Table 1

Demographic and clinical characteristics at each site of onset

Upper limbsLower limbsBulbarCombinedTotal
Age (years)61.4±9.160.5±11.163.1±6.5362.1±7.8361.6±9.24
Sex (M/F)32/1830/2215/178/885/65
FS-FE (months)15.5±11.815.9±9.4212.2±6.479.36±5.58*14.3±9.71
FS-SS (months)15.2±13.413.6±12.010.8±8.320.35±0.5012.8±12.1
MST (months)33322618**32
5 year (%)182116017
PS (%)26 (52)24 (46)4 (13)5 (31)59 (36)
  • U, L, B indicate numbers of patients in whom each function was normally preserved at the onset of respiratory symptoms.

  • Combined (C) type patients were diagnosed significantly earlier compared with those with limb onset (*C vs L, p<0.001; C vs U, p<0.001) and MST was shortest for C-type as compared to limb onset (**p=001). Bulbar onset showed less frequent preservation (13%) of lower motor neuron function at the appearance of respiratory symptom as compared to those with U (52%) and L (46%). Age, age at onset; B, bulbar symptoms; FRS-D, Amyotrophic Lateral Sclerosis Functional Rating Scale revised score at diagnosis; FS-FE, interval between first symptoms and first examination; FS-SS, interval between first symptoms and second symptoms; L, lower limb symptoms; MST, median survival time; 5 year (%), percentage of patients surviving >5 years for each type of onset; PS (%), number (percentage) of normally preserved sites (not number of patients) at each site of onset; U, upper limb symptoms.