltd 2004
By Carol Cruzan Morton, Globe Correspondent More than 50 years ago, US Navy doctors stationed on the Pacific island of Guam found a shocking rate of an unknown neurodegenerative disease with the fatal progressive paralysis of Lou Gehrig's disease, the tremors of Parkinson's, and the forgetfulness of dementia. Guam's indigenous Chamorro people were 50 times to 100 times more likely to suffer the symptoms of amyotrophic lateral sclerosis, also known as Lou Gehrig's disease and ALS, than populations just about anyplace else on the planet. In one village, more than a quarter of the adults who died between 1944 and 1953 were the victims of this mysterious combination of brain illnesses. Hundreds of research papers since have investigated and dismissed a variety of suspected causes, including microbes, genes, mineral deficiencies and nerve toxins. © Copyright 2003 Globe Newspaper Company.
Veterans of the 1991 Persian Gulf War may be more than twice as likely to be stricken with Lou Gehrig's disease than members of the general population are, new findings suggest. What is more, the condition often strikes vets sooner than those who did not serve, according to the results of two studies published in the current issue of the journal Neurology. Some soldiers who completed tours of duty during the first Gulf conflict complained of ill health after their return home. Gulf War syndrome, which lists fatigue, joint pain, depression, balance problems and diarrhea among its symptoms, was first diagnosed in 1997 and is said to be linked to exposure to toxic chemicals. That same year, Robert Haley of the University of Texas Southwestern Medical Center began investigating a potential connection between time served in the Persian Gulf and Lou Gehrig's disease--amyotrophic lateral sclerosis (ALS)--after receiving a request from a 35-year-old veteran suffering from the fatal affliction. The researchers located 17 veterans under the age of 45 who were diagnosed with ALS between 1991 and 1998. Of these individuals, five were diagnosed in 1998--in contrast to the 1.38 cases expected for an age group of this size. "The increasing slope of the epidemic curve beginning three years after the Gulf War and still increasing in 1998 further supports a true excess. © 1996-2003 Scientific American, Inc. All rights reserved.
Nathan Seppa Scientists' best efforts have failed to vanquish amyotrophic lateral sclerosis (ALS). There was no cure for the nerve-degenerating disease when it struck down baseball star Lou Gehrig 64 years ago, and there is none today. In fact, scientists have yet to pinpoint a cause of the disease except in individuals with certain rare genetic mutations. In the August Nature Genetics , researchers report on other, more common genetic variations that crop up in ALS patients more often than they do in healthy people. Experiments show that similar genetic variations leave mice vulnerable to the sort of nerve degeneration seen in ALS patients, says Peter Carmeliet of Leuven University in Belgium. He and his colleagues compared genetic profiles of 750 ALS patients with those of 1,219 healthy people of similar age in Belgium, Sweden, and Great Britain. The people with ALS were nearly twice as likely to have one of two variant forms of a gene for the protein called vascular endothelial growth factor (VEGF). Copyright ©2003 Science Service.
LeRoy Hunter says in his family, falling down was a serious concern By Tyler Vincent, The Journal-Standard FREEPORT - Growing up with a mother afflicted with amyotrophic lateral sclerosis, or Lou Gehrig's disease, is bound to have an effect on a young man's outlook on life. Certainly that was the case for Freeport resident LeRoy Hunter, who reflects on those memories in his home office, in front of his walker that he has used for the last two years. Hunter, who was diagnosed with ALS in 1995, will be appearing on the Rockford edition of the Jerry Lewis Labor Day Muscular Dystrophy Association Telethon, which began Sunday night and runs through Labor Day. Copyright © 1995 - 2002 PowerOne Media, Inc. All Rights Reserved.
Copyright © 2002 AP Online By MALCOLM RITTER, AP Science Writer An ordinary antibiotic slowed the progression of Lou Gehrig's disease in mice, suggesting a potential new approach for treating people, researchers report. The disease, formally known as amyotrophic lateral sclerosis or ALS, attacks nerve cells that control movement. As these cells degenerate, an affected person becomes progressively paralyzed. Most cases appear between the ages of 40 and 70, and death follows an average of four years after symptoms appear. The antibiotic, minocycline, was shown recently to prolong the lives of mice with a version of Huntington's disease, another neurodegenerative disorder. It is now being tested against Huntington's in people. Copyright © 2001 Nando Media
It is one of the most difficult diseases I deal with, without question, a neurologist says. By Kathleen O?Dell News-Leader The disease already had its deadly grip on respiratory therapist Jeff Taylor when the first symptom showed up: His right-hand grip was weak. Then his arm. Then his left hand; his left arm. Surgeon Mark Avery began slurring his speech; his tongue felt thick. By the time the Springfield men were diagnosed with ALS, amyotrophic lateral sclerosis, it was already too late. The disease gradually took away their strength, then their movement and, finally, their lives. ALS, also known as Lou Gehrigs disease, is a fatal disease that researchers are scrambling to understand even as the number of cases seem to be growing in Missouri and nationwide, said Kim Goble, with the Springfield ALS chapter. Part of that growth could stem from more accurate diagnoses, said Springfield neurologist Rodney Quinn. (There used to be something called 'creeping paralysis,' Goble observed.) Copyright © 2002, The Springfield News-Leader
By EMMA ROSS AP Medical Writer LONDON (AP)--Some of the world's leading scientists gathered Monday at Cambridge University to celebrate the 60th birthday of physicist Stephen Hawking, who has survived a remarkable 38 years with motor neuron disease. The university is staging a week of events to honor Hawking, concluding Friday with a symposium celebrating his contributions to fundamental physics and cosmology. His birthday is Tuesday. Hawking, a professor of mathematics at Cambridge and author of ``A Brief History of Time,'' was told he could not expect to live long when he was diagnosed at age 22 with amyotrophic lateral sclerosis, also known as Lou Gehrig's disease. The average survival time is three to five years from the onset of symptoms. © 2002 Cox Interactive Media
NewScientist.com news service A cellular version of a traffic jam may be what causes neurodegenerative diseases like the one suffered by the astrophysicist Stephen Hawking. Using a genetically engineered mouse, researchers in Pennsylvania have shown that clogging up the routes between motor neurons and the muscles they control can be a cause, rather than a symptom, of lost motor control. Motor neurons reside in the brain and spinal cord but control movements in body parts as far away as our toes and fingers. To do this, they have very long projections, or axons, which sometimes stretch up to a metre in length. Good motor control depends on these axons acting as supply routes, to transport proteins and other molecules back and forth. © Copyright Reed Business Information Ltd.
By Brooke Adams The mice at the top of a column of stacked plastic bins at Q Therapeutics are shivering so hard they seem to be jumping. Their nonstop shivering and seizures are caused by a genetic defect that robs the mice of the crucial myelin sheath that surrounds nerve cells and helps them send signals. Because of the defect, the mice are soon paralyzed and die prematurely. It is a related problem -- loss of this myelin sheath -- that in humans causes the progressive loss of function in multiple sclerosis and several other diseases that can cause paralysis in humans. And that's why what has happened to the mice is so promising: After being treated with an adult stem cell therapy developed at Q Therapeutics, they are no longer shivering. The product, called Q-Cells, also may be applicable to such neurodegenerative diseases as Parkinson's, Alzheimer's and amyotrophic lateral sclerosis, or ALS -- better known as Lou Gehrig's disease. Now, the National Institutes of Health have awarded a $5 million grant to Q Therapeutics, the University of Utah's Cell Therapy Facility and Johns Hopkins University School of Medicine, which as a team has had success in animal models of ALS.
Scientists have identified a protective gene that increases survival in motor neuron disease. People with the KIFAP3 gene lived 14 months longer on average than other MND patients. Experts hope they will be able to use this knowledge to develop life-extending treatments for patients with this debilitating and fatal disease. So far, one drug, riluzole, has been proven to extend life expectancy, but only by a few months. MND attacks the nerves that control movement and is often rapidly progressive. The vast majority of people with MND die within two to five years. Half of people die within 14 months of diagnosis. The researchers wanted to find out why a small minority appear to be more resistant to the disease. To do this they looked at 300,000 genetic variants in 2,359 people with MND and 2,814 unaffected volunteers from six different countries. They found that people with two beneficial variants of KIFAP3 lived on average four years while those with only one or none lived on average for two years and eight months. This improved the chances of surviving five years from about 10% to more than 30% for those carrying the "good" variants of KIFAP3. Lead researcher Professor Ammar Al-Chalabi, of King's College London, said scientists would now be able to work on designing new treatments based on KIFAP3. Treatments can now be directly designed to exploit the effect of this gene variation." (C)BBC
A gene linked to a type of motor neurone disease that runs in families has been found after a 10-year search. Along with a related gene reported last year, it opens up an unexplored area of research into the condition, investigators said. The finding will also help doctors screen and counsel families at risk of the disease, the US and UK team wrote in Science. Up to 10% of cases are inherited within families because of genetic mutations. Motor neurone disease (MND) involves the progressive wasting of the muscles, while usually leaving the mind unaffected. It affects some 5,000 people in the UK. The first MND gene - SOD1 - was found in 1993 and it has been a major focus of research. But then researchers found a protein called TDP-43 is deposited in the neurons of 90% of people with the condition. However, it was not apparent in animal models with the SOD1 mutation, suggesting that the first gene found is not linked with the major underlying biology of the disease. For the past decade the UK and US team have been looking for a gene believed to be located on chromosome 16. They eventually found a mutation in the FUS gene in one family with inherited MND - also known as amyotrophic lateral sclerosis. Further studies showed that 4% of all families had FUS mutations. The FUS gene is related to TDP-43, the gene for which was found by the same researchers last year. Professor Christopher Shaw, from the Institute of Psychiatry at Kings College London, said the FUS gene was a very important clue as to what causes motor neurons to degenerate. "It's very interesting, we really have wrung SOD1 out. We have looked at cells and mice endlessly, but the major pathways are not SOD pathways. The genetic pieces of the jigsaw puzzle are beginning to fit together, leading us in new and exciting directions of research." (C)BBC
Scientists have identified a molecule which could be key to understanding the cause of motor neurone disease (MND) and other neurodegenerative disorders. The Proceedings of the National Academy of Sciences study raise the hope of new treatments being developed. The London-based team showed the molecule, Wnt3, plays a key role in establishing connections between nerve cells and the muscles they control. These connections become progressively weaker in MND patients. Without properly-formed connections - or synapses - the muscle cannot receive the nerve signal that tells it to contract. This results in the muscle weakness that is typical of MND. However, scientists have not been clear how synapses are formed in normal circumstances and this has made it very difficult to pin down what goes wrong in MND. The researchers, from University College London and King's College London, identified Wnt3 as key to the process. It assists a second molecule, called Agrin, which co-ordinates construction of the connection - or synapse. Lead researcher Professor Patricia Salinas said: "The work we are publishing today puts an important piece of the puzzle in place and offers up a new possibility for developing drugs to treat MND and other neurodegenerative diseases. "If we can build up a thorough picture to show how synapses are normally formed between nerves and muscles we can start to look for any elements that aren't working properly in people with MND. This might also lead to strategies for nerve repair after an injury." (C)BBC
By Adam Brimelow British scientists are embarking on a major new trial to assess the impact of the mood stabiliser lithium as a treatment for motor neurone disease. They say the research is necessary because positive findings from a small-scale Italian study were "too dramatic to ignore". But they are urging patients with the disease not to take the treatment in advance of their results. They warn that some side-effects of lithium are potentially dangerous. There are about 5,000 people in the UK living with motor neurone disease (MND). At the moment there is no effective cure or treatment. It is often rapidly progressive and always fatal, usually within two to five years. The disease can affect any adult at any age, although it is more commonly found in men, and is most likely to strike between the ages of 50 and 70. Lithium, a naturally occuring element, has long been used as a treatment for some forms of depression, such as bipolar disorder. But recent laboratory tests and animal trials have suggested that it may also have a protective effect with MND. The recent trial of 16 people in Italy reported encouraging results. But the MND Association said the study was small and poorly designed, and that its findings should be treated with caution. The association's president, Professor Sir Colin Blakemore, said: "If you read the publication optimistically it might be taken to mean that lithium literally cures this disease. "But it's very important, against the background of patient hopes and expectations, to stand back and ask whether the trial was large enough to make the claims that it did." (C)BBC
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 16: Psychopathology: Biological Basis of Behavior Disorders
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 12: Psychopathology: Biological Basis of Behavioral Disorders
Link ID: 12201 - Posted: 11.03.2008
CHICAGO - A mutation in a single gene may raise one's risk of developing amyotrophic lateral sclerosis (ALS), also know as Lou Gehrig's disease, by as much as 30 percent, offering a potential new target for drug research, Dutch scientists said on Sunday. They said a variant in the DPP6 gene may give rise to ALS in people without a family susceptibility to the untreatable and fatal disease. Familial ALS, which accounts for 10 percent of all cases of the disease, has been linked with mutations in a number of other genes. Researchers have had less luck finding a gene associated with non-familial, sporadic ALS, which accounts for 90 percent of ALS cases. But researchers at the University Medical Center Utrecht said a SNP or single-letter change in the genetic code of the DPP6 gene is "consistently and strongly associated with susceptibility to amyotrophic lateral sclerosis in different populations of European ancestry." The DPP6 gene controls an enzyme found mostly in the brain that has been linked with spinal cord injury in rats. (c) Reuters 2007.
Scientists are hopeful that they have found a way to halt the progression of motor neurone disease (MND). A team at Bath University discovered a causal link between the gene involved in the formation of blood vessels and the development of some forms of MND. Mutant versions of the gene's product - angiogenin - are toxic to motor neurones, so blocking this process may stop the disease, they say. The latest UK work is published in the journal Human Molecular Genetics. There are about 5,000 people suffering with MND at any one time in the UK. The condition affects men more than women and one or two people in every 100,000 will be newly diagnosed with MND each year. In MND, over time, the cells responsible for transmitting the chemical messages that enable muscle movements become injured and subsequently die. Ultimately, the disease fatally interferes with those muscles involved in breathing. Last year, scientists discovered that some patients with MND have a mutated version of the human angiogenin gene. Since then, experts have been trying to find out what role angiogenin plays in the maintenance and development of motor neurones. Lead researcher Dr Vasanta Subramanian said: "We have found that mutated versions of this molecule are toxic to motor neurones and affect their ability to put out extensions called the axons. (C)BBC
Stem cells show potential for treating the debilitating nerve condition motor neurone disease, research suggests. A US team found injecting rats with stem cells delayed the onset of MND. Writing in the Transplantation, the researchers from Johns Hopkins Medical Institutions warned clinical use of stem cells was still a long way off. But they said their findings would help scientists to better understand how stem cells behaved when they were transplanted into the body. Motor neurone disease (MND) affects about 5,000 people in the UK. It is a progressive disorder caused by the break-down of the nerve cells, called motor neurones, which control the muscle activity. It is characterised by muscle-wasting, loss of mobility, and difficulties with speech, swallowing and breathing. To investigate whether stem cells - cells that can transform into any type of cell in the body - could help MND sufferers, scientists injected rats, bred to carry the most common form of MND, amyotrophic lateral sclerosis (AMS), with live human stem cells into their lower spines. They found that 70% of the transplanted cells developed into new nerve cells, and many of them had grown new endings connecting with other cells in the rats' spinal cords. The onset of the disease, marked by weight loss, was also delayed. It began on average at 59 days, in the rats injected with live stem cells, compared with 52 days for control rats that had been injected with dead, and therefore inactive, stem cells. They also discovered the rats with live stem cells grew weaker more slowly and lived longer than those that had received dead stem cell transplants. (C)BBC
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 7: Life-Span Development of the Brain and Behavior
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 13: Memory, Learning, and Development
Link ID: 9489 - Posted: 10.17.2006