Table 1 Demographic and disease-related variables in the ALS autopsy cohort

DiagnosisNo.Age, y, mean (SE)Sex ratio, M:FAge at onset, y, mean (SE)Disease duration, mo, mean (SE)Interval from last assessment, d, range
All comparisons between disease categories are nonsignificant.
* Two cases with a pure lower motor neuron (LMN) disorder were excluded from the analysis because of the absence of typical ALS-type ubiquinated LMN inclusions in LMN.
PMA = progressive muscular atrophy; PMA→ALS = PMA presentation later developing typical ALS.
PMA*1064.3 (3.1)6:463.0 (3.2)16.2 (1.9)2–90
PMA→ALS668.8 (5.7)4:266.7 (5.7)25.7 (5.9)
ALS6364.5 (1.4)32:3163.4 (1.6)22.5 (1.8)