Table 3.

Demographic and clinical features of non-tracheostomized prevalent cases on January 1 2015 and January 1 2019

	Prevalent cases on January 1 2015 (n.121)	Prevalent cases on January 1 2019 (n.124)	p
Sex (men/women)	77/44	76/48	0.8
Mean age at diagnosis (years) + SD	65.2 ± 12.5	61.9 ± 11.1	0.02
N. patients aged 65 or more at the prevalence date (%)	76 (62%)	97 (53%)	0.01
Mean diagnostic delay (months) + SD	20.5 ± 19.8	18.5 ± 16.4	0.2
Clinical phenotype at onset
Classic limb onset	47	50	0.81
Bulbar onset	22	20	0.61
Flail arm	16	22	0.33
Flail leg	26	15	0.05
Predominantly upper motor neuron	8	13	0.31
Pure upper motoneuron	0	0	NA
Pure lower motoneuron	2	2	0.96
Respiratory	0	2	0.17
Unknown	0	0	NA
Family history of
Amyotrophic lateral sclerosis	22	28	0.27
Dementia/parkinsonism alone	12	15	0.36
Genetic analysis
C9orf72	6	8	0.45
TARDPB	40	28
C9orf72 and/or TARDPB	3	2
PEG at prevalence date	8	12	0.4