Table 1.
Baseline characteristics of participants according to treatment group shown in frequency (N) and percents, mean and standard deviations (SD), or median and inter-quartile ranges (IQR); Bulbar onset= symptoms of ALS beginning in the cranial nerves; BIPAP=Bilevel positive airway pressure; ALSFRS-R= ALS Functional Rating Scale-Revised; Duration=time (in months) from diagnosis to screening; FVC= Forced Vital Capacity (percent of predicted); BMI= body mass index; LDL= low-density lipoprotein; HDL= high-density lipoprotein; IL-6= interleukin-6 (normal range 1·2 to 15·3 pg/mL); hs-CRP= high sensitivity C-reactive protein (normal range 0·0–3·0 mg/L). Two HC/HC participants had laboratory-supported probable ALS and one participant in the control arm had lower motor neuron features only.
| P values | ||||||
|---|---|---|---|---|---|---|
| Baseline Characteristic of Participants |
Control N=7 | HC/HC N=9 | HF/HC N=8 | Overall | HC/HC vs. Cntl |
HF/HC vs. Cntl |
| Male N(%) | 6 (86%) | 4 (44%) | 3 (38%) | 0•16 | 0•15 | 0•12 |
| Age (mean±SD yrs) | 63•2±9•4 | 57•5±15•4 | 64•0±6•9 | 0•28 | 0•19 | 0•82 |
| White Not-Hispanic N(%) | 7 (100%) | 9 (100%) | 8 (100%) | >•99 | >•99 | >•99 |
| Probable or Definite ALS N(%) | 6 (86%) | 7 (78%) | 8 (100%) | 0•61 | >•99 | 0•47 |
| Bulbar Onset N(%) | 3 (43%) | 6 (67%) | 6 (75%) | 0•50 | 0•61 | 0•31 |
| Taking Riluzole N(%) | 4 (57%) | 6 (67%) | 7 (88%) | 0•47 | >•99 | 0•28 |
| ALSFRS-R (mean±SD) | 23•0±3•5 | 25•5±9•4 | 30•7±7•0 | 0•16 | 0•85 | 0•04* |
| Duration (median (IQR) months) | 14•9 (8•8, 27•3) | 10•9 (3•3, 23•3) | 16•7 (9•5, 19•1) | 0•77 | 0•49 | 0•91 |
| FVC (mean±SD % predicted) | 54•4±14•5 | 59•3±22•4 | 54•5±22•2 | 0•82 | 0•63 | 0•38 |
| Prescribed BIPAP N(%) | 5 (71%) | 4 (44%) | 5 (63%) | 0•59 | 0•36 | >•99 |
| BMI (mean±SD kg/m2) | 25•0±4•5 | 22•4±3•0 | 24•7±4•4 | 0•34 | 0•15 | 0•91 |
| Percent Lean Body Mass (mean ±SD) | 64•8±16•0 | 66•1±11•5 | 62•1±12•5 | 0•78 | 0•77 | >•99 |
| Percent Body Fat (Mean ±SD) | 29•7±11•6 | 30•2±11•4 | 34•5±13•2 | 0•69 | 0•91 | 0•46 |
| Percent body weight loss (mean ±SD) | 19•6±6•8 | 19•2±9•1 | 19•4±11•0 | 0•96 | 0•87 | 0•82 |
| Total Cholesterol (mean ±SD mg/dL) | 217•2±32•2 | 218•7±26•6 | 212•8±33•8 | 0•96 | 0•88 | 0•86 |
| LDL Cholesterol (mean ±SD mg/dL) | 135•0±30•5 | 126•3±26•8 | 110•8±31•6 | 0•35 | 0•46 | 0•23 |
| HDL Cholesterol (mean ±SD mg/dL) | 47•2±5•5 | 59•1±13•7 | 63•2±13•3 | 0•06 | 0•05 | 0•03* |
| LDL/HDL ratio (mean±SD) | 2•89±0•69 | 2•25±0•78 | 1•83±0•69 | 0•14 | 0•19 | 0•07 |
| Uric Acid (mean ±SD mg/dL) | 5•9±0•7 | 4•4±1•7 | 4•7±0•8 | 0•07 | 0•06 | 0•05 |
| Pre-albumin (mean ±SD mg/dL) | 31•7 ±4•0 | 31•8 ±1•8 | 31•4 ±9•3 | 0•82 | 0•79 | 0•65 |
| IL-6 (mean ±SD pg/mL) | 1•9±0•6 | 2•1±1•0 | 2•8±0•6 | 0•07 | 0•88 | 0•03* |
| hs-CRP (median (IQR) mg/L) | 0•7 (0•5, 3•0) | 1•8 (1•1, 9•3) | 2•9 (1•8, 6•3) | 0•16 | 0•13 | 0•09 |