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Table 1 Clinical characteristics of study subjects

From: Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients

  n Sex F/M Age at onset Age at biopsy ALSFRS Rate of progression FVC (%) BMI Onset S/B
Controls 91 0.88 60.3 (47–83) NA
sALS 171 0.67 58.3 (26–79) 59.7 (27–80) 34.2 (8–47) 1.0 (0.06–3.4) 76.1 (6–138) 26.4 (16.2–39.7) 1.98
PLS 34 0.79 51.7* (32–74) 59.2 (41–81) 33.2 (14–44) 0.2* (0.07–0.37) 89.6* (31–143) 26.9 (19–34.6) 2.67
C9Orf72 13 1.50 56.3 (40–70) 58.3 (38–72) 34.9 (30–41) 0.9 (0.24–2.0) 79.3 (38–115) 27.8 (20.5–50.4) 3.00
  1. Values indicate averages and values in brackets indicate ranges. Sex F/M, is the female to male ratio; ALSFRS, is the ALS functional rating scale at time of skin biopsy; Rate of progression is the % of ALSFRS decline per month; FVC is the forced vital capacity at time of skin biopsy expressed as % of normal; BMI is the body mass index at time of skin biopsy; Onset S/B is the ratio of site of disease onset, spinal (S) or bulbar (B). NA not available
  2. *p < 0.005 PLS vs. ALS, based on Mann-Whitney U test