Case 12-1999

Abstract

A 67-year-old, right-handed man was admitted to the hospital because of increasing dementia.Three years before admission, the patient's cognitive function began to decline. One year later, a neurologist diagnosed progressive dementia with parkinsonism.During the two months before admission, the patient became agitated, and trazodone was prescribed. His family noted transient periods when he stared into space and did not respond to his surroundings. He fell several times, with apparent loss of consciousness but no obvious injury.One week before admission, the patient punched his wife because she would not let him drive. Soon thereafter, he began to look under his bed, locked the doors and windows, and became convinced that the neighbors were trying to steal the family's car. Subsequently, he struck his teenage grandson without provocation and wandered from home. He was referred to this hospital.The patient had boxed professionally for 10 years in over 100 bouts. He drank alcohol excessively in his youth but had been sober for several decades. There was no family history of psychiatric problems. A sibling had died 10 years earlier of probable amyotrophic lateral sclerosis.The temperature was 37°C, the pulse was 84, and the respirations were 16. The blood pressure was 145/80 mm Hg. The results of a general physical examination were normal.On neurologic examination, the patient was alert but was oriented only to himself. He believed that he was in his house in the city and that the year was 1970. He lost concentration when attempting serial tasks. Abstract tasks were performed poorly. He recalled none of three objects at five minutes, even with cues. Remote autobiographical memories were relatively preserved. Left-right confusion was present. The patient's language was intact, and phrases were repeated accurately. His speech was hypophonic. His facial expression was reduced, and he drooled. Cranial-nerve functions were preserved. There was severe atrophy of the shoulder girdles and arms and legs. Diffuse fasciculations and muscle twitches were observed. Motor power was normal. The patient's posture was slightly stooped. His spontaneous gait was normal, and tandem walking was unsteady. No resting tremor or cogwheel rigidity was detected. His sense of joint position and perception of vibration were intact. The deep-tendon reflexes were + or ++ and symmetric. No bradykinesia or postural instability was evident, and the glabellar-tap sign and Babinski signs were absent. The electrocardiographic and laboratory findings were normal.A magnetic resonance imaging (MRI) examination of the brain showed severe, diffuse ventricular and cortical sulcal enlargement (Fig. 1). T 2 -weighted images showed slight thinning of the corpus callosum, atrophy of the mamillary body, a moderately reduced signal in the globus pallidus and putamen, and minimal hyperintensity of periventricular white matter. The pars compacta was thinned on the proton-densityweighted images. A cavum septi pellucidi was noted. The major intracranial vessels were p...

Chronic neuropathologies of single and repetitive TBI: substrates of dementia?

“…This feature, which was present in 64 of 99 (65%) cases in which it was examined for (Table 1), is often accompanied by a fenestrated septum, communicating hydrocephalus and, in some instances, complete absence of the septum or its detachment from the fornix or corpus callosum. 17,19–21,24,25,29,31,32,52–61,63,69–71 In addition to these autopsy studies, various radiological investigations have confirmed the presence of CSP in vivo in boxers with neuropsychiatric symptoms. 32,35,55,72 However, as CSP can occur as a ‘normal’ observation in the general population, with estimates ranging from 1–28%, 18,73–76 the diagnostic utility of these findings has been questioned.…”

“…17,18,21,32,52–63 In addition, atrophy of the cerebellum, both generalized and focal, has been reported. 31,56 However, in cases of non-boxing athletes with CTE, regional atrophy has not been described at the same frequency as in boxers, with overt global atrophy being rarer still.…”

“…One proposal is that it develops as a consequence of cerebral atrophy and ventricular dilation, a frequent observation in boxers with CTE. 17,18,52–54,56,57,60,61,63 Alternatively, it may arise as a consequence of repeated transient increases in intracranial pressure, producing both the ventricular dilation and septal changes. 32 Of further note, periventricular midline structures are highly vulnerable to mechanical strain during trauma.…”

“…Regardless of these limitations, not all reported cases of CTE seem to be plaque- positive. 21,22,27,29,31,58,60,61,63,69–71,82,89 Notably, plaques in CTE are typically described as diffuse and do not display the histochemical or morphological features of the neuritic plaques that are characteristic of AD.…”

“…18–20,23,24,28,31, 52–54,56,59,61–63,71,108 In addition to neuronal dropout in the cerebral cortex, several reports describe specific regions or structures with notable cell loss, including the hippocampus, 18,53 locus coeruleus 18,19,54,61,63,108 and cerebellum. 18,19,23,24,31,52,53,56,71 Varying distributions of cell loss have been described, from widespread and diffuse, to more ‘patchy’ or selective changes.…”