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Locked-In syndrome

Locked-In syndrome is a condition in which a patient is aware and awake, but cannot move or communicate due to complete paralysis of the body. It is the result of a brain stem stroke in which the ventral part of the brain stem is damaged. It results in quadriplegia and inability to speak in otherwise cognitively intact individuals. Those with Locked-In syndrome may be able to communicate with others by coding messages by blinking or moving their eyes, which are not affected by the paralysis. more...

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Locked-in syndrome is also known as Cerebromedullospinal Disconnection, De-Efferented State, Pseudocoma, and ventral pontine syndrome. Unlike persistent vegetative state, in which the upper portions of the brain are damaged and the lower portions are spared, locked-in syndrome is caused by damage to specific portions of the lower brain and brainstem with no damage to the upper brain.

Patients who have Locked-In Syndrome are fully aware. They will know exactly where their arms and legs are, and unlike paralyzed patients, they can still feel sensations of pain and touch. Some patients may have the ability to move certain facial muscles. The majority of locked-in syndrome patients do not regain motor control, but several devices are available to help patients communicate.

Patients with locked-in syndrome report feeling mostly tranquil, and some report feeling a little sad. This is contrary to the panic and terror that would be expected in people who cannot move or speak. This finding indicates that emotions are due to interpretations of bodily sensations. Since those who are locked-in have no bodily feeling, the brain fails to receive feedback indicative of alarm.

Parisian journalist Jean-Dominique Bauby had a stroke in 1995, and when he awoke 20 days later he found that his body had all but stopped working: he could only control his left eyelid. By blinking his eye he dictated a word at a time and in this way he wrote The Diving Bell and the Butterfly.

This syndrome was a plot point of the CSI: NY series premiere episode "Blink."

The original version of this article contained text from the NINDS public domain pages on TBI at

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SECOND IMPACT SYNDROME: Sports Confront Consequences of Concussions
From USA Today (Society for the Advancement of Education), 5/1/00 by Jeffrey H. Tyler

EACH YEAR, more than 300,000 people suffer brain injuries while playing a sport, most of which are concussions. In football slang, players say a team member got "dinged" or had his "bell rung." Boxers who get stunned by a blow to the head may be described as "punch-drunk." What an athlete experiences during a concussion is actually a temporary, trauma-induced alteration in mental status. Many concussions occur without the individual ever losing consciousness, but researchers have determined that even a seemingly minor "bell-ringing" in the course of play can cause lasting physical and mental injury, or even result in death.

Sports concussions have become more publicized recently, as celebrity athletes announce early retirement, and tragic accidents and major lawsuits in sports are covered more closely by the media. Nevertheless, such injuries persist across the country in recreational and school sports where clear preventive guidelines for managing head injuries are slow to reach the field.

Studies released by the American Academy of Neurology and the National Brain Injury Association indicate that 10% of college and 20% of high school football players receive brain injuries in any given season. Most of these are transitory, but those who suffer a first concussion may be four times more likely to suffer a second than someone who has never had one.

Researchers have identified a serious series of brain injury events they call second impact syndrome (SIS). According to James P. Kelly, director of the Brain Injury Program at the Rehabilitation Institute of Chicago, SIS occurs when an athlete suffers a mild head injury, returns to play too soon, and suffers what may be a relatively minor second hit before the brain has fully healed. If the second injury occurs while the individual still has symptoms from the first impact, the result can be a rapid, catastrophic increase in pressure within the brain. Effects of SIS include physical paralysis, mental disabilities, and epilepsy. Death can occur approximately 50% of the time.

"SIS can affect anyone exposed to a mild or moderate concussion; there's no age discrimination," notes Kelly. "But it seems to affect teenagers more often because they are the least likely to report their injuries or take the time to recover from a concussion when they do get hurt. They head back into the game too soon, not fully aware of the risks they are taking."

It is unclear how many cases of SIS occur each year in the U.S. because the first injury is not usually reported to a physician. The Center for Disease Control counted 17 cases of SIS between 1992 and 1995, but experts believe that the tree numbers are higher. Most cases involve male adolescents or young adults, who received a second catastrophic concussion while participating in football, boxing, ice hockey, or snow skiing.

The case of Brandon Schultz, a high school football player from Anacortes, Wash., is a prime example of the devastating consequences of SIS. Schultz's recently resolved lawsuit against the Anacortes School District is the first of its kind to argue that a school district was negligent for failing to prevent an SIS injury.

On Oct. 25, 1993, just two weeks after his 16th birthday, Schultz's life changed forever when he made a tackle during the final seconds of the first half of a junior varsity football game. Viewed from the field, and by his parents' home video recording, the tackle looked harmless enough. It did not appear that Schultz made head-to-head contact with another player, let alone received a concussion.

Schultz was slow to get up, but did not appear to lose consciousness. After a minute or two, he stood and returned to the end zone for the halftime huddle. Schultz was able to walk and talk, telling his coach only that his head hurt. Ten minutes later, he collapsed into a fit of seizures before losing consciousness, the result of uncontrollable brain swelling. During this 10-minute period, Schultz's brain essentially exploded inside his skull.

What his parents' game tape did not show is that, one week earlier, Schultz was pulled from play following a concussion that left him momentarily unconscious. He had suffered headaches throughout the week and was held out of practice, but was never referred to a doctor by his coaches.

Schultz is now confined to a neurological facility in California and still suffers daily from the consequences of his injury. His physicians have described his condition as being locked in a state of permanent adolescence. His cognitive functions are impaired, and he requires almost constant supervision because of his ongoing behavioral difficulties. As a result of the many strokes suffered in the aftermath of his injury, Schultz also experiences motor control problems on his left side and must wear a brace to walk.

Seattle attorney Michael E. Nelson [one of this article's authors], himself a survivor of a serious brain injury, was able to negotiate a pre-trial settlement of Schultz's lawsuit. Nelson's legal argument was that the school district was liable for negligence when it failed to require Schultz to see a doctor after his first concussion. The school district's coaches regularly required a medical clearance following even minor orthopedic injuries, but did not do so in the case of Schultz's initial concussion.

The confidential settlement amount should provide the lifetime care required for Schultz. Experts have estimated that his lifetime care costs will exceed $12,000,000.

Many athletes and their parents do not grasp the risk of returning to sports while still suffering even mild concussion symptoms. Most concussions occur without a loss of consciousness, so players and their parents may not realize that a persistent headache indicates that the athlete's brain has not yet recovered from the first blow. The American Academy of Neurology and the Brain Injury Association have issued recommendations for return to play. The guidelines divide concussions into three types:

Grade I: No loss of consciousness; transient confusion; mental status abnormalities last less than 15 minutes. The athlete may play again that day if symptoms resolve within 15 minutes.

Grade II: No loss of consciousness; transient confusion; mental status abnormalities last more than 15 minutes. The athlete can play again only after he or she has been symptom-free for a full week.

Grade III: Any loss of consciousness, either for brief seconds or prolonged. An athlete who is unconscious for just a few seconds can resume play after a full week of no symptoms. If the loss of consciousness lasts several minutes or more, the waiting period is at least two weeks.

Researchers from the Henry Ford Health Systems, Detroit, Mich., have been working with the National Football League, National Hockey League, and National Collegiate Athletic Association to promote a new, more-individualized system to determine when an athlete who has suffered a concussion can safely return to competition. Currently, most physicians rely on various grading systems that rank symptoms and recommend varying lengths of symptom-free observation--ranging from 20 minutes to several weeks--before allowing an athlete to return to play.

According to Mark R. Lovell, head of the Ford Division of Neuropsychology, those guidelines no longer reflect the state of medical knowledge. "For years, our understanding of concussion has been modeled after boxing," thus placing the greatest weight on loss of consciousness. However, physicians have learned that "short-circuiting," which occurs when brain tissue slams against the inside of the skull, can often make itself known in a more gradual way. "We see players who weren't even close to being knocked out who later develop symptoms that suggest more severe concussions, and players who are knocked out who afterward have very little in the way of symptoms." The best way to tell if an athlete's mental processes have returned to normal, he argues, is to compare them with data from preseason tests of memory skills and reaction times.

Stephen Rice, a national expert on pediatric sports medicine at the Jersey Shore Medical Center in Neptune, NJ., argues that the best tool for preventing catastrophic brain injuries is seeing a doctor. In reviewing SIS incidents, he discovered there has never been a documented case of SIS when the injured player was referred to a physician after the first impact. According to Rice and other head injury experts, the referral of the concussion victim to a physician after first impact is an effective preventive measure.

To minimize future SIS tragedies, Nelson is pushing for standard head injury instructions for parents to use when their child suffers any sort of head injury. Nelson is also urging school districts to have sports officials use similar brain injury prevention guidelines and develop better health education programs for athletes and coaches.

"When a school district operates a sports program, it has the obligation to operate a safe program," says Rice. "Conversely, if a school district cannot operate a safe sports program, should it have the right to run a sports program at all?"

Pro athletes feel the blow

Concussion risks do not end with teenage athletes. In professional sports, participants can suffer the cumulative effect of repeated concussions, otherwise known as post-concussion syndrome (PCS). With each successive blow, the damaged brain takes longer to heal or never recovers to the pre-trauma state, so that each injury becomes more severe. As with SIS, players are much more likely to become impaired from a second injury following a previous concussion.

Veteran New York Ranger defenseman Jeff Beukeboom announced his retirement from hockey in July, 1999, after suffering three concussions in his last season. Beukeboom was the second Rangers player to retire in two years due to post-concussion syndrome, exiting on the heels of center Pat LaFontaine.

Beukeboom claims he suffered five to 10 concussions during his 13-year career. Since his last injury in February, 1999, he continues to experience headaches and loss of concentration in conversations. With a family that includes three small children, Beukeboom says that everyday tasks have become a chore and that his memory is spotty. He can no longer concentrate enough to train physically.

Multiple concussions, even when spaced several months or years apart, can have serious neurological consequences. Behavioral and magnetic resonance tests have proven that repeated concussions result in decreased mental performance, brain atrophy, and dementia. These physical features, normally associated with Parkinson's disease, were noted in boxers and termed Dementia Pugilistica.

Currently, the NHL and NFL are taking steps to understand and manage sports concussions better. An NHL official who would not comment on Beukeboom's retirement did maintain the league is at the forefront of all major sports by conducting mandatory baseline screening at training camp.

One clinical researcher agrees. "The NHL set a precedent, a truly great example for the rest of the professional sports industry to emulate," maintains Rosemarie Scolaro Moser, director of the RSM Psychology Center in Lawrenceville, N.J. "They provide a strong and comprehensive model that

educational institutions should also be judged by. The baseline screening may be the one tool we can use accurately to keep players from returning to their sport too soon. If we can keep them safe from a critical SIS injury or PCS, we can potentially prevent concussions and more serious brain damage."

Sensitive to the invisible symptoms of concussions, the NHL is taking precautions to help prevent them. LaFontaine and Philadelphia Flyers center Eric Lindros, whose brother Brett was forced to retire from the New York Islanders following repeated concussions, contributed to the development of shock-absorbing mouth guards and more-protective helmet designs to reduce susceptibility to concussions. Today, more players are voluntarily wearing mouth guards to decrease their risks. (Meanwhile, Eric Lindros' career is threatened by a series of concussions.)

In addition, the league's Board of Governors in 1999 ordered that all NHL arenas using seamless glass technology be fitted with a board system called CheckFlex, which provides more "give" on contact and could reduce concussions. Despite this step, some rinks still have not installed it.

The NFL has also lost its share of players to multiple concussions and is reacting. NFL Charities has funded a program at Thomas Jefferson University Hospital in Philadelphia to study Philadelphia Eagles players who sustain concussions during game actions. Researchers hope to examine eight players during the next two years.

"Ultimately, we want to use data from our study to construct a clinical examination that can be used right when a player comes to the sideline with a concussion during an actual game," indicates John McShane, team physician for the Eagles and director of primary care sports medicine at the hospital.

As part of the study, when someone sustains a concussion, he will first undergo magnetic resonance spectroscopy, which looks at brain metabolism and detects chemical markers that trace physical changes in the brain. Next, researchers will test coordination, reasoning, and concentration skills. The players will continue to undergo exams until the results return to normal. By correlating test results, researchers can determine when the ill effects of a concussion have disappeared.

The key to reducing an athlete's risk is for players, coaches, trainers, and parents to know the symptoms of concussion and how to manage them. The pro leagues have immense resources and team physicians to assess the symptoms on the sidelines. Many suburban high schools keep certified athletic trainers on site for games, and a number have doctors on the sidelines. Yet, a safety gap remains in many school systems and recreational leagues, where only a coach and players may be on hand during practices and games.

The American Academy of Neurology and the National Brain Injury Association offer the following guidelines for operating a high school or youth sports program focused on safe, proper brain injury management:

* Adequate education for coaches, including sufficient knowledge of head injuries and symptom management.

* Clearly defined standards regarding when and why to refer athletes to physicians.

* A comprehensive system for athletic health care, including these elements: a team physician; athletic trainers; formal education in sports medicine for coaches; informed consent by parents and athletes regarding risks of head injuries; head injury information sheets for parents; requiring that a player with a head injury visit a doctor and receive a return-to-play note; and developing clearly defined and appropriate criteria for returning to play after head injuries.

As the result of the Brandon Schultz lawsuit, the Anacortes School District has donated funds for his mother, Lane Phelan, to travel to school districts and brain injury organizations nationwide to educate parents and players about the dangers of concussion in sports. "I want parents and players to know that concussions are always serious--every concussion has the potential for a catastrophic outcome," she says. "When there are any signs of a concussion, whether it's just a headache or nausea, you need to seek medical attention."

Jeffrey H. Tyler and Michael E. Nelson are attorneys with Nelson Tyler Langer, a Seattle, Wash., law firm whose emphasis is traumatic brain and spinal injuries.

COPYRIGHT 2000 Society for the Advancement of Education
COPYRIGHT 2000 Gale Group

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