Chronic Traumatic Encephalopathy. From ‘punch drunk’ to CTE; The names have changed, but the brain damage remains.

In September 2024, Miami Dolphin quarterback Tuo Tagovailoa “got his bell rung.” He was running for a first down when his helmet made contact with Buffalo Bill’s defensive quarterback, Damar Hamlin. He was knocked unconscious, and suffered his third concussion on the NFL football field. This time, while lying unconscious on his back, his arms temporarily went into the “fencing position” which lasted a few seconds with one arm flexed, and the other arm extended suggesting significant brain trauma.

Fencing Position, also known as the “en garde” position, is the stance used in the sport of fencing that involves, in part, the arm holding the weapon is extended forward, while the other arm is bent and held back for balance often at a 45 degree angle.

The National Football League’s Competition Committee has enacted rules, and equipment changes to reduce the number of brain injuries to players on the field, but the problem persists. What is worrisome is the force and the number of head traumas incurred by the players that result in acute and chronic brain injuries. Such injuries have led to a devastating condition, called Chronic Traumatic Encephalopathy.

Chronic Traumatic Encephalopathy (CTE)

CTE is the current terminology used to describe the chronic, neurodegenerative brain disease that worsens over time caused by repeated trauma to the brain. Traumatic brain injuries (TBI) have been in the scientific literature with different names since the 1920’s.

Dr. Harrison Martland, a pathologist, described the clinical picture of TBI as the “punch drunk” syndrome of boxers. In 1928, he identified ruptured blood vessels in different parts of the brain which he theorized contributed to the brain damage that led to the symptoms of prize fighters.

About a decade later, “dementia pugilistca,” which means boxer’s dementia, entered the medical literature’s vocabulary to be supplanted by chronic traumatic encephalopathy in the 1940’s.

Causes

CTE is caused by repeated blows to the head. Contact sports such as boxing and football traumatize the brain causing CTE symptoms. Any activity which forcefully and repetitively strikes the head can lead to this condition. The other aspect is the accumulation of the faulty, misshaped tau protein in the brain, which harms communications between neurons, the nerve cells in the brain.

Diagnosis

Dr. Bennet Omalu, a forensic neuropathologist working in Pittsburgh in 2005, conducted a postmortem autopsy on the brain of former Pittsburgh Steeler and K. C. Chiefs football Hall of Fame player, Mike Webster, in 2005. Webster played center during his sixteen year football career, and was probably hit in the head thousands of times. At autopsy, Dr. Omalu found a buildup of Tau protein in Webster’s brain, published his finding in the Journal of Neurosurgery, and called his discovery, Chronic Traumatic Encephalopathy.

Presently, the only definitive way to diagnose chronic traumatic encephalopathy is by post-mortem autopsy, The diagnosis of CTE requires the accumulation of Tau proteins that cluster around the brain’s blood vessels. This disease progresses to involve more areas of the brain with evidence of degeneration of brain tissue.

Symptoms

Chronic traumatic encephalopathy is a progressive disease. It may take months, or years after the last traumatic incident before symptoms appear. The symptoms vary depending on the parts of the brain involved, and whether the individual is in an early, or a late stage of disease.

Early on, the tau protein may be localized to a certain region of the brain. For example, the protein may accumulate around blood vessels in the brain’s cortex and the person may exhibit mild symptoms. As time goes on, the destructive protein spreads to other parts of the brain eliciting different and more severe symptoms.

Symptoms vary, but in general, CTE affects a person’s:

• Cognition: The individual may experience memory loss, difficulty with thinking, reasoning (solving problems. decision making), trouble planning, or paying attention.
• State of Mind: The person may be anxious, detached, or depressed
• Behavior: People may be aggressive, intolerant, have impulse control problems, or exhibit suicide tendencies. Several professional football players diagnosed with CTE have committed suicide.
• Motor Symptoms: People may experience problems with walking and balance. Also, they may show slow movement, shaking and trouble with speech—Parkinsonism.

Treatment

There is no cure for chronic traumatic encephalopathy, but there are symptomatic treatments. Medication have temporarily been used to help treat symptoms such as memory and thinking, and behavioral problems. Some people with CTE benefit from speech and language therapy, or physiotherapy depending on the symptoms.

Chronic traumatic encephalopathy is a disease that results mainly from sports-related trauma to the brain. It’s a progressive, neurodegenerative disease that is a death blow to the nerve cells of the brain. It’s also a death blow to families who watch, and care for a once strong, healthy young man become feeble, or abusive, or both. The names have changed, but the damage remains with no remedy in sight.

Addendum

CTE is caused by repeated head trauma. The initial symptoms generally occur in a person’s 40’s (forties).and are often problems with impulse control, aggression, impaired judgment, and reasoning. Definitive diagnosis is made after death with postmortem autopsy.

Alzheimer’s disease is not caused by head trauma. The initial symptoms typically presents in a person’s 60’s (sixties), and most often with memory complaints. Diagnosis is based on clinical evaluation, cognitive testing and imaging studies. Both diseases have abnormal tau protein. In the case of CTE, the distribution of tau is more pronounced around blood vessels (perivascular).

Glossary

Tonic Neck Reflex — Fencing Reflex: When an infant’s head is turned to one side, the arm on that side stretches out and the opposite arm bends up at the elbow. This is often called the fencing position. This reflex lasts until the baby is about 5 to 7 months old. In adults, a strong impact to the head (concussion) can elicit the fencing position.

Damar Hamlin: It’s a strange coincidence; in January 2023, Hamlin suffered a cardiac arrest after being tackled with a blow to the chest by an opposing football player; and he was the player who was involved with the Miami’s quarterback, Tuo Tagovailoa concussion.

Suicides. Several football players including Junior Seau, and Aaron Hernandez, took their own lives. Upon autopsy, they were found to have CTE.

References

1. Joseph R. Anticaglia, MD; Head Trauma: “Shake It and You Break It!” Traumatic Brain Injury; Doctors Column HC Smart, July 25, 2021
2. Abhinav R Changa, et al; Dr Harrison Martland and the history of punch drunk syndrome; Brain, December 28, 2017
3. Bennet Omalu et al; Chronic traumatic encephalopathy in a National Football League player; J Neurosurgery, July 2005
4. BU Research Center; What is CTE; Frequently Asked Questions
5. Christopher J. Inserra; Bradley W. DeVrieze; Chronic Traumatic Encephalopathy; StatPearls, August 7, 2023.
6. Joseph R. Anticaglia, MD; Damar Hamlin: Sudden Cardiac Arres (Commotio Cordis) and the Game of Life; HC Smart, January 15, 2023