How common is CTE? What we can learn from research on former NFL players

Boston University has found Chronic Traumatic Encephalopathy (CTE), a degenerative brain disease that has been linked to repetitive brain injuries, in the brains of 345 out of 376, or 92%, of former NFL players studied. While this is a striking and concerning number, it does not mean that 92% of all former NFL players have this disease. Let’s put this finding in context and discuss what it means for all contact sport athletes today.

What is CTE?

CTE is a degenerative brain disease that has been linked to exposure to repetitive brain trauma. That brain trauma can include both concussions and repetitive “subconcussive” impacts. The disease has been diagnosed in some individuals with no documented concussions but a history of repetitive head impacts that do not result in symptoms. Those repetitive impacts can occur on every play in many sports, including every tackle or collision between linemen in football, every check in hockey, and every header in soccer.

CTE has been diagnosed in individuals with a variety of exposure to repetitive brain trauma, including football, soccer, hockey, and rugby athletes, as well as military veterans and victims of domestic violence. CTE symptoms often begin in middle-age, sometimes years after the last exposure to brain trauma, and can include cognitive difficulties, memory loss, behavioral difficulties, impulsivity, and depression, among other symptoms.

At this time CTE cannot be diagnosed during life. It can only be diagnosed by postmortem examination of the brain. Generous donors and their families have donated their brain to brain banks studying the disease, such as the Boston University/Concussion Legacy Foundation Brain Bank, after they pass away. This incredibly gift from donors has lead to breakthroughs in research about the long-term consequences of repetitive brain trauma in sports, the military, and beyond.

Do 92% of NFL players have CTE?

The answer is almost certainly “no.” Brain banks have a selection bias. The sample they study is not random. Most players or their families don't think to donate their or their loved one's brain unless they think they have the disease. As a result, it is not surprising that many of the donors had CTE.

It is highly unlikely that ever other former NFL player that passed away during the same time period as those studied at Boston University had CTE. Thus, it is unlikely that 92% of all NFL players have the disease.

Despite the bias, the prevalence of CTE in NFL players is still likely high. After previous research showed 110 of 111 former NFLers examined had CTE, a study found that, if it is assumed that all other players who passed away in the same time period did NOT have CTE, the minimum prevalence rate would be 9.6%. That is about one in ten players, or about 5 players on every active NFL roster.

It is highly likely that some of the former players who passed away but were not studied did have CTE but were not diagnosed, making that prevalence even higher.

What does this mean for most current or former contact sport athletes?

We don’t know the true prevalence of CTE in the NFL population or in any contact sport athletes at any level. There is evidence that the risk for developing CTE increases with a greater number of repetitive head impacts over a lifetime. In one study, those who played tackle football for 14 years or more were ten times more likely to develop CTE, while those who played tackle football for 4 years or less were ten times less likely to develop the disease.

Still, there are cases of athletes who played contact sports only through the high school level that developed CTE. The youngest documented case I am aware of was in a 17-year-old. While the prevalence at this level is likely low, there is still risk.

For those who are concerned that they may have CTE based on their athletic history and current symptoms, CTE symptoms are not unique to CTE. Depression, certain cognitive difficulties, and anxiety, for example, can have many causes, and these symptoms can be treated.

As Dr. Ann McKee said in a Boston.com article:

“While the most tragic outcomes in individuals with CTE grab headlines, we want to remind people at risk for CTE that those experiences are in the minority,” Ann McKee, director of the BU CTE Center and chief of neuropathology at VA Boston Healthcare System, said in a statement. “Your symptoms, whether or not they are related to CTE, likely can be treated, and you should seek medical care. Our clinical team has had success treating former football players with mid-life mental health and other symptoms.”

If you are concerned that you or a loved one may have symptoms that may be related to CTE or previous concussions, you can contact the Concussion Legacy Foundation HelpLine. The Concussion Legacy Foundation has many resources available about CTE, Concussions, and Post-concussion syndrome. The HelpLine can provide referrals, online support groups, one-on-one peer support, and other resources.

Did Lou Gehrig Actually Have Lou Gehrig's Disease? Brain Trauma, ALS, and CTE with Motor Neuron Disease

On June 3, 1941, Lou Gehrig died at age 36 of what was thought to be amyotrophic lateral sclerosis, or ALS. The famous New York Yankee was forced to retire from baseball as a result of the disease two years earlier. His battle with ALS brought attention to this rare and poorly-understood disease, and since his death ALS has come to be known as “Lou Gehrig’s disease.”

But some experts now question whether or not Lou Gehrig actually had the disease that was named after him. There is now evidence of an ALS-like disease associated with chronic traumatic encephalopathy, or CTE, the neurodegenerative disease thought to be caused by repetitive brain trauma. Gehrig played fullback on the football team at Columbia University, and he had a long history of concussions, including several incidents in which he lost consciousness. Yet, he played through these injuries, setting a record for playing in 2,130 consecutive baseball games.

"Lou Gehrig 001" by rchdj10 is licensed under CC BY-ND 2.0

ALS and Brain Trauma

ALS is a neurodegenerative disease that affects both the neurons, or nerve cells, traveling from the motor parts of the brain to the spinal cord and those traveling from the spinal cord to innervate our muscles for voluntary movements. Both of these neurons in the motor pathway, or motor neurons, are necessary for our muscles to contract and allow us to move. When these neurons are affected in ALS, it causes muscle weakness and eventually paralysis because signals cannot get from the brain to the muscle to initiate movement. Eventually the muscles involved in critical functions such as swallowing and breathing become affected, eventually leading to death. The average survival time after diagnosis is around three years, though a small percentage of patients will live for decades with the disease.

ALS is a rare disease. Globally the prevalence is around 4.4 individuals per 100,000 people in the general population. There are several risk factors for ALS, including older age, male sex, and having a family history of the disease. However, around 90% of cases are sporadic in nature and not linked to a family history.

Another risk factor is a history of brain trauma. The odds of being diagnosed with ALS are around 38% higher in those who have a history of head injury compared to the general population. Those who have sustained multiple head injuries are at a slightly higher odds of developing ALS than those who experienced just one head injury.

Several studies show that the prevalence of ALS is higher in athletes who are exposed to repetitive brain trauma in their sport. Compared to the general population in the United States, mortality from ALS is more than four times higher in NFL football players. Several studies have shown that the odds of dying from ALS are two to ten times higher in professional soccer players in Europe. One study found that the longer a soccer player played professionally, the greater their risk of dying of ALS.

The increased risk of ALS in contact-sport athletes is striking, but also concerning is the age that the disease is diagnosed. In Europe the average age of diagnosis of ALS in the general population is around 65 years old. In one study of European professional soccer players, the average age of ALS diagnosis was 45 years old. Another study found that the diagnosis of ALS before age 49 was substantially higher in professional soccer players. Given the short life expectancy after diagnosis with ALS, having an average onset 20 years earlier than the general population means most of these athletes died years or even decades before the average age most people are diagnosed with the disease. This diagnosis is devastating at any age, but a diagnosis in a person’s 30s or 40s exceptionally tragic.

It’s not known exactly how brain trauma leads to an increased risk of ALS, but there is some evidence that blood-brain barrier disruption might play a role. The blood-brain barrier is a highly selective membrane that regulates the passage of molecules between the blood and the environment around the neurons in order to protect the neurons from potentially harmful substances. Disruption of this barrier that can occur with brain trauma leading to alterations in the environment around neurons could play a role in the development of ALS. Mouse models have also shown that brain trauma can trigger pathology involving a protein called TDP-43, which is found in ALS as well as many cases of CTE.

To be clear, a history of brain injury doesn’t make the risk of getting ALS high. It is still a rare disease even in those with a history of either repetitive or a single brain trauma. The studies of athletes have only been conducted in professional athletes, and the vast majority of athletes never reach that level. At this time it isn’t known whether or not the risk of developing ALS is higher in those who play sports that expose athletes to repetitive brain trauma only through the youth, high school, or even college level.

CTE-Motor Neuron Disease

While the risk of ALS appears to be higher in former professional football and soccer players, there is some question as to whether these athletes actually have ALS or another disease.

In 2010 Dr. Ann McKee and her colleagues at the Boston University Chronic Traumatic Encephalopathy Center published the first study showing a variant of CTE in former athletes that was similar to ALS. In these cases, pathology seen in the brain in CTE also affected the neurons in the spinal cord, leading to symptoms during life that appeared to be caused by ALS. The connection to CTE could only be seen with postmortem examination of the brain and spinal cord tissue.

The prevalence of both CTE and CTE with the motor neuron disease is currently unknown. Without the ability to diagnose the disease during life, it isn’t possible to know how many people have the disease. In postmortem studies of former football players, the motor neuron disease variant of CTE was present in around 6% to 12% of CTE cases. However, individuals or their families are more likely to donate their or their loved one’s brain and spinal cord to research if they think they may have a disease, making this a biased sample. Far more research is needed to determine how common CTE with the motor neuron disease is.

Still, CTE with the motor neuron variant raises questions about the ALS diagnoses in former professional athletes. It is possible that at least some of those athletes may have had CTE motor neuron disease and not ALS. Without examination of their brain and spinal cord after death, there is no way for us to know.

And that bring us back to Lou Gehrig. It is clear that a disease with ALS symptoms took his life, but the underlying pathology that caused his symptoms has been questioned by experts in recent years. Given his long history of brain trauma, it is possible that he may not have had ALS, the disease that is named after him, he may have had CTE with the motor neuron disease. But without the ability to examine his brain and spinal cord, we will never know.