Table 2.

Mean ± standard deviation of each short chain fatty acid concentration in patients and healthy controls. ALS= amyotrophic lateral sclerosis. HC = healthy controls. EEC = EL Escorial Criteria; EEC-R0 = definite; EEC-R1 = clinically probable; EEC-R2 = probable-laboratory-supported; EEC-R3 = possible. Bp = Bulbar phenotype; Cp = Classic phenotype; Fp = Flail arm/leg phenotype; UMNp = Upper Motor Neuron predominant phenotypes.

Condition		Acetic Acid (umol/g)	Propionic Acid (umol/g)	Butyric Acid (umol/g)	Iso-Butyric Acid (umol/g)	Iso-Valeric Acid (umol/g)	Valeric Acid (umol/g)	Total (umol/g)
HC		27.05 ± 10.59	8.79 ± 3.48	7.28 ± 3.99	1.43 ± 0.81	1.15 ± 0.89	1.28 ± 0.40	54.22 ± 19.88
ALS (all patients)		22.89 ± 13.9	8.05 ± 5.03	8.80± 6.92	1.49 ± 1.28	0.95 ± 1.23	1.36 ± 1.21	52.93 ± 34.23
ALS Subgroups
El Escorial Criteria	EEC-R0	17.91 ± 11.06	5.72 ± 4.78	6.79 ± 6.19	1.05 ± 1.48	0.95 ± 1.31	1.07 ± 1.28	37.73 ± 31.80
	EEC-R1	15.76 ± 12.06	7.83 ± 4.22	4.84 ± 6.42	1.03 ± 1.30	0.85 ± 1.49	1.72 ± 1.46	35.62 ± 30.28
	EEC-R2	2.80	1.54	0.69	0.51	0.63	0.30	7.10
	EEC-R3	27.22 ± 17.27	5.75 ± 6.67	7.81 ± 8.67	1.76 ± 1.23	1.61 ± 1.09	1.13 ± 0.88	53.18 ± 41.38
Clinical phenotype	Cp	16.90 ± 16.54	7.83 ± 4.92	6.05 ± 7.38	0.65 ± 1.19	0.54 ± 0.98	1.25 ± 1.19	37.07 ± 37.51
	Bp	17.68	5.72	4.54	0.35	0.28	1.07	34.08
	Fp	22.81 ± 5.27	5.25 ± 0.71	6.38 ± 1.99	1.76 ± 0.50	1.61 ± 0.49	1.13 ± 0.19	45.74 ± 10.93
	UMN-p	20.00 ± 15.20	12.17 ± 6.83	15.38 ± 8.49	3.07 ± 1.53	2.70 ± 1.56	2.68 ± 1.67	75.16 ± 42.11
Progression rate	Slow	19.50 ± 12.37	6.07 ± 4.62	5.85 ± 6.38	1.40 ± 1.12	1.28 ± 1.04	1.26 ± 1.11	41.08 ± 31.99
	Medium	17.91 ± 13.28	7.48 ± 3.41	6.79 ± 5.34	1.35 ± 0.46	1.12 ± 0.44	1.67 ± 0,78	37.73 ± 27.31
	Fast	18.84 ± 17.09	8.78 ± 6.28	9.09 ± 8.40	0.69 ± 1.69	0.56 ± 1.64	1.05 ± 1.54	54.62 ± 41.77