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The fatal disorder ALS wreaks its havoc by harming nerve cells that control muscles. As a consequence of the damage, patients experience progressive muscle weakness that can hinder movement and speech, even swallowing and breathing. Once, little could be done to help these patients who often spend their last days in an isolating paralysis. But now following years of research, scientists are finding ways to shield cells from the wrath of ALS and help delay the disorder’s progression. Recent developments include the potential use of growth factors, special substances that appear to offer cell protection. No crime was committed. Yet an estimated 5,000 Americans afflicted annually with ALS will suffer a particularly torturous sentence. People with this disorder, also known as amyotrophic lateral sclerosis or Lou Gehrig’s disease, often end up imprisoned in their bodies. Many spend their last days completely unable to move while their minds remain sharp and alert. ALS wreaks its havoc by harming nerve cells that control muscles. As a consequence of the damage, people with ALS experience progressive muscle weakness that can hinder movement and speech, creating an isolating paralysis. Swallowing and breathing also may be harmed and many die within three to five years after they experience symptoms, usually in mid-life. Once, doctors could do little to help these patients. But now following years of study, scientists are finding ways to protect cells and help delay the progression of ALS. Copyright © 2005 Society for Neuroscience
the virtues of proteins that can save dying neurons Diseases characterized by neurodegeneration affect individuals over 50 years of age and they attack one particular class of neurons in the brain or spinal cord. The research interest of Professor Ann Kato and her team is principally focalized on ALS in which there is a progressive paralysis caused by the destruction of motor neurons which exist in both the brain and spinal cord. These cells are responsible for the movements of the upper and lower limbs and their elimination causes muscular atrophy and finally death. During the last 3 years, the objectives of the experiments of Professor Kato consist in trying to understand why these neurons die and to study the intracellular and molecular pathways which can prevent their degeneration. It was during the course of their experiments to attempt to prolong the survival of the cells that the group of Professor Kato found that a newly discovered family of proteins called Inhibitors of Apoptosis Proteins (IAP’s) were capable of preventing the death of motor neurons. Nature Cell Biology, February 2002, vol. 4, and pages175-179 © AlphaGalileo 2001
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: 1426 - Posted: 06.24.2010
Perhaps one of the most astonishing features of the human nervous system is the fact that muscles in one part of the body, for example the feet, can be controlled by neurons whose cell bodies are located extremely far away in the spinal cord. These cell bodies therefore must extend processes incredibly long distances. As most of the proteins in a neuron are made in the cell body, the transport of proteins and other molecules through these very long processes called axons is critically important for proper motor control. In certain neurodegenerative diseases in which motor control is impaired, e.g., amyotrophic lateral sclerosis, traffic along the axons slows down and certain molecules accumulate. Could this cellular “traffic jam” be responsible for the degenerative phenotype and associated muscular atrophy? Copyright © 1995-2002 UniSci. All rights reserved.
By MARCUS WOHLSEN CLAREMONT, Calif. -- Until last year, Alan Felzer was an energetic engineering professor who took the stairs to his classes two steps at a time. Now the 64-year-old grandfather sits strapped to a wheelchair, able to move little but his left hand, his voice a near-whisper. Felzer suffers from ALS, also known as Lou Gehrig's disease. The fatal neurological disorder steals the body's ability to move, speak and ultimately to breathe. But rather than succumb to despair along with his illness, Felzer turned to the Web to become his own medical researcher _ and his own guinea pig. Dozens of ALS patients are testing treatments on their own without waiting on the slow pace of medical research. They are part of an emerging group of patients willing to share intimate health details on the Web in hopes of making their own medical discoveries. Some doctors caution that such patient-led research lacks rigor and may lead to unreliable results, false hopes and harm to patients. "The Internet is a wonderful tool, but you know, it's buyer beware," said Dr. Edward Langston, immediate past chairman of the American Medical Association's board. In Felzer's case, the experiment's results illustrate the obstacles that stand between patients and self-discovered breakthroughs. The drug he tried did no good. But he and his family felt they had little time and little to lose in trying. © Copyright 1996-2008 The Washington Post Company
By Claire Panosian Dunavan When my friend Nikki Tal died in 2004, the world lost a strong, brave soul -- despite a thuggish disease that had by then utterly ravaged her body. From her earliest years, Nikki had been a reader. She also loved the ocean. Her favorite book was "Life of Pi," about a shipwrecked boy trapped on a raft with a hungry predator. The parallels with her own life weren't hard to see. Nikki's predator was amyotrophic lateral sclerosis, better known as ALS, or Lou Gehrig's disease. As any doctor will tell you, for patients with neurologic blights, ALS is about as bad as it gets. In less than a year, it can extinguish an entire set of motor neurons, rendering its victims limp and powerless. It was October 1996 when Nikki first learned something was wrong. While attending a baby shower, the 5-foot-8 lawyer realized she couldn't lift a 10-pound newborn. Later that month, she destroyed her car's ignition after repeatedly mis-inserting her key; then she begged off timing duty at her daughter's swim meets because she could no longer depress a stopwatch. A few months later came the inability to walk or care for herself; the big, padded wheelchair and the handicap van; and, eventually, the feeding tube and the portable ventilator. Finally, ALS robbed Nikki of the ability to hold her head erect and of producing even remotely intelligible speech. © 2008 The Washington Post Company
By Devin Powell Even with the entire human genome in hand, scientists can still have trouble rooting out the genes behind a disease. Consider amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. One ALS-related gene mutation was found in 1993, but it affects only 1% of all ALS patients. Now, after a long dry spell, researchers have finally hit on a second. It's just as rare as the first, but it seems to be more closely related to aspects of ALS pathology found in all patients. ALS typically appears in middle age, slowly destroying the motor neurons used to control muscles and usually killing a patient within 5 years of diagnosis. Five percent of all adult-onset ALS cases are known to have a strong genetic component, affecting multiple family members. But the rest seem to appear spontaneously in people with no prior family history. Scientists know little about what causes these sporadic cases. And the one known ALS gene in inherited ALS, called SOD1, doesn't seem to lead to neuronal death--the primary characteristic of ALS--so the link between this gene and the disease is still unclear. Christopher Shaw, a neurologist at King's College London, started hunting for other ALS genes by recruiting patients who have the inherited form of the disease but not the SOD1 mutation. While screening 154 people with familial ALS, Shaw and his colleagues found four individuals in one family who shared the same mutation: a single changed base on chromosome 1. The base was located in the TDPB gene, which encodes a protein called TDP-43 whose function isn't clear. In 2006, scientists reported that in both inherited and sporadic ALS, this protein disappears from the nucleus and clumps up in the cytoplasm of brain and motor neurons. © 2008 American Association for the Advancement of Science.
A rigorous study in Italy has confirmed claims that professional soccer players have a higher than normal risk of developing a type of motor neuron disease, also known as amyotrophic lateral sclerosis. The reason remains a mystery. ALS involves the death of motor neurons, the nerve cells responsible for voluntary movement, and eventually leads to paralysis and death. Adriano Chiņ's team at the University of Turin surveyed the medical records of 7000 professional footballers who played in Italy's first or second division between 1970 and 2001. Based on the normal incidence of the disease and the players' ages, the researchers calculated that there should have been 0.8 cases of ALS in this group. Instead, there were five. The study was prompted by what the Italian press dubbed "the motor neuron mystery" - the discovery a few years ago of 33 cases of ALS during an investigation of illicit drug use among 24,000 pro and semi-pro players in Italy. Dubious about the methodology of that initial investigation, Chiņ's group applied stricter diagnostic criteria to their data, such as only including players born in Italy. "I think the researchers have been conservative," says Ammar Al-Chalabi of the Institute of Psychiatry in London, who has written a commentary on the study in Brain. © Copyright Reed Business Information Ltd.
By Jennifer Warner -- Despite the famous link between ALS and baseball great Lou Gehrig, a new study shows that there's no medical link between the disease and physical activity. Researchers found no association between increased physical activity and the risk of developing ALS (amyotrophic lateral sclerosis). Those results contradict several previous studies that have shown that slim, athletic individuals may be more likely to develop ALS. ALS is also sometimes called "Lou Gehrig's disease," after the famous baseball player whose career was cut short by the disease, which gradually erodes muscle strength. Although ALS is often very disabling, many people live for years with the disease. The average life expectancy is only two to five years. In the study, researchers compared the amount of physical activity reported by 219 people with ALS with 254 healthy people. All of the participants were asked whether they engaged in sports as youngsters or as an adult or whether they performed extreme physical activity. They reported total physical activity levels as well as activity levels in three different phases of their lives: before age 25, the last 10 years before symptoms of the disease emerged, and one year before the start of the disease. ©1996-2005 WebMD Inc.
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.