Chronic traumatic encephalopathy is reported in 25 year old former American football player

A 25 year old man who was repeatedly concussed while playing American football as an amateur and subsequently developed learning and behavioral problems has been found at autopsy to have chronic traumatic encephalopathy, a neurodegenerative disorder associated with repetitive head injury.

The disorder, which currently can be diagnosed only on autopsy, has become a growing concern among young athletes and their families in the United States, owing to an increasing number of reports of it among older National Football League athletes and a heightened awareness of the frequency of concussions among young people playing contact and non-contact sports.

The report comes from the Understanding Neurologic Injury and Traumatic Encephalopathy (UNITE) study (www.bu.edu/alzresearch/ctecenter/cte-center-research/unite-study/), a project designed to develop consensus criteria for the pathological diagnosis of chronic traumatic encephalopathy, establish the relation between cumulative brain trauma and clinical symptoms, and identify neuroimaging signatures of the neuropathology.

Although chronic traumatic encephalopathy has been reported in young athletes before,1 the authors of the new study believe that theirs is the first report of a case in which neuropsychological testing had been done shortly before death, offering the opportunity to determine whether the man’s clinical course and testing results might indicate criteria that could be used to diagnose chronic traumatic encephalopathy while patients are alive.

The results of the testing and autopsy are described in a case report published online on 4 January in JAMA Neurology.2 Jesse Mez, assistant professor of neurology at Boston University School of Medicine in Boston, Massachusetts, was the paper’s lead author.

The young man, who had congenital bicuspid aortic valve and died of cardiac arrest secondary to Staphylococus aureus endocarditis, had played football for 16 years, beginning at age 6 and continuing into college. During that time he experienced more than 10 concussions, the first at age 8.

During his freshman year of college he had a concussion with momentary loss of consciousness and afterward began experiencing a variety of neurological symptoms, including ongoing headaches, insomnia, anxiety, and difficulty with memory and concentration. He was treated with cyclobenzaprine and topiramate, which had limited benefit.

Because of his symptoms he stopped playing football at the beginning of his junior season, began failing his courses, despite having been an above average student previously, and left college without obtaining a degree. After leaving college his symptoms persisted, which included apathy, anhedonia, feelings of worthlessness, and suicidal ideation. At age 23 he became verbally and physically abusive to his wife, on whom he became increasingly dependent

At age 24 he underwent neuropsychological testing that found that his intelligence was average but that he had impairment in executive function, needed for such tasks as problem solving, and impairment in learning, though what he did learn he was able to remember. Mez said that because this pattern was seen not only in chronic traumatic encephalopathy but in post-concussive syndrome and in depression it was not possible to make the diagnosis of chronic traumatic encephalopathy on the basis of these clinical findings alone.

At autopsy, however, his brain was found to have mild ventricular dilation and hippocampal atrophy; and hyperphosphorylated τ proteins consisting of neurofibrillary tangles, neurites, and astrocytes around small blood vessels were found at the sulcal depths of the frontal and temporal lobes. These lesions, considered pathognomonic for chronic traumatic encephalopathy in preliminary consensus criteria of the National Institute of Neurological Disorders and Stroke, were also found in the parietal lobes, entorhinal cortex, anterior hippocampus, hypothalamus, nucleus basalis of Meynert, substantia nigra, locus coeruleus, and median raphe, the researchers reported.

The researchers concluded, “While the case suggests that CTE [chronic traumatic encephalopathy] should be considered in the differential diagnosis of a young adult with extensive repetitive head impact exposure and persistent mood and behavioral symptoms, it does not allow us to infer the likelihood of CTE in this setting.”