Links for Keyword: Movement Disorders

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By Diana Kwon Like humans, some golden retrievers develop Duchenne muscular dystrophy (DMD), a hereditary muscle wasting condition that begins early in life. Using gene therapy, scientists were able to restore muscle function in dogs with the disease, according to a study published today (July 25) in Nature Communications. Researchers injected microdystrophin, a shortened version of the dystrophin gene that individuals with DMD lack, into 12 dogs with the disease. The treatment led to improved muscle function in those animals for more than two years. “This preclinical study demonstrates the safety and efficacy of microdystrophin, and makes it possible to consider developing a clinical trial in patients,” study coauthor Caroline Le Guiner of the Université de Nantes in France, says in a statement. “Indeed, this is the first time that it has been possible to treat the whole body of a large-sized animal with this protein.” Scientists have also used CRISPR to correct the disease-causing mutations in mouse models of DMD and in the cells of a human patient with the condition. “This [study] is very encouraging, as current treatments for muscular dystrophy are merely palliative and patients are under constant medical care throughout their life,” John Counsell, a research associate at University College London who was not involved in the study, in a statement published by the Science Media Center. “Further preclinical trials will be required to show that this treatment can be effective in patients.” © 1986-2017 The Scientist

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 23883 - Posted: 07.27.2017

By Aylin Woodward Keep your head up. Today, navigating the urban jungle can be challenging, with uneven sidewalks and errant kerbs presenting obstacles to easy walking. So why do we rarely trip up even though we hardly ever give walking our full attention? It seems that all we need is a brief glimpse of what’s coming next on the road in front of us, just one step ahead of time, to keep up upright. Humans have a unique kind of locomotion – we’re bipedal, meaning we move around on two legs rather than four. Scientists are still struggling to unravel the mystery behind our shift to two legs – for instance, some suggest it freed up our hands to carry food. Others point out that our human gait is much more energetically efficient. Our walking style exploits external forces like gravity and inertia to use as little muscular energy as possible so that we actually fall forward onto the lifted foot with each step. Jonathan Samir Matthis at the University of Texas at Austin wanted to know how we aim and control this forward motion – particularly since the way ahead is rarely level and obstacle-free. “We have to be much more careful about where we place our feet than we would if we had four legs on the ground,” he says. “Because if we do it wrong, there’s serious consequences like breaking your leg.” © Copyright New Scientist Ltd.

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 18: Attention and Higher Cognition
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 14: Attention and Consciousness
Link ID: 23872 - Posted: 07.25.2017

By Bob Grant Prosthetic limbs are rejected by amputees’ bodies at a rate of about 20 percent. Researchers at MIT are seeking to reduce that number, using an amputation procedure that encourages increased feedback between muscles, tendons, and the nervous system so that an artificial limb might stimulate them in a more natural way—giving patients a better sense of proprioception, or where their limb is in space. The key to the surgical technique, demonstrated in rats so far, is to emulate the normal agonist-antagonist pairing of muscles (think biceps and triceps) at the amputation site so that the muscles and nerves surrounding a prosthetic can sense and transmit proprioceptive information about the artificial limb and how much force is being applied to it. The researchers published their work today (May 31) in Science Robotics. “We’re talking about a dramatic improvement in patient care,” Hugh Herr, an MIT professor of media arts and sciences and a coauthor of the study, said in a statement. “Right now there’s no robust neural method for a person with limb amputation to feel proprioceptive positions and forces applied to the prosthesis. Imagine how that would completely hinder one’s ability to move, to successfully balance, or to manipulate objects.” Herr, himself a double-amputee, and his team operated on seven rats, cutting through muscles and nerves in their hind legs. The researchers then grafted on paired muscles, wiring them up to severed nerves. After healing for four months, the rats’ new muscles were contracting and relaxing in tandem, as in naturally paired muscles, and sending electrical signals that reflected the amplitude of the artificial stimulation Herr and his colleagues applied. © 1986-2017 The Scientist

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 23693 - Posted: 06.02.2017

By LISA SANDERS, M.D. The woman woke to the sound of her 57-year-old husband sobbing. They’d been married for 30 years, and she had never heard him cry before. “I hurt so much,” he wailed. “I have to go back to the hospital.” The symptoms started two weeks earlier. One afternoon, coming home from his job as a carpenter, he felt hot and tired. He shook with shivers even though the day was warm. He drank a cup of tea and went to bed. The next day he felt fine, until the end of the day, when he felt overwhelmed by the heat and chills again. The day after that was the same. When he woke one morning and saw that his body was covered with pale pink dots — his arms, his face, his chest and thighs — he started to worry. His wife took him to the Griffin Hospital emergency room in Derby, Conn. The first doctor who saw him thought he probably had Lyme disease. Summer had just started, and he’d already seen a lot of cases. He sent the patient home with an antibiotic and steroid pills for the rash. The man took the medications but didn’t get any better. Soon everything started to hurt. His muscles, his joints and his back felt as if he’d been beaten. He dragged himself back to the E.R. He was given pain pills. A few days later, he went to the E.R. a third time and was given more pain meds. After waking up crying, he went yet again, and this time, the doctors admitted him. By then the patient had had several blood tests, which showed no sign of Lyme or other tick-borne diseases. A CT scan was equally uninformative. The next day, the man was walking to the bathroom when his legs gave out and he fell down. The doctor in charge of his care came and examined him once again. The man looked fit and healthy, despite the now-bright-red rash, but his legs were extremely weak. If the doctor applied even light pressure to the raised leg, it sagged back down to the bed. And his feet felt numb. He had a sensation of tingling in his hands, as if they had gone to sleep. That was how the weakness and numbness in his legs started, he told the doctor. And the next day, his hands were so weak he had to use both just to drink a cup of water. © 2017 The New York Times Company

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 15: Emotions, Aggression, and Stress
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 11: Emotions, Aggression, and Stress
Link ID: 23644 - Posted: 05.22.2017

By Paul Taylor One of the bummers of getting older, as most baby boomers can attest, is that the list of stuff you don’t do as well as you once did keeps getting longer. Bennett Beach, 67, can measure his decline with a stopwatch. Three hours, 27 minutes, 56 seconds: That’s the difference between his best time in the Boston Marathon (2:27:26) and his worst (5:55:22). On April 17, he’ll be running the famous race once again. If he completes the course in less than six hours, he will have officially finished his 50th consecutive Boston Marathon. No one has ever done that. Nor, as far as he knows, will any of his 32,000 fellow racers be coping, as he is, with the rare and debilitating neurological movement disorder known as task-specific dystonia. Whenever he strides, Beach’s left leg gets hijacked by erratic signals from his brain. His walk is nearly normal, but for the past 15 years he has been running with a severe limp. His pursuit of the milestone has been fueled in roughly equal measure by antithetical parts — an Ahab-grade obsession mixed with an older-but-wiser acceptance of his body’s limits. “If someone had told me 30 years ago I’d be struggling to finish this race in six hours, I’d have said, ‘Spare me.’ Now I’m grateful.” Beach is a marathoner by demeanor: quiet, unassuming, self-effacing, iron-willed. And by body type: 5-foot-7, 125 pounds. He played all sports as a kid, distinguishing himself at none: “I just didn’t have the size or strength.” As a senior in prep school, he happened upon a radio broadcast of the Boston Marathon. “It was 30 degrees, it was sleeting, and these guys were out there running 26 miles,” he remembers. “Just the sort of bizarre, crazy thing I was drawn to. I already knew I’d be in Boston the next year, so I decided I’d give it a shot.” © 1996-2017 The Washington Post

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 23471 - Posted: 04.10.2017

Sarah Jane Tribble In response to outrage from patients and lawmakers, Marathon Pharmaceuticals has delayed the launch of an $89,000 drug for Duchenne muscular dystrophy. The company had announced the annual list price for Emflaza, which is a steroid, after the Food and Drug Administration approved the drug Thursday. Emflaza is approved as an orphan drug, which means it is intended to treat a rare disease. Duchenne is an inherited disorder that causes muscles to become weak. There is no cure for the condition, which mainly affects boys, but some drugs, including Emflaza, are used to lessen symptoms. For years, many American patients have imported deflazacort, the generic version of Emflaza, for about $1,200 a year. But because the medicine wasn't approved in the U.S., the cost of the medicine wasn't typically covered by insurers. That contrast in price between became a flash point Monday as Sen. Bernie Sanders, I-Vt., and Rep. Elijah Cummings, D-Md., sent a letter to Marathon on Monday morning demanding answers about the $89,000 price for a drug that isn't new. It has been used routinely by Duchenne patients in the U.S. since at least 2005. "We believe Marathon is abusing our nation's 'orphan drug' program, which grants companies seven years of market exclusivity to encourage research into new treatments for rare diseases — not to provide companies like Marathon with lucrative market exclusivity rights for drugs that have been available for decades," Sanders and Cummings wrote. Marathon said FDA approval would help more patients get the drug. © 2017 npr

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 23234 - Posted: 02.15.2017

Valerie Piro The alarm goes off at 4:30 a.m. Groggy, I turn on the lamp on my night stand and try to sit up. I put my right hand on the wall next to my bed to steady myself, and push my left into the bed. Right away, my abs and back seize up and my legs spasm and kick out straight, forcing me back down onto the bed. Clearly my body thinks it is too early to get up, but I don’t have time to argue with it. I have to get physical therapy out of the way so I can be on time for my medieval history class. After I sit up, I place my hands under my right knee and clasp them together as I bring my knee up and closer to my chest. I reach out to my right foot and cross its heel over my left thigh so that I can plant my heel on the bed. I hug my right leg against my torso and chest and feel a stretch in my lower back and butt. I repeat this on my other side and then proceed to stretch each ankle. Paralysis requires maintenance. I then hop toward the foot of my bed, where my commode chair sits. I set both feet on the footrests as best I can, grab the armrest on the far side of the chair with my left hand, and, using my right hand to drive down into my bed, lift myself onto the commode wheelchair, and wheel to the bathroom. I emerge at 5:35 a.m. I transfer now into a wheelchair whose dimensions are friendly toward my Functional Electrical Stimulation (F.E.S.) cycle — something like a gym exercise bike, without the seat. I pull some milk out of the mini-fridge and pour it over a bowl of cereal. I eat while checking and answering email. At 6:30 it’s time to start cycling. I put two small rectangular electrodes on my left shin muscles, and then two on my right, connect them to the cycle, then strap in my legs and feet. Then two more electrodes then two more, and so on, until most of my lower body is tapped and wired. After I turn on the tablet that’s attached to the cycle, I choose from one of several preset programs to start my workout. Within a couple of minutes, electrical shocks are pulsing into my legs, causing them to contract into pedaling. Imagine pedaling a bicycle uphill for an hour; this is my workout. © 2017 The New York Times Company

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 23081 - Posted: 01.11.2017

By KATIE THOMAS The Food and Drug Administration has approved the first drug to treat patients with spinal muscular atrophy, a savage disease that, in its most severe form, kills infants before they turn 2. “This is a miracle — seriously,” Dr. Mary K. Schroth, a lung specialist in Madison, Wis., who treats children who have the disease, said of the approval, which was made last week. “This is a life-changing event, and this will change the course of this disease.” Dr. Schroth has previously worked as a paid consultant to Biogen, which is selling the drug. The drug, called Spinraza, will not come cheap — and, by some estimates, will be among the most expensive drugs in the world. Biogen, which is licensing Spinraza from Ionis Pharmaceuticals, said this week that one dose will have a list price of $125,000. That means the drug will cost $625,000 to $750,000 to cover the five or six doses needed in the first year, and about $375,000 annually after that, to cover the necessary three doses a year. Patients will presumably take Spinraza for the rest of their lives. The pricing could put the drug in the cross hairs of lawmakers and other critics of high drug prices, and perhaps discourage insurers from covering it. High drug prices have attracted intense scrutiny in the last year, and President-elect Donald J. Trump has singled them out as an important issue. “We believe the Spinraza pricing decision is likely to invite a storm of criticism, up to and including presidential tweets,” Geoffrey C. Porges, an analyst for Leerink Partners, said in a note to investors on Thursday. Mr. Porges said the price could lead some insurers to balk or to limit the drug to patients who are the most severely affected, such as infants, even though the F.D.A. has approved Spinraza for all patients with the condition. © 2016 The New York Times Company

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 23040 - Posted: 12.31.2016

By Meredith Wadman There have been few happy endings when it comes to spinal muscular atrophy (SMA), the most common genetic cause of death in childhood. The disease inexorably destroys the motor neurons of the spinal cord and brainstem that control movement, including swallowing and breathing. In its most severe form, SMA kills those afflicted at about age 2, most commonly by suffocating them. There are no Food and Drug Administration (FDA)–approved drugs for the disease. That is almost certainly about to change. An innovative drug that helps cells bypass the genetic flaw responsible for SMA may be approved as soon as this month, on the heels of strongly positive results from late-stage clinical trials. On 7 November, a trial of the drug, nusinersen, in wheelchair-bound children aged 2 to 12, was stopped on the grounds that it was unethical to deny the drug to children in the control arm, given the positive results in the treated children. In August, a similar trial in infants was stopped for the same reason, allowing the untreated infants in a control arm to begin receiving the drug. And today, a paper appearing in The Lancet provides compelling biological evidence that nusinersen is having its desired effect in the cells of the brain and spinal cord. “These [infant-onset] SMA kids are going to die. And not only are they now not dying, you are essentially on the path to a true cure of a degenerative [neurological] disease, which is unheard of,” says Jeffrey Rothstein, a neurologist at the Johns Hopkins School of Medicine in Baltimore, Maryland, who was not affiliated with the trials of the drug and is not connected with either of the two companies involved in its development: Ionis of Carlsbad, California, and Biogen of Cambridge, Massachusetts. © 2016 American Association for the Advancement of Science

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: 22967 - Posted: 12.08.2016

David Cyranoski For more than a decade, neuroscientist Grégoire Courtine has been flying every few months from his lab at the Swiss Federal Institute of Technology in Lausanne to another lab in Beijing, China, where he conducts research on monkeys with the aim of treating spinal-cord injuries. The commute is exhausting — on occasion he has even flown to Beijing, done experiments, and returned the same night. But it is worth it, says Courtine, because working with monkeys in China is less burdened by regulation than it is in Europe and the United States. And this week, he and his team report1 the results of experiments in Beijing, in which a wireless brain implant — that stimulates electrodes in the leg by recreating signals recorded from the brain — has enabled monkeys with spinal-cord injuries to walk. “They have demonstrated that the animals can regain not only coordinated but also weight-bearing function, which is important for locomotion. This is great work,” says Gaurav Sharma, a neuroscientist who has worked on restoring arm movement in paralysed patients, at the non-profit research organization Battelle Memorial Institute in Columbus, Ohio. The treatment is a potential boon for immobile patients: Courtine has already started a trial in Switzerland, using a pared-down version of the technology in two people with spinal-cord injury. © 2016 Macmillan Publishers Limited

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22858 - Posted: 11.12.2016

Ian Sample Science editor Partially-paralysed monkeys have learned to walk again with a brain implant that uses wireless signals to bypass broken nerves in the spinal cord and reanimate the useless limbs. The implant is the first to restore walking ability in paralysed primates and raises the prospect of radical new therapies for people with devastating spinal injuries. Scientists hope the technology will help people who have lost the use of their legs, by sending movement signals from their brains to electrodes in the spine that activate the leg muscles. One rhesus macaque that was fitted with the new implant regained the ability to walk only six days after it was partially paralysed in a surgical procedure that severed some of the nerves that controlled its right hind leg. “It was a big surprise for us,” said Grégoire Courtine, a neuroscientist who led the research at the Swiss Federal Institute of Technology. “The gait was not perfect, but it was almost like normal walking. The foot was not dragging and it was fully weight bearing.” A second animal in the study that received more serious damage to the nerves controlling its right hind leg recovered the ability to walk two weeks after having the device fitted, according to a report published in the journal, Nature. Both monkeys regained full mobility in three months. The “brain-spine interface” is the latest breakthrough to come from the rapidly-advancing area of neuroprosthetics. Scientists in the field aim to read intentions in the brain’s activity and use it to control computers, robotic arms and even paralysed limbs. © 2016 Guardian News and Media Limited

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22854 - Posted: 11.10.2016

By Neuroskeptic A new paper could prompt a rethink of a basic tenet of neuroscience. It is widely believed that the motor cortex, a region of the cerebral cortex, is responsible for producing movements, by sending instructions to other brain regions and ultimately to the spinal cord. But according to neuroscientists Christian Laut Ebbesen and colleagues, the truth may be the opposite: the motor cortex may equally well suppress movements. Ebbesen et al. studied the vibrissa motor cortex (VMC) of the rat, an area which is known to be involved in the movement of the whiskers. First, they determined that neurons within the VMC are more active during periods when the rat’s whiskers are resting: for instance, like this: whiskerThe existence of cells whose firing negatively correlates with movement is interesting, but by itself it doesn’t prove that much. Maybe those cells are just doing something else than controlling movement? However, Ebbesen et al. went on to show that electrical stimulation of the VMC caused whiskers to stop moving, while applying a drug (lidocaine) to suppress VMC activity caused the rat’s whiskers to whisk harder. Ebbesen et al. go on to say that the inhibitory role of VMC may extend to other regions of the rat motor cortex, and to other movements beyond the whiskers: Rats can perform long sequences of skilled, learned motor behaviors after motor cortex ablation, but motor cortex is required for them to learn a task of behavioral inhibition (they must learn to postpone lever presses)35. When swimming, intact rats hold their forelimbs still and swim with only their hindlimbs. After forelimb motor cortex lesions, however, rats swim with their forelimbs also36.

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22837 - Posted: 11.07.2016

By Dan Hurley The Centers for Disease Control and Prevention has confirmed 89 cases of the paralyzing disease in the United States through September. A 6-year-old boy suspected of having AFM died in Seattle on Sunday, the first death believed to be caused by the disease. One of the drugs in development, pocapavir, was used briefly on a few patients during a 2014 outbreak of AFM under a compassionate-use exception that allows extremely sick patients to be given unapproved drugs without the usual kinds of placebo-controlled trials required by the Food and Drug Administration. “There were a couple of kids who got pocapavir in the Colorado outbreaks,” said Benjamin Greenberg, a neurologist who has treated children with AFM at the University of Texas Southwestern in Dallas. “It had relatively weak but measurable impact on viral replication. A larger study would definitely be warranted. We'll take anything we can get.” Although the CDC says no cause has been conclusively linked to AFM, many researchers suspect a family of viruses known as enteroviruses. “I have been studying enteroviruses for 40 years now,” said John Modlin, deputy director of the polio eradication program at the Bill and Melinda Gates Foundation. “If I had a child with acute flaccid myelitis, I would be on the phone in a second to the companies making these drugs.” © 1996-2016 The Washington Post

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22830 - Posted: 11.04.2016

By Carl Luepker For the past 35 years, a relentless neurological disorder has taken over my body, causing often painful muscle spasms that make it hard for me to walk and write and that cause my speech to be garbled enough that people often can’t understand me I can live with my bad luck in getting this condition, which showed up when I was 10; what’s harder to accept is that I have passed on this disorder, carried in my genes, to my 11-year-old son, Liam. As a parent, you hope that your child’s life will follow an upward trend, one of emotional and physical growth toward an adulthood of wide-open possibilities where they can explore the world, challenge themselves emotionally and physically, and perhaps play on a sports team. And you hope that you can pass down to your child at least some of what was passed down to you. Yet my generalized dystonia, as my progressive condition is called, was one thing I had hoped would end with me. Liam poses for a photograph just months before his diagnosis with dystonia. He “has just moved into middle school,” his father writes, where “he will have to both advocate for himself and educate his new teachers and peers about this genetic disorder.” When my wife and I started thinking of having kids, the statistics were fairly reassuring: There was a 1-in-2 chance that our child would inherit the gene that causes the disorder, but most people who have the gene don’t go on to manifest dystonia. We wanted a family and rolled the dice — twice. Our daughter does not have the gene. © 1996-2016 The Washington Post

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22720 - Posted: 10.02.2016

By Alison F. Takemura Bodies like to keep their pH close to 7.4, whether that means hyperventilating to make the blood alkaline, or burning energy, shifting to anaerobic metabolism, and producing lactate to make the blood acidic. The lungs and kidneys can regulate pH changes systemically, but they may not act quickly on a local scale. Because even small pH changes can dramatically affect the nervous system, a study led by Sten Grillner of Karolinska Institute in Sweden looked for a mechanism for pH homeostasis in the spinal cord. Using the lamprey as a model system, the researchers observed that a type of spinal canal neuron, called CSF-c, fired more rapidly when they bathed it with high pH (7.7) or low pH (7.1) media. They could suspend the elevated activity by blocking two ion channels: PKD2L1 channels, which stimulate neurons in alkaline conditions, or ASIC3 channels, which, the team showed previously, do the same in acidic states. As the neurons fired, they released the hormone somatostatin, which inhibited the lamprey’s locomotor network. These results suggest that, whichever direction pH deviates, “the response of the system is just to reduce activity as much as possible,” Grillner says. The pH-regulating role of CSF-c neurons is likely conserved among animals, the authors suspect, given the presence of these neurons across vertebrate taxa. © 1986-2016 The Scientist

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 8: General Principles of Sensory Processing, Touch, and Pain
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 5: The Sensorimotor System
Link ID: 22688 - Posted: 09.24.2016

By Michael Price A soft brush that feels like prickly thorns. A vibrating tuning fork that produces no vibration. Not being able to tell which direction body joints are moving without looking at them. Those are some of the bizarre sensations reported by a 9-year-old girl and 19-year-old woman in a new study. The duo, researchers say, shares an extremely rare genetic mutation that may shed light on a so-called “sixth sense” in humans: proprioception, or the body’s awareness of where it is in space. The new work may even explain why some of us are klutzier than others. The patients’ affliction doesn’t have a name. It was discovered by one of the study’s lead authors, pediatric neurologist Carsten Bönnemann at the National Institutes of Health (NIH) in Bethesda, Maryland, who specializes in diagnosing unknown genetic illnesses in young people. He noticed that the girl and the woman shared a suite of physical symptoms, including hips, fingers, and feet that bent at unusual angles. They also had scoliosis, an unusual curvature of the spine. And, significantly, they had difficulty walking, showed an extreme lack of coordination, and couldn’t physically feel objects against their skin. Bönnemann screened their genomes and looked for mutations that they might have in common. One in particular stood out: a catastrophic mutation in PIEZO2, a gene that has been linked to the body’s sense of touch and its ability to perform coordinated movements. At about the same time, in a “very lucky accident,” Bönnemann attended a lecture by Alexander Chesler, a neurologist also at NIH, on PIEZO2. Bönnemann invited Chesler to help study his newly identified patients. © 2016 American Association for the Advancement of Science.

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 8: General Principles of Sensory Processing, Touch, and Pain
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 5: The Sensorimotor System
Link ID: 22683 - Posted: 09.22.2016

By SABRINA TAVERNISE WASHINGTON — The Food and Drug Administration approved the first drug to treat patients with the most common childhood form of muscular dystrophy, a vivid example of the growing power that patients and their advocates wield over the federal government’s evaluation of drugs. The agency’s approval went against the recommendation of its experts. The main clinical trial of the drug was small, involving only 12 boys with the disease known as Duchenne muscular dystrophy, and did not have an adequate control group of boys who had the disease but did not take the drug. A group of independent experts convened by the agency this spring said there was not enough evidence that it was effective. But the vote was close. Large and impassioned groups of patients, including boys in wheelchairs, and their advocates, weighed in. The muscular dystrophy community is well organized and has lobbied for years to win approval for the drug, getting members of Congress to write letters to the agency. A decision on the drug had been delayed for months. The approval was so controversial that F.D.A. employees fought over it, a dispute that was taken to the agency’s commissioner, Dr. Robert M. Califf, who ultimately decided that it would stand. The approval delighted the drug’s advocates and sent the share price of the drug’s maker, Sarepta Therapeutics, soaring. But it was taken as a deeply troubling sign among drug policy experts who believe the F.D.A. has been far too influenced by patient advocates and drug companies, and has allowed the delicate balance in drug approvals to tilt toward speedy decisions based on preliminary data and away from more conclusive evidence of effectiveness and safety. © 2016 The New York Times Company

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22671 - Posted: 09.20.2016

By CATHERINE SAINT LOUIS In seven countries that recently experienced Zika outbreaks, there were also sharp increases in the numbers of people suffering from a form of temporary paralysis, researchers reported Wednesday. The analysis, published online in The New England Journal of Medicine, adds to substantial evidence that Zika infections — even asymptomatic ones — may bring on a paralysis called Guillain-Barré syndrome. The syndrome can be caused by a number of other factors, including infection with other viruses. Researchers studying the Zika epidemic in French Polynesia had estimated that roughly 1 in 4,000 people infected with the virus could develop the syndrome. The Centers for Disease Control and Prevention has said that the Zika virus is “strongly associated” with Guillain-Barré, but has stopped short of declaring it a cause of the condition. The new data suggest a telling pattern: Each country in the study saw unusual increases in Guillain-Barré that coincided with peaks in Zika infections, the researchers concluded. “It’s pretty obvious that in all seven sites there is a clear relationship,” said Dr. Marcos A. Espinal, the study’s lead author and the director of communicable diseases at the Pan American Health Organization, which collected data on confirmed and suspected cases of Zika infection and on the incidence of Guillain-Barré. “Something is going on.” In Venezuela, officials expected roughly 70 cases of Guillain-Barré from December 2015 to the end of March 2016, as mosquitoes were spreading the virus. Instead, there were 684 cases. Similarly, during five months in which the Zika virus was circulating in Colombia, officials recorded 320 cases of Guillain-Barré when there should have been about 100. From September 2015 to March 2016, while Zika infections peaked in El Salvador, cases of Guillain-Barré doubled to 184 from 92. © 2016 The New York Times Company

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22618 - Posted: 09.01.2016

By Kas Roussy, In a room at Sunnybrook Health Sciences Centre in Toronto, Brian Smith gives one last hug to his wife, Noreen. "You're doing really well, sweetheart," he says to her. Doctors have finished prepping the 76-year-old patient. She's clad in a blue hospital gown, her head has been shaved and metallic headgear is attached to her skull. Google's latest a spoon that steadies tremors New technology could help seniors stay independent longer She's ready to be wheeled into an MRI room, where she'll undergo a procedure that her doctors believe will revolutionize the way brain diseases are treated. Before that happens, Noreen leans into her husband for a kiss. "Best buddy," she whispers. Noreen Smith is among the three per cent of the Canadian population who suffer from a nervous system disorder called essential tremor. It causes uncontrollable shaking, most often in a person's hands. Smith noticed the first signs when she was 33. "It started developing in my dominant hand, which is my right hand," she said the day before her medical procedure from her home in Bobcaygeon, Ont. She went to a specialist who delivered the diagnosis: essential tremor. Media placeholder Smith ‘really, really excited’ about treatment’s potential0:48 Just as shocking was what he said next, alluding to a high-profile actor who had the condition. "This particular person wasn't terribly helpful because he said: 'Do you happen to know Katharine Hepburn? I'm going to give you some medication, and you can go home and get used to the idea that eventually you're going to end up looking like Katharine Hepburn.' I was devastated," says Smith. Medication helped for the first few years. But Smith's tremor was still severe and like others who suffer from this disorder, the shaking worsened with simple movements or everyday tasks like applying makeup or pouring a glass of water. ©2016 CBC/Radio-Canada.

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity
Related chapters from MM:Chapter 5: The Sensorimotor System
Link ID: 22603 - Posted: 08.25.2016

In a global study of myasthenia gravis, an autoimmune disease that causes muscle weakness and fatigue, researchers found that surgical removal of an organ called the thymus reduced patients’ weakness, and their need for immunosuppressive drugs. The study, published in the New England Journal of Medicine, was partially funded by the National Institutes of Health. “Our results support the idea that thymectomy is a valid treatment option for a major form of myasthenia gravis,” said Gil Wolfe, M.D., Professor and Irvin and Rosemary Smith Chair of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, New York, and a leader of the study. The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) was a randomized, controlled study conducted on 126 patients aged 18-65 between 2006 and 2012. The researchers compared the combination of surgery and immunosuppression with the drug prednisone with prednisone treatment alone. They performed extended transternal thymectomies on 57 patients. This major surgical procedure aims to remove most of the thymus, which requires opening of a patient’s chest. On average the researchers found that the combination of surgery and prednisone treatment reduced overall muscle weakness more than prednisone treatment alone. After 36 months of prednisone treatment, both groups of patients had better QMG scores, a measure of muscle strength. Scores for the patients who had thymectomies and prednisone were 2.84 points better than patients who were on prednisone alone.

Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 15: Emotions, Aggression, and Stress
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 11: Emotions, Aggression, and Stress
Link ID: 22547 - Posted: 08.12.2016