Chapter 11. Motor Control and Plasticity

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By KATHRYN SHATTUCK After his short film screened at the Sundance Film Festival in 2008, a euphoric Simon Fitzmaurice was walking the snowy streets of Park City, Utah, when his foot began to hurt. Back home in Ireland that summer, by then dealing with a pronounced limp, he received a shattering diagnosis: motor neuron disease, or M.N.D. (more commonly known in the United States as A.L.S., or Lou Gehrig’s Disease), a neurological disorder that causes increasing muscle weakness and eventual paralysis and is, in most cases, fatal. The doctor gave Mr. Fitzmaurice, then 33, three or four years to live. That might have been the end of any normal existence. But Mr. Fitzmaurice, by his own measure a “bit of a stubborn bastard,” was determined to leave his wife, Ruth, and their two young sons — with a third on the way — a legacy other than self-pity. The result is Mr. Fitzmaurice’s first feature film, and perhaps his salvation — “My Name Is Emily.” The movie, which opened in limited release in the United States on Feb. 17, stars Evanna Lynch, the airy Luna Lovegood of “Harry Potter” fame, as a teenage outlier in both her Dublin foster home and high school who goes on the lam with her only friend (George Webster) to free her father (Michael Smiley) from a mental hospital. The film — with gorgeous scenes of Ms. Lynch plunged, nymphlike, into a cerulean sea or riding shotgun through the emerald countryside in a canary-yellow vintage Renault — won for best cinematography when it debuted at the Galway Film Fleadh in 2015. “I am not trying to prove anything,” Mr. Fitzmaurice wrote in an email, before quickly reconsidering. “Actually, I am trying to prove something. I remember thinking, ‘I must do this to show my children to never give up.’” Mr. Fitzmaurice was writing with his hands when he began the script for “My Name Is Emily.” By the time he was finished, he was writing with his eyes. © 2017 The New York Times Company

Keyword: ALS-Lou Gehrig's Disease
Link ID: 23275 - Posted: 02.24.2017

Laurel Hamers Clusters of a toxic bacterial protein have a surprising structure, differing from similar clumps associated with Alzheimer’s and Parkinson’s in humans, scientists report in the Feb. 24 Science. These clusters, called amyloids, are defined in part by their structure: straight regions of protein chains called beta strands, folded accordion-style into flat beta sheets, which then stack up to form a fiber. That definition might now need to be broadened. “All the amyloids that have been structurally looked at so far have certain characteristics,” says Matthew Chapman, a biologist at the University of Michigan in Ann Arbor who wasn’t part of the work. “This is the odd amyloid out right now.” In the human brain, misfolded proteins can form amyloids that trigger neurodegenerative diseases. But amyloids aren’t always a sign of something gone wrong — some bacteria make amyloids to help defend their turf. In Staphylococcus aureus, for example, the PSMα3 protein assembles into amyloids that help the bacteria kill other cells. Previous research suggested that PSMα3 clusters were like any other amyloid. But researchers using X-ray crystallography found that instead of straight beta strands, the PSMα3 fiber was made up of curly structures called alpha helices that resemble an old-fashioned phone cord. The helices still formed a familiar fiber shape just like the beta strands did, but the sheets making up that fiber were rippled instead of flat. |© Society for Science & the Public 2000 - 2017.

Keyword: Alzheimers
Link ID: 23274 - Posted: 02.24.2017

Daqing Li and Ying Li In 1969 Geoffrey Raisman, who has died aged 77, introduced the term “plasticity” to describe the ability of damaged nerve tissue to form new synaptic connections. He discovered that damaged nerves in the central nervous system (CNS) could be repaired and developed the theory that white matter (nerve fibres and supporting cells) is like a pathway – when it is disrupted by injury, such as spinal cord injury, growth of the regenerating fibres is blocked. In 1985 he described how olfactory ensheathing cells (OECs) “open doors” for newly formed nerve fibres in the nose to enter the CNS. Believing that reconstruction of the damaged pathway is essential to repair of the injured CNS and using the unique door-opening capability of OECs, in 1997, together with colleagues, Geoffrey showed that transplantation of OECs into the damaged spinal cord in experimental models repairs the damaged pathway and results in the regeneration of severed nerve fibres and the restoration of lost functions. The study led to a joint clinical trial with Pawel Tabakow and his team at Wroclaw Medical University, Poland. In 2014 the first patient with a complete severance of the thoracic spinal cord received transplantation of his own OECs. The operation enabled the patient, Darek Fidyka, to gain significant neurological recovery of sensation and voluntary movement. He can now get out of his wheelchair and ride a tricycle. The wider application of OECs has also been investigated. In 2012, with his team at University College London, collaborating with the UCL Institute of Ophthalmology and Southwest hospital, at the Third Military Medical University in Chongqing, China, Geoffrey described the protective effect of OECs in an experimental glaucoma model. The discovery has led to a plan to translate this research to clinical application which, it is hoped, will help many sufferers regain sight.

Keyword: Regeneration; Glia
Link ID: 23266 - Posted: 02.22.2017

By Jessica Hamzelou Three people with paralysis have learned to type by thought alone using a brain implant – at the fastest speeds recorded using such a system. Two have motor neurone disease, also known as ALS – a degenerative disorder that destroys neurons associated with movement – while the other has a spinal cord injury. All three have weakness or paralysis in all of their limbs. There is a chance that those with ALS will eventually lose the ability to speak, too, says Jaimie Henderson, a neurosurgeon at Stanford University Medical Center in California. People who have lost the ability to talk may be offered devices that allow them to select letters on a screen using head, cheek or eye movements. This is how Stephen Hawking communicates, for example. But brain-machine interfaces are also being developed in the hope that they may one day be a more intuitive way of communicating. These involve reading brain activity, either externally or via an implant embedded in the brain, and turning it into a signal that can be used to direct something in the environment. At the moment, these devices are a little slow. Henderson and his colleagues wanted to make a device that was quicker and easier to use than those currently in trials. © Copyright Reed Business Information Ltd.

Keyword: ALS-Lou Gehrig's Disease ; Robotics
Link ID: 23264 - Posted: 02.22.2017

Many people think of fish and seafood as being healthy. However, new research suggests eating certain species that tend to have high levels of mercury may be linked to a greater risk of developing amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. Questions remain about the possible impact of mercury in fish, according to a preliminary study released Monday that will be presented at the American Academy of Neurology's 69th annual meeting in Boston in April. Fish and seafood consumption as a regular part of the diet was not associated with ALS, the study said. "For most people, eating fish is part of a healthy diet," said study author Elijah Stommel of Dartmouth College in Hanover, N.H., and a fellow of the academy. In addition, the authors said their study does not negate the fact that eating fish provides many health benefits. Instead, it suggests people may want to choose species that are known to have a lower mercury content, and avoid consuming fish caught in waters where there is mercury contamination. The researchers stressed that more research is needed before fish consumption guidelines for neurodegenerative illness can be made. While the exact cause of ALS is not known, some previous studies have suggested the neurotoxic metal to be a risk factor for ALS, a progressive neurological disease. ©2017 CBC/Radio-Canada.

Keyword: ALS-Lou Gehrig's Disease ; Neurotoxins
Link ID: 23257 - Posted: 02.21.2017

By Timothy Revell It can be difficult to communicate when you can only move your eyes, as is often the case for people with ALS (also known as motor neurone disease). Microsoft researchers have developed an app to make talking with your eyes easier, called GazeSpeak. GazeSpeak runs on a smartphone and uses artificial intelligence to convert eye movements into speech, so a conversation partner can understand what is being said in real time. The app runs on the listener’s device. They point their smartphone at the speaker as if they are taking a photo. A sticker on the back of the phone, visible to the speaker, shows a grid with letters grouped into four boxes corresponding to looking left, right, up and down. As the speaker gives different eye signals, GazeSpeak registers them as letters. “For example, to say the word ‘task’ they first look down to select the group containing ‘t’, then up to select the group containing ‘a’, and so on,” says Xiaoyi Zhang, who developed GazeSpeak whilst he was an intern at Microsoft. GazeSpeak selects the appropriate letter from each group by predicting the word the speaker wants to say based on the most common English words, similar to predictive text messaging. The speaker indicates they have finished a word by winking or looking straight ahead for two seconds. The system also takes into account added lists of words, like names or places that the speaker is likely to use. The top four word predictions are shown onscreen, and the top one is read aloud. © Copyright Reed Business Information Ltd.

Keyword: ALS-Lou Gehrig's Disease ; Robotics
Link ID: 23248 - Posted: 02.18.2017

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

Keyword: Movement Disorders
Link ID: 23234 - Posted: 02.15.2017

Katherine Bourzac Kristopher Boesen, who broke his neck in a car accident, regained the ability to move his arms and hands after his spinal cord was injected with stem cells. Two years after having a stroke at 31, Sonia Olea Coontz remained partially paralysed on her right side. She could barely move her arm, had slurred speech and needed a wheelchair to get around. In 2013, Coontz enrolled in a small clinical trial. The day after a doctor injected stem cells around the site of her stroke, she was able to lift her arm up over her head and speak clearly. Now she no longer uses a wheelchair and, at 36, is pregnant with her first child. Coontz is one of stem-cell therapy's “miracle patients”, says Gary Steinberg, chair of neurosurgery at Stanford School of Medicine in California, and Coontz's doctor. Conventional wisdom said that her response was impossible: the neural circuits damaged by the stroke were dead. Most neuroscientists believed that the window for functional recovery extends to only six months after the injury. Stem-cell therapies have shown great promise in the repair of brain and spinal injuries in animals. But animal models often behave differently from humans — nervous-system injuries in rats, for example, heal more readily than they do in people. Clinical trial results have been mixed. Interesting signals from small trials have faded away in larger ones. There are plenty of unknowns: which stem cells are the right ones to use, what the cells are doing when they work and how soon after an injury they can be used. © 2016 Macmillan Publishers Limited,

Keyword: Regeneration; Stem Cells
Link ID: 23210 - Posted: 02.10.2017

Swedish researchers say a simple blood test is effective at differentiating symptoms of Parkinson's disease from similar disorders, but it isn't ready for clinical use. In its early stages, neurologists say Parkinson's is difficult to distinguish from rarer disorders, called atypical parkinsonian disorders. They have overlapping symptoms that tend to worsen more quickly and are more likely to lead to death. Researchers are on the hunt for biomarkers to help diagnosis these disorders. One potential biomarker, a nerve protein that can be detected when nerve cells die, is found in higher concentrations in spinal fluid collected by lumbar puncture. Now medical scientists have also found the protein in less invasive blood tests. For the study published in Wednesday's online issue of the journal Neurology, Dr. Oskar Hansson of Sweden's Lund University and his team examined 504 people in three groups. Two of the groups, in England and Sweden, included healthy people and those who had been living with one of the disorders for an average of four to six years. The third group of 109 patients had the diseases for three years or less. "The results of the present study strongly indicate that NfL when measured in blood can be used to distinguish between patients with Parkinson's disease and patients with progressive supranuclear palsy multiple system atrophy and corticobasal degeneration with high diagnostic accuracy," the study's authors said. ©2017 CBC/Radio-Canada.

Keyword: Parkinsons
Link ID: 23206 - Posted: 02.09.2017

Rae Ellen Bichell By the time Kay Schwister got her diagnosis last summer, she couldn't talk anymore. But she could still scowl, and scowl she did. After weeks of decline and no clue what was causing it, doctors had told Schwister — a 53-year-old vocational rehab counselor and mother of two from Chicago — that she had an incurable disease called Creutzfeldt-Jakob disease, or CJD. The disease was shrinking Kay's brain, and riddling it with holes. She would likely only live a few more weeks, the doctors said. It was a diagnosis that no one could ever want. But the fact that Schwister was able to get a firm diagnosis while still alive is a relatively new development that represents a step forward in understanding a group of devastating neurological disorders. And, some biochemists say, it could lead to better ways of diagnosing brain diseases that are much more common, including Parkinson's and Alzheimer's. For Kay Schwister it all started in the spring of 2016, when she started getting headaches and feeling dizzy all the time. Aging, she told herself, just didn't feel very good. Over the next few weeks, she got steadily worse. "She got to the point where she was so nauseous and so dizzy that she stopped driving and actually stopped working," says her husband, Tim Schwister. By the time Kay entered the emergency room last June her speech had changed. She was enunciating things in a strange way, and finishing each sentence on a really high note. Doctors drew blood and spinal fluid and tested it for things like multiple sclerosis and mercury poisoning. Those tests came back negative. Soon, Kay couldn't talk or walk. © 2017 npr

Keyword: Prions
Link ID: 23191 - Posted: 02.06.2017

Sara Constantino Certain multisensory conditions can alter the experience of bodily ownership. For instance, in the rubber hand illusion, simultaneous visual and haptic inputs lead to the adoption of sensations applied to an artificial limb as one's own. Understanding body ownership, and its malleability, has implications for the development of prosthetics. In a recent paper, Kelly Collins and colleagues at the University of Washington and Karolinska Institute elicited the illusion of ownership of an artificial hand in two epilepsy patients with embedded electrodes through the direct electrical stimulation of the hand area in somatosensory cortex (SI) applied in synchrony with visible touches to a rubber hand. When stimulation was asynchronous or administered to a different SI area, feelings of ownership were no longer induced, stressing the importance of temporal and spatial congruence. They also found that the details of the visual signal (for example, type of touch) affected the sensation. This method extends previous studies by eliciting ownership without stimulation of the peripheral nervous system, which is damaged in patients with spinal cord or nerve lesions. Human–technology mixtures have a long history, with the first known prosthesis, a wooden toe, dating as far back as 950 bc. Today, recent materials, electronics and neuroscience advances are enabling the development of prosthetic limbs that both look and feel real. © 2017 Macmillan Publishers Limited,

Keyword: Pain & Touch
Link ID: 23168 - Posted: 01.31.2017

By GRETCHEN REYNOLDS When people get up and move, even a little, they tend to be happier than when they are still, according to an interesting new study that used cellphone data to track activities and moods. In general, the researchers found, people who move are more content than people who sit. There already is considerable evidence that physical activity is linked to psychological health. Epidemiological studies have found, for example, that people who exercise or otherwise are active typically are less prone to depression and anxiety than sedentary people. But many of these studies focused only on negative moods. They often also relied on people recalling how they had felt and how much they had moved or sat in the previous week or month, with little objective data to support these recollections. For the new study, which was published this month in PLoS One, researchers at the University of Cambridge in England decided to try a different approach. They would look, they decided, at correlations between movement and happiness, that most positive of emotions. In addition, they would look at what people reported about their activity and compare it with objective measures of movement. To accomplish these goals, they first developed a special app for Android phones. Available free on the Google app store and ultimately downloaded by more than 10,000 men and women, it was advertised as helping people to understand how lifestyle choices, such as physical activity, might affect people’s moods. (The app, which is no longer available for download, opened with a permission form explaining to people that the data they entered would be used for academic research.) The app randomly sent requests to people throughout the day, asking them to enter an estimation of their current mood by answering questions and also using grids in which they would place a dot showing whether they felt more stressed or relaxed, depressed or excited, and so on. © 2017 The New York Times Company

Keyword: Depression; Obesity
Link ID: 23147 - Posted: 01.26.2017

By JANE E. BRODY Susan Sills, a Brooklyn artist who until recently made life-size cutouts on plywood using a power saw, long suspected she might be at risk for developing Parkinson’s disease. Both her mother and grandfather had this neurological movement disorder, and she knew that it sometimes runs in families. So she was not surprised when at age 72 she first noticed hand tremors and a neurologist confirmed that she had the disease. But to watch her in action three years later, it would be hard for a layperson to tell. She stands straight, walks briskly, speaks in clarion tones and maintains a schedule that could tire someone half her age. Having wisely put the power saw aside, Ms. Sills now makes intricately designed art jewelry. She is also a docent at the Brooklyn Museum, participates in a cooperative art gallery and assists her husband’s business by entertaining customers. Ms. Sills attributes her energy and well-being partly to the medication she takes but primarily to the hours she spends working out with a physical therapist and personal trainer, who have helped her develop an exercise regimen that, while not a cure, can alleviate Parkinson’s symptoms and slow progression of the disease. “The exercises opened me up,” said Ms. Sills, allowing such symptoms as small steps, slow movements and tiny, cramped handwriting to subside. “The earlier people begin exercising after a Parkinson’s diagnosis, and the higher the intensity of exercise they achieve, the better they are,” Marilyn Moffat, a physical therapist on the faculty of New York University, said. “Many different activities have been shown to be beneficial, including cycling, boxing, dancing and walking forward and backward on a treadmill. If someone doesn’t like one activity, there are others that can have equally good results.” © 2017 The New York Times Company

Keyword: Parkinsons
Link ID: 23132 - Posted: 01.23.2017

Charles Q. Choi Prions, the infectious agents best known for causing degenerative brain disorders such as ‘mad cow’ disease, may have been spotted in bacteria. A section of a protein in Clostridium botulinum, the microbe that causes botulism, can behave like a prion when it is inserted into yeast and Escherichia coli bacteria, researchers report in the 13 January issue of Science1. Prions are formed by proteins that can fold in a number of structurally distinct ways. A prion version of a protein can perpetuate itself in an infectious manner by converting normal forms of that protein into the prion version. Scientists first discovered prions in the 1980s as the agents behind fatal brain disorders known as transmissible spongiform encephalopathies. Since then, researchers have found the misfolded proteins in mammals, insects, worms, plants and fungi2, and learned that not all prions harm their hosts. But until now, prions were only seen in the cells of eukaryotic organisms, a group that includes animals, plants and fungi. In the latest study, researchers analysed roughly 60,000 bacterial genomes using software trained to recognize prion-forming proteins in yeast. They focused on a section of the bacterial protein Rho. In many bacteria, such as C. botulinum and E. coli, Rho is a global regulator of gene expression, meaning that it can control the activity of many genes. © 2017 Macmillan Publishers Limited,

Keyword: Prions
Link ID: 23104 - Posted: 01.14.2017

Parkinson’s disease, a chronic, progressive movement disorder characterized by tremors and stiffness, is not considered a fatal disease in and of itself, though it may reduce life expectancy by a modest amount. It is often said that people die “with” Parkinson’s rather than “of” the disease. “People who are healthy when diagnosed will generally live about as long as other people in their age cohort,” said James Beck, the vice president for scientific affairs at the Parkinson’s Disease Foundation, which is involved in research, education and advocacy. “It is not a death sentence.” Since Parkinson’s generally affects people later in life — patients are typically given a diagnosis in their 60s — patients often die of unrelated age-related diseases like cancer, heart disease or stroke. But the most common cause of death in those with Parkinson’s is pneumonia, because the disease impairs patients’ ability to swallow, putting them at risk for inhaling or aspirating food or liquids into their lungs, leading to aspiration pneumonia. Since Parkinson’s also impairs mobility and balance, those with the disease are also at high risk for falls and accidents, which can trigger a cascade of medical problems, including being bedridden and developing pneumonia, Dr. Beck said. In its advanced stages, the disease can make walking and talking difficult and cause other problems not related to movement, including cognitive impairment. Patients often cannot care for themselves and need assistance carrying out simple activities of daily living. One long-term study followed a group of 142 Parkinson’s patients after they were given their diagnosis; their mean age at diagnosis was around 70. The researchers found that 23 percent were generally doing well 10 years later, meaning they could maintain their balance and did not have dementia. But over half of the patients in the original group had died, with the most common cause related to Parkinson’s being pneumonia. © 2017 The New York Times Company

Keyword: Parkinsons
Link ID: 23094 - Posted: 01.13.2017

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

Keyword: Movement Disorders
Link ID: 23081 - Posted: 01.11.2017

By Anna Azvolinsky Hummingbirds are efficient hoverers, suspending their bodies midair using rapid forward and backward strokes. Aside from their unique ability to hover, the tiny avians are also the only known birds that can fly in any direction, including sideways. Hummingbird brains appear to be adapted for this flying ability, researchers have now shown. According to a study published today (January 5) in Current Biology, a highly conserved area of the brain—the lentiformis mesencephali (LM), which receives panoramic visual motion information directly from the retina—processes the movement of objects from all directions. In contrast, the LMs of other bird species and all other four-limbed vertebrates studied to date predominantly sense back-to-front motion. While the authors had predicted the neurons of this hummingbird brain region would be tuned to slow motion, they in fact found the opposite: LM neurons were sensitive to quick visual motion, most likely because hummingbirds must process and respond to their environments quickly to avoid collisions, both during hovering and in other modes of flight. “This ancient part of the brain the authors studied has one job: to detect the motion of the image in front of the eyes,” explained Michael Ibbotson, a neuroscientist at the University of Melbourne who penned an accompanying editorial but was not involved in the research. The results of this study suggest that “hummingbirds evolved this area of the brain to have fine motor control to be able to hover and push in every direction possible,” Ibbotson said. © 1986-2017 The Scientist

Keyword: Vision
Link ID: 23064 - Posted: 01.07.2017

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

Keyword: Movement Disorders; Genes & Behavior
Link ID: 23040 - Posted: 12.31.2016

Ian Sample Science editor The first subtle hints of cognitive decline may reveal themselves in an artist’s brush strokes many years before dementia is diagnosed, researchers believe. The controversial claim is made by psychologists who studied renowned artists, from the founder of French impressionism, Claude Monet, to the abstract expressionist Willem de Kooning. While Monet aged without obvious mental decline, de Kooning was diagnosed with Alzheimer’s disease more than a decade before his death in 1997. Strobe lighting provides a flicker of hope in the fight against Alzheimer’s Alex Forsythe at the University of Liverpool analysed more than 2,000 paintings from seven famous artists and found what she believes are progressive changes in the works of those who went on to develop Alzheimer’s. The changes became noticeable when the artists were in their 40s. Though intriguing, the small number of artists involved in the study means the findings are highly tentative. While Forsythe said the work does not point to an early test for dementia, she hopes it may open up fresh avenues for investigating the disease. The research provoked mixed reactions from other scientists. Richard Taylor, a physicist at the University of Oregon, described the work as a “magnificent demonstration of art and science coming together”. But Kate Brown, a physicist at Hamilton College in New York, was less enthusiastic and dismissed the research as “complete and utter nonsense”. © 2016 Guardian News and Media Limited

Keyword: Alzheimers
Link ID: 23033 - Posted: 12.29.2016

By DANNY HAKIM LONDON — Syngenta, the Swiss pesticide giant, claims on its website that data from an influential 2011 study shows that farmers who use the weed killer paraquat are less likely to develop Parkinson’s disease than the general population. However, Syngenta’s claim is at odds with the actual findings of the study, according to two of its authors. The 2011 study, carried out by the National Institutes of Health and researchers from other institutions around the world, found that people who used paraquat or another pesticide, called rotenone, were roughly two and a half times more likely to develop Parkinson’s. The work is known as the Farming and Movement Evaluation, or FAME, study. It drew on a sweeping United States government project called the Agricultural Heath Study, which tracked more than 80,000 farmers and their spouses, as well as other people who applied pesticides, in Iowa and North Carolina. The FAME researchers identified 115 people from the Agricultural Health Study who developed Parkinson’s, and studied 110 of them who provided information on the pesticides they used. The study was influential even among some people who had been skeptics of a connection between the chemicals and the disease. Gary W. Miller, a professor of environmental health at Emory University, referred to a link between Parkinson’s and paraquat as a “red herring” in a 2007 publication. But while Dr. Miller said in a recent email exchange that he had concerns about some previous research making the connection, “the FAME data are strong and should be considered.” He said the study “appears to show a connection between paraquat exposure and Parkinson’s disease.” Because of the prominence of the FAME study, Syngenta addresses it on one of its websites, paraquat.com. Syngenta claims that the study shows that only 115 people had Parkinson’s out of the more than 80,000 people in the broader Agricultural Health Study. Therefore, “the incidence of Parkinson’s disease” in the study “appears to be lower than in the general U.S. population,” Syngenta says. © 2016 The New York Times Company

Keyword: Parkinsons; Neurotoxins
Link ID: 23016 - Posted: 12.23.2016