Table 1.

Demographic features of clinical patients with PMA and ALS

	Clinical PMA	Clinical ALS	p Value
Number of patients	14	93
Age at onset (years, mean±SD)	60.8±10.8	63.0±12.7	0.388*
Male/female	10/4	57/36	0.563†
Duration from onset to death (months; median, range)‡	21 (5–192)	29 (2–348)	0.764*
Initial symptoms (number of patients)
Bulbar symptoms	3 (21.4%)	23 (24.7%)	0.738†
Upper limb weakness	5 (35.7%)	38 (40.9%)	0.738†
Lower limb weakness	5 (35.7%)	30 (32.3%)	0.738†
Respiratory symptoms	1 (7.1%)	2 (2.2%)

*Mann-Whitney U test.

†Fisher's exact test.

‡Including the TPPV treatment period.

ALS, amyotrophic lateral sclerosis; PMA, progressive muscular atrophy; TPPV, tracheostomy positive-pressure ventilation.