Chronic traumatic encephalopathy
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Chronic traumatic encephalopathy is a neurodegenerative tauopathy that is thought to result from mild repetitive head trauma.
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The exact incidence and prevalence are unknown. It is most commonly seen in amateur and professional sports players where head contact is common (e.g. boxing, American football, rugby, ice hockey), as well as in military personnel exposed to explosive blasts 1,3.
Symptoms have an insidious onset, most often years after the initial injuries, with loss of normal attention, concentration and memory. This can progress, in some cases in 2-3 years, to include motor symptoms such as impaired gait, impaired, executive function, lack of insight and poor judgment 1,2,4.
Although the details remain to be elucidated, there is mounting evidence that reactive gliosis and redistribution of aquaporin 4 molecules, leading to disruption of transparenchymal CSF clearance of interstitial beta-amyloid via the glymphatic system may be a key component 8.
There is generalized cerebral atrophy with more pronounced atrophy of the frontal and temporal lobes (including mesial temporal lobe) as well as the thalamus, hypothalamus, and mammillary bodies. A cavum septum pellucidum may be present 2.
Chronic traumatic encephalopathy is characterized by the presence of neurofibrillary tangles and TDP-43 binding protein in subcortical and perivascular regions, often with reactive astrocytes and microglia 7.
Imaging features of chronic traumatic encephalopathy are non-specific, but the following may be seen 4,7:
generalized cortical atrophy
features of diffuse axonal injury (e.g. microhemorrhages)
History and etymology
This syndrome was first recognized in boxers in 1928, and has been known as "punch drunk" and "dementia pugilistica".
There is clinical and imaging overlap of chronic traumatic encephalopathy with other neurodegenerative diseases. Traumatic brain injury is a risk factor for their development (i.e. not just chronic traumatic encephalopathy) and should be considered in the differential diagnosis 5,6:
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- 2. Baugh CM, Stamm JM, Riley DO et-al. Chronic traumatic encephalopathy: neurodegeneration following repetitive concussive and subconcussive brain trauma. Brain Imaging Behav. 2012;6 (2): 244-54. doi:10.1007/s11682-012-9164-5 - Pubmed citation
- 3. Stern RA, Daneshvar DH, Baugh CM et-al. Clinical presentation of chronic traumatic encephalopathy. Neurology. 2013;81 (13): 1122-9. doi:10.1212/WNL.0b013e3182a55f7f - Free text at pubmed - Pubmed citation
- 4. Costanza A, Weber K, Gandy S et-al. Review: Contact sport-related chronic traumatic encephalopathy in the elderly: clinical expression and structural substrates. Neuropathol. Appl. Neurobiol. 2011;37 (6): 570-84. doi:10.1111/j.1365-2990.2011.01186.x - Free text at pubmed - Pubmed citation
- 5. McKee AC, Stern RA, Nowinski CJ et-al. The spectrum of disease in chronic traumatic encephalopathy. Brain. 2013;136 (1): 43-64. doi:10.1093/brain/aws307 - Free text at pubmed - Pubmed citation
- 6. Washington PM, Villapol S, Burns MP. Polypathology and dementia after brain trauma: Does brain injury trigger distinct neurodegenerative diseases, or should they be classified together as traumatic encephalopathy?. Exp. Neurol. 2015; . doi:10.1016/j.expneurol.2015.06.015 - Pubmed citation
- 7. Lucke-Wold BP, Turner RC, Logsdon AF et-al. Linking traumatic brain injury to chronic traumatic encephalopathy: identification of potential mechanisms leading to neurofibrillary tangle development. J. Neurotrauma. 2014;31 (13): 1129-38. doi:10.1089/neu.2013.3303 - Free text at pubmed - Pubmed citation
- 8. Iliff JJ, Nedergaard M. Is there a cerebral lymphatic system?. Stroke. 2013;44 (6, Supplement 1): S93-5. doi:10.1161/STROKEAHA.112.678698 - Free text at pubmed - Pubmed citation