Table 1.

Demographic and clinical features of amyotrophic lateral sclerosis prevalent cases on January 1 2015 and January 1 2019

	January 1 2015 (n. 147)	January 1 2019 (n. 170)	p
Sex (men/women)	93/54	103/67	0.9
Mean age at diagnosis (years) ± SD	64.4 ± 11.9	61.6 ± 11.4	0.02
N. patients aged 65 or more at the prevalence date	103 (70%)	97 (53%)	0.02
Mean diagnostic delay (months) ± SD	18.3 + 18.5	16.7 + 15.1	0.2
Clinical phenotype at onset
Classic limb onset	65	66	0.33
Bulbar onset	25	28	0.89
Flail arm	18	27	0.35
Flail leg	28	24	0.24
Predominantly upper motor neuron	8	15	0.25
Pure upper motoneuron	0	0	NA
Pure lower motoneuron	2	3	0.77
Respiratory	1	3	0.63
Unknown	0	4	0.1
Family history of
Amyotrophic lateral sclerosis	24	36	0.27
Dementia/parkinsonism alone	13	21	0.36
Genetic analysis
C9orf72	10	18	0.45
TARDPB	44	55
C9orf72 and/or TARDPB	3	3
Tracheostomy at prevalence date	26	46	0.047