Chronic Traumatic Encephalopathy Within an Amyotrophic Lateral Sclerosis Brain Bank Cohort

Abstract Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disorder linked to repetitive head impacts and has been associated with amyotrophic lateral sclerosis (ALS), a fatal, degenerative neuromuscular disorder. The Department of Veterans Affairs Biorepository Brain Bank (V...

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Veröffentlicht in:Journal of neuropathology and experimental neurology 2018-12, Vol.77 (12), p.1091-1100
Hauptverfasser: Walt, Gabriel S, Burris, Hannah M, Brady, Christopher B, Spencer, Keith R, Alvarez, Victor E, Huber, Bertrand R, Guilderson, Latease, Abdul Rauf, Nazifa, Collins, Derek, Singh, Tarnjit, Mathias, Rebecca, Averill, James G, Walker, Sean E, Robey, Ian, McKee, Ann C, Kowall, Neil W, Stein, Thor D
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Sprache:eng
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Zusammenfassung:Abstract Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disorder linked to repetitive head impacts and has been associated with amyotrophic lateral sclerosis (ALS), a fatal, degenerative neuromuscular disorder. The Department of Veterans Affairs Biorepository Brain Bank (VABBB) is a tissue repository that collects antemortem disease progression data and postmortem central nervous system tissue from veterans with ALS. We set out to determine the frequency of co-morbid ALS and CTE in the VABBB cohort and to characterize the clinical, genetic, and pathological distinctions between participants with ALS only and those with both ALS and CTE (ALS+CTE). Of 155 participants, 9 (5.8%) had neuropathologically confirmed ALS+CTE. Participants with ALS+CTE were more likely to have a history of traumatic brain injury (p < 0.001), served during the first Persian Gulf War (p < 0.05), and to have more severe tau pathology within the frontal cortex and spinal cord (p < 0.05). The most common exposures to head impacts included contact sports (n = 5) and military service (n = 2). Clinically, participants with ALS+CTE were more likely to have bulbar onset ALS (p = 0.006), behavioral changes (p = 0.002), and/or mood changes (p < 0.001). Overall, compared with ALS in isolation, comorbid ALS+CTE is associated with a history of TBI and has a distinct clinical and pathological presentation.
ISSN:0022-3069
1554-6578
DOI:10.1093/jnen/nly092