Case Records of the Massachusetts General Hospital: Dementia Pugilistica

abstract & commentary

Synopsis: The dementia typically seen in fighters is contrasted to other disorders, such as Alzheimer’s disease and Parkinsonism.

Source: Drachman DA (reviewer), Scully RE (ed). Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 12-1999. A 67-year-old man with three years of dementia. N Engl J Med 1999; 340:1269-1277.

The weekly clinical pathological exercise in this issue of the New England Journal of Medicine focuses on a case of dementia occurring in a 67-year-old man whose cognitive function began to decline three years prior to presentation at the Massachusetts General Hospital (MGH). One year later, he was diagnosed as having progressive dementia with Parkinsonism. Two months before his admission, he became agitated and was staring off into space and not responding to his surroundings. He fell several times and apparently lost consciousness. One week before admission, he became very irritable, punched his wife, and struck his teenage grandson without provocation.

The past medical history was significant, as the patient had boxed professionally for more than 10 years in more than 100 bouts. Indeed, in one fight he was knocked down 13 times. After retiring from fighting, he functioned well for almost 40 years with no sign of a neurological disease or dementia.

In his review, Drachman outlined an extensive differential diagnosis of dementia and concluded that this patient’s clinical picture was most consistent with the diagnosis of dementia pugilistica, even though there was a long period of normal functioning after his retirement. He points out that up to 87% of professional boxers develop some type of neurological impairment, 17% have clinically important neurological deficits, and about 6% have severe dementia, particularly frequent in those who have boxed in more than a dozen bouts. The patient died suddenly of an aspiration pneumonia while hospitalized. The anatomic pathology was characterized by extensive neurofibrillary degeneration of the nuclei in the brain stem, the cerebellum, and the cerebral hemispheres. Extensive changes in the frontal and temporal lobes characterized by neurofibrillary changes point to the anatomic diagnosis of dementia pugilistica.

Comment by James D. Heckman, MD

The case records of the MGH always provide interesting and challenging clinical cases. The discussion of this particular case provides a nice overview of the dementias and their differential diagnosis. Drachman contrasts the dementia typically seen in fighters to other disorders, such as Alzheimer’s disease and Parkinsonism. Several interesting references are cited, which describe the long-term effects of repeated boxing exposure.