Table 1.
Demographic, Autopsy and Family History Characteristics of Cases Studied
| Mean AOO* (years +/- 1SD) | 57.2 +/- 12.8 (range 8-82) | |
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| Mean AOD* (years +/- 1SD) | 63.0 +/- 11.8 (range 37-92) | |
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| M:F (% Male) | 1.24:1 (55.4%) | |
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| N | Percent | |
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| Total cases evaluated | 259 | |
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| Dementia | ||
| Present# | 64 | 24.7% |
| Absent | 188 | 72.6% |
| Unknown | 7 | 2.7% |
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| Autopsy diagnosis | ||
| Total | 91 | 35.0% |
| ALS | 44 of 91 | 48.3% |
| FTLD-U or FTLD-MND | 47 of 91 | 51.6% |
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| Ethnicity | ||
| Caucasian | 237 | 91.5% |
| Guam Chammoro | 5 | 5.0% |
| Asian | 3 | 1.2% |
| Black | 1 | 0.4% |
| Unknown | 13 | 1.9% |
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| Family History | ||
| Familial ALS (# without dementia) | 65 (39)¥ | 25.1% (60%)¥ |
| Family history of NDD other than ALS | 41 | 15.8% |
| Sporadic | 86 | 33.2% |
| Unknown or unavailable history | 67 | 25.9% |
*
Mean age of onset (AOO) of 149 clinical cases and mean age of death (AOD) of 88 autopsy cases with available information from total of 259 cases.
#
Dementia was predominantly of the frontotemporal dementia type
¥
The number of familial cases without any dementia is given in parenthesis as well as the percentage of the total familial cases represented by this subgroup.
NDD, neurodegenerative disorder; SD, standard deviation