TABLE 1.
Case 1 | Case 2 | Case 3 | |
---|---|---|---|
Age at death, years | 66 | 49 | 67 |
Cause of death | Respiratory insufficiency | Respiratory insufficiency | Cardiac arrhythmia |
Sports history | |||
Sport played | Football | Football | Boxing |
Total years played | 27 | 16 | 10 |
Years as a professional | 16 | 3 | 10 |
Age at retirement from sport, years | 37 | 36 | 22 |
Concussion history | |||
No. reported | >10 | 3–4 | “Many” |
Cervical spine injury history | |||
No. reported | Multiple | 0 | 0 |
Substance history | |||
Alcohol abuse | 0 | 0 | + in youth |
Illicit drug use | 0 | 0 | 0 |
Family history | |||
ALS | 0 | 0 | Sibling with probable ALS |
Dementia | 0 | Paternal grandmother with AD | 0 |
Motor neuron symptoms | |||
Age at onset, years | 64 | 47 | 67 |
Initial motor symptoms | Weakness of neck flexion, finger extension | Right arm and hand weakness, hyperactive DTRs, diffuse fasciculations | Hypophonic speech, drooling, severe atrophy of shoulder girdles and arms, diffuse fasciculations |
New symptoms 6 months after onset | Slurred spastic speech, decreased walking | Slow, slurred speech, swallowing difficulty, excessive emotionality, tongue weakness, slow gait, numerous falls, respiratory difficulty | Progressive muscular atrophy and speech impairment |
Clinical diagnosis | |||
ALS | ALS | Atypical ALS with dementia | |
Cognitive and behavioral changes | |||
Cognitive symptoms | Cognitive impairment | None | Dementia |
Age at symptom onset, years | 56 | NA | 64 |
Depression | 0 | ++ | 0 |
Outbursts of anger or aggression, “short fuse” | 0 | + | +++ |
Apathy | +++ | 0 | 0 |
0, absent; +, mild; ++, moderate; +++, severe.
AD, Alzheimer disease; ALS, amyotrophic lateral sclerosis; DTR, deep tendon reflexes; NA, not applicable.