The relationship between ventilatory function and cognitive and behavioral impairment in ALS

Figures & data

Figure 1 Study flow diagram.

Table 1 Patient characteristics.

	Median/n	IQR/% total
Age at onset (years)	61.6	55.0, 68.4
Bulbar onset	126	26.2
Female	229	47.6
Disease duration (months)	15	9, 25
NIV use at evaluation	89	18.5
ALSFRS-R	33	28,38
Bulbar subscore	9	7, 11
Resp. subscore	10	8, 12
Motor subscore	15	11, 18
Education (years)	14	12, 16
MIP	–43	–61, –28
FVC	77	59, 90

NIV: noninvasive ventilation; ALSFRS-R: Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised; MIP: maximal inspiratory pressure; FVC: forced vital capacity.

Table 2 ALS Cognitive Behavioral Screen scores, mean (SD).

Test	Total N= 480	FVC < 50% N= 79	FVC 50–80% N= 179	FVC ≥80% N= 222	Correlation with FVC	Correlation with MIP
Cognition total score	14.5 (3.4)	12.8 (4.4)	14.4 (3.2)	15.3 (3.0)	0.135**	−0.164**
Concentration	4.1 (0.9)	3.8 (1.3)	4.1 (0.8)	4.2 (0.8)	0.061	−0.065
Attention	3.0 (1.3)	2.7 (1.3)	3.0 (1.3)	3.1 (1.3)	0.068*	−0.110**
Tracking/monitoring	4.1 (1.2)	3.7 (1.5)	4.0 (1.2)	4.3 (1.1)	0.099**	−0.091*
Initiation/retrieval	3.3 (1.4)	2.6 (1.7)	3.3 (1.4)	3.6 (1.2)	0.168**	−0.199**
Behavioral inventory score	37.4 (7.7)	33.8 (7.8)	36.3 (8.4)	39.6 (6.2)	0.220**	−0.182**

FVC: forced vital capacity; MIP: maximal inspiratory pressure.

* p < 0.01.

** p < 0.001.

Table 3 Linear regression analysis.

	ALS-CBS cognition total score			Caregiver behavioral inventory
Variable	B (SE)	Std. B	p Value	B (SE)	Std. B	p Value
Female	0.391 (0.320)	0.057	0.218	–0.439 (0.763)	–0.028	0.565
Bulbar subscore	0.153 (0.059)	0.131	0.010	0.457 (0.143)	0.175	0.002
Motor subscore	–0.027 (0.031)	–0.043	0.382	0.160 (0.073)	0.114	0.029
Age at onset	–0.064 (0.015)	–0.197	<0.001	0.025 (0.038)	0.033	0.512
Education	0.226 (0.059)	0.175	<0.001	0.338 (0.139)	0.118	0.015
Symptom duration	0.000 (0.005)	0.002	0.967	0.006 (0.013)	0.023	0.645
NIV use at eval.	0.374 (0.430)	0.042	0.384	1.245 (1.028)	0.062	0.227
FVC	0.030 (0.008)	0.204	<0.001	0.069 (0.020)	0.203	<0.001
	ALS-CBS cognition total score			Caregiver behavioral inventory
Variable	B (SE)	Std. B	p Value	B (SE)	Std. B	p Value
Female	0.693 (0.326)	0.102	0.034	–0.175 (0.788)	–0.011	0.824
Bulbar subscore	0.158 (0.062)	0.136	0.011	0.497 (0.149)	0.191	0.001
Motor subscore	–0.007 (0.030)	–0.011	0.818	0.198 (0.072)	0.144	0.006
Age at onset	–0.057 (0.016)	–0.180	0.001	0.052 (0.041)	0.070	0.207
Education	0.205 (0.060)	0.162	0.001	0.338 (0.142)	0.120	0.017
Symptom duration	0.002 (0.006)	0.014	0.775	0.008 (0.013)	0.013	0.529
NIV use at eval.	0.140 (0.426)	0.016	0.742	0.487 (1.025)	0.024	0.635
MIP	–0.020 (0.008)	–0.151	0.009	–0.040 (0.019)	–0.133	0.033

B: linear regression coefficient; SE: standard error; Std. B: standardized linear regression coefficient.

Italic values emphasize significant values (p < 0.05) in the table.

Data availability statement

The data that support the findings of this study are available from the corresponding author, JSS, upon reasonable request.