National Institute of Neurological Disorders and Stroke Consensus Diagnostic Criteria for Traumatic Encephalopathy Syndrome
Douglas I. Katz, Charles Bernick, David W. Dodick, Jesse Mez, Megan Mariani, Charles H. Adler, Michael L. Alosco, Laura J. Balcer, Sarah J. Banks, William Barr, David L. Brody, Robert C. Cantu, Kristen Dams-O’Connor, Yonas E. Geda, Barry D. Jordan, Thomas W. McAllister, Elaine R. Peskind, Ronald Petersen, Jennifer V. Wethe, Ross Zafonte, Éimear M. Foley, Debra Babcock, Walter J. Koroshetz, Yorghos Tripodis, Ann C. McKee, Martha E. Shenton, Jeffrey L. Cummings, Eric M. Reiman, Robert A. Stern
Abstract
Objective To develop evidence-informed, expert consensus research diagnostic criteria for traumatic encephalopathy syndrome (TES), the clinical disorder associated with neuropathologically diagnosed chronic traumatic encephalopathy (CTE). Methods A panel of 20 expert clinician-scientists in neurology, neuropsychology, psychiatry, neurosurgery, and physical medicine and rehabilitation, from 11 academic institutions, participated in a modified Delphi procedure to achieve consensus, initiated at the First National Institute of Neurological Disorders and Stroke Consensus Workshop to Define the Diagnostic Criteria for TES , April, 2019. Before consensus, panelists reviewed evidence from all published cases of CTE with neuropathologic confirmation, and they examined the predictive validity data on clinical features in relation to CTE pathology from a large clinicopathologic study (n = 298). Results Consensus was achieved in 4 rounds of the Delphi procedure. Diagnosis of TES requires (1) substantial exposure to repetitive head impacts (RHIs) from contact sports, military service, or other causes; (2) core clinical features of cognitive impairment (in episodic memory and/or executive functioning) and/or neurobehavioral dysregulation; (3) a progressive course; and (4) that the clinical features are not fully accounted for by any other neurologic, psychiatric, or medical conditions. For those meeting criteria for TES, functional dependence is graded on 5 levels, ranging from independent to severe dementia. A provisional level of certainty for CTE pathology is determined based on specific RHI exposure thresholds, core clinical features, functional status, and additional supportive features, including delayed onset, motor signs, and psychiatric features. Conclusions New consensus diagnostic criteria for TES were developed with a primary goal of facilitating future CTE research. These criteria will be revised as updated clinical and pathologic information and in vivo biomarkers become available. bvFTD= : behavioral variant frontotemporal dementia; CTE= : chronic traumatic encephalopathy; DIAGNOSE CTE= : Diagnostics, Imaging, and Genetics Network for the Objective Study and Evaluation of CTE; MBI= : Mild behavioral impairment; NIBIB= : National Institute of Biomedical Imaging and Bioengineering; NINDS= : National Institute of Neurological Disorders and Stroke; PTSD= : posttraumatic stress disorder; RHI= : repetitive head impact; TBI= : traumatic brain injury; TES= : traumatic encephalopathy syndrome; UNITE= : Understanding Neurologic Injury and Traumatic Encephalopathy