A 63-year-old man presented with progressive memory, concentration, mood, and behavior problems over several years. He was a veteran of the army for six years in his twenties, and reported being “knocked out” or “dazed” more than a dozen times while in combat. In the army he also boxed competitively for several years and frequently had his “bell rung,” although he reported only being knocked unconscious five times. After the army he married and owned a successful small business. For the past 4–5 years his wife noted that he became increasingly withdrawn and moody. After making several poor financial decisions he closed down his business. In the last three years he began very uncharacteristic behaviors including yelling and “flying off the handle” over minor issues. His primary care physician referred him to a psychiatrist who prescribed fluoxetine, which improved his mood and anger. Two years ago his mood and anger worsened, and increasing doses of fluoxetine and other selective serotonin reuptake inhibitors (SSRIs) could not control the symptoms; after an episode of physical aggression toward his wife risperidone was prescribed. In the past year he had clear-cut impairment of his concentration, memory, and function. His wife notes that he now has “no short-term memory” and that he “does nothing” all day long. Neurological examination revealed mild bilateral 3–4 Hz tremor present at rest and with action. He scored 15 on the MoCA, making errors on the visuospatial/executive, attention, delayed recall, and orientation sections. Head CT revealed atrophy (most notably in hippocampi and frontal lobes, bilaterally) and a cavum septum pellucidum.
Definition and etiology |
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Cognitive and behavioral symptoms, in order of prevalence at presentation |
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Summary of diagnostic criteria | General Criteria for Traumatic Encephalopathy Syndrome: All five criteria must be met:
Core Clinical Features of Traumatic Encephalopathy Syndrome: At least one must be met:
Supportive Features of Traumatic Encephalopathy Syndrome: At least two must be present: (1) Impulsivity, (2) Anxiety, (3) Apathy, (4) Paranoia, (5) Suicidality, (6) Headache, (7) Motor Signs, including dysarthria and features of parkinsonism, (8) Documented Decline, for a minimum of one year, (9) Delayed Onset, at least two years. Traumatic encephalopathy syndrome diagnostic subtypes: (1) Behavioral/Mood Variant, (2) Cognitive Variant, (3) Mixed Variant, (4) Dementia. |
Imaging findings |
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Treatment |
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Top differential diagnoses |
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Prevalence, Definition, Pathology, and Pathophysiology
There is increasing evidence that head trauma may be associated with encephalopathy and dementia later in life. The exact pathogenesis of how head trauma at one point in life can cause dementia decades later is complex and is still being determined. We do know that, in addition to causing an encephalopathy that is maximal at the time of the head injury and generally improves (though not necessarily back to baseline), repetitive head injury has been associated with chronic traumatic encephalopathy, a progressive tauopathy with distinctive clinical and pathological features ( ) (see Table 13-1 and Fig. 13-1 ).
Stage | Pathology | Clinical Symptoms and Signs |
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I | Perivascular phospho-tau neurofibrillary tangles in focal epicenters at the depths of the sulci in frontal cortex | Headache, loss of attention and concentration |
II | Stage I plus neurofibrillary tangles in superficial cortical layers adjacent to the focal epicenters and in the nucleus basalis of Meynert and locus coeruleus | Depression and mood swings, explosivity, loss of attention and concentration, headache, and short-term memory loss |
III | Stage II plus mild cerebral atrophy, septal abnormalities, ventricular dilatation, concave third ventricle, depigmentation of locus ceoruleus and substantia nigra, dense phospho-tau pathology in the cortex, medial temporal lobe, diencephalon, brainstem, and spinal cord | Cognitive impairment with memory loss, executive dysfunction, loss of attention and concentration, depression, explosivity, and visuospatial abnormalities |
IV | Stage III plus further cerebral, medial temporal lobe, hypothalamic, thalamic, and mammillary body atrophy, septal abnormalities, ventricular dilation, and pallor of substantia nigra and locus coeruleus; phospho-tau in widespread regions including white matter, with prominent neuronal loss, gliosis of cortex, and hippocampal sclerosis | Dementia with profound short-term memory loss, executive dysfunction, attention and concentration loss, explosivity, and aggression. Most also show paranoia, depression, impulsivity, and visuospatial abnormalities. Many also have parkinsonism, speech, and gait abnormalities |