Table 2

Association of cerebrospinal fluid tau proteins with clinical disease parameters reported in patients with ALS

Total tau (t-tau)
Disease parameters			Positive (↑)/inverse(↓) association, reference		No association, reference
Clinical measures of disease severity	ALSFRS-R		↓	Steinacker et al 7 Grossman et al 39	Lanznaster et al 41 Li et al 37 Scarafino et al 16 Schreiber et al 13 Brettschneider et al 19
	LMN	Medical Research Council (MRC) scale			Scarafino et al 16 Brettschneider et al 19
	Sniff nasal inspiratory pressure		↓	Scarafino et al 16
	Cognitive performance				Grossman et al 39
Measures of disease pathology	UMN	Corticospinal tract integrity in MRI			Steinacker et al 7
Measures of disease pathology	LMN	Compound motor action potential, cross-sectional nerve area			Schreiber et al 13
Speed of progression	DPR		↑	Scarafino et al 16	Grossman et al 39 Schreiber et al 13
	FRS variation (ALSFRS-R at 6 or 9 months—ALSFRS-R at baseline)		↓	Lanznaster et al 41
	Diagnostic delay		↓	Scarafino et al 16
	Time to generalisation				Scarafino et al 16
	MRCs decline				Brettschneider et al 19
Survival	Survival		↓	Scarafino et al 16	Schreiber et al 13 Lanznaster et al 41
phosphorylated tau (p-tau)
Clinical measures of disease severity	ALSFRS-R		↓	Grossman et al 39	Lanznaster et al 41 Li et al 37 Scarafino et al 16 Schreiber et al 13 Steinacker et al 7
	LMN	MRC scale			Scarafino et al 16
	Cognitive performance		↑	Grossman et al 39
Measures of disease pathology	UMN	Corticospinal tract integrity in MRI			Steinacker et al 7
Measures of disease pathology	LMN	Compound motor action potential, cross-sectional nerve area			Schreiber et al 13
Speed of progression	DPR				Grossman et al 39 Scarafino et al 16 Schreiber et al 13
	FRS variation		↑	Lanznaster et al 41
	Diagnostic delay				Scarafino et al 16
	Time to generalisation				Scarafino et al 16
Survival	Survival				Lanznaster et al 41 Schreiber et al 13 Scarafino et al 16
p-tau/t-tau ratio
Clinical measures of disease severity	ALSFRS-R			Lanznaster et al 41	Schreiber et al 13 Scarafino et al. Grossman et al 39
	No. of regions with UMN and/or LMN affection				Abu-Rumeileh et al 11
	UMN	Penn UMN score	↓	Schreiber et al 13
	UMN	No. of regions with UMN degeneration			Abu-Rumeileh et al 11
	LMN	MRC scale			Scarafino et al 16
	LMN	No. of regions with LMN degeneration			Abu-Rumeileh et al 11
	Sniff nasal inspiratory pressure		↑	Scarafino et al 16
	Cognitive performance		↑	Grossman et al 39
Measures of disease pathology	UMN	Corticospinal tract integrity in MRI		Schreiber et al 13 Grossman et al 39
	UMN	Whole brain grey matter atrophy	↑	Schreiber et al 13
	LMN	Compound motor action potential, sonographic cross-sectional nerve area			Schreiber et al 13
Speed of progression	DPR		↑	Abu-Rumeileh et al 11 Scarafino et al 16	Grossman et al 39 Schreiber et al 13
	FRS variation		↑	Lanznaster et al 41
	Diagnostic delay		↑	Scarafino et al 16
	Time to generalisation				Scarafino et al 16
Survival	Survival		↑	Abu-Rumeileh et al 11	Lanznaster et al 41 Schreiber et al 13 Scarafino et al 16

ALS, amyotrophic lateral sclerosis; ALSFRS-R, ALS functional rating scale-revised ; DPR, disease progression rate; LMN, lower motor neurons ; UMN, upper motor neurons .