A cross-sectional controlled developmental study of neuropsychological functions in patients with glutaric aciduria type I

Boy, Nikolas; Heringer, Jana; Haege, Gisela; Glahn, Esther M.; Hoffmann, Georg F.; Garbade, Sven F.; Kölker, Stefan; Burgard, Peter

doi:10.1186/s13023-015-0379-6

Orphanet Journal of Rare Diseases

Table 2 Parameter estimates of the exponential model with F-test for comparison of groups

From: A cross-sectional controlled developmental study of neuropsychological functions in patients with glutaric aciduria type I

Test	Variable	Model parameter		Parameter estimate (PE)	SE of PE	t-test for PE	p value of t-test	RSS	AICc		PE	SE of PE	t-test for PE	P value of t-test	RSS	AICc	F (df1, df2)	p	F (df1, df2)	p
			n	GA-I patients						n	Controls						Pat vs. Controls		Asymp. vs. Controls
SRT	Log Mean	b	30	2.38	0.07	33.95	<0.001	0.362	−38	196	2.38	0.01	309.48	<0.001	1.014	−467	3.40 (3,220)	0.019	−0.63 (3,207)	0.595
		c	30	0.58	0.11	5.33	<0.001			196	0.92	0.13	7.05	<0.001
		d	30	−0.46	0.24	−1.8	0.071			196	−0.92	0.11	−8.15	<0.001
CP	Log Mean	b	27	0.88	0.05	16.81	<0.001	0.508	−22	183	0.75	0.03	25.37	<0.001	1.231	−388	2.57 (3,204)	0.056	0.54 (3,193)	0.657
		c	27	1.01	0.44	2.32	0.030			183	0.62	0.04	16.41	<0.001
		d	27	−0.91	0.45	−2.00	0.057			183	−0.31	0.05	−5.91	<0.001
Track-ing	Log Mean	b	30	1.40	0.05	29.61	<0.001	0.444	−32	119	1.38	0.02	81.71	<0.001	1.458	−178	2.63 (3,143)	0.052	3.64 (3,130)	0.015
		c	30	0.62	0.30	2.07	0.049			119	0.87	0.15	5.88	<0.001
		d	30	−0.80	0.52	−1.53	0.137			119	−0.72	0.14	−5.26	<0.001
VS 3	Log Mean L3	b	21	3.01	0.07	46.22	<0.001	0.596	−6	172	2.88	0.03	107.14	<0.001	2.362	−241	2.93 (3,187)	0.035	2.32 (3,179)	0.077
		c	21	1.63	0.69	2.37	0.029			172	0.97	0.12	8.20	<0.001
		d	21	−0.90	0.39	−2.31	0.033			172	−0.46	0.08	−5.92	<0.001
VWM	Log Mean Contrast	b	27	0.69	0.17	4.10	<0.001	1.603	9	125	0.66	0.03	24.80	<0.001	5.923	−18	1.90 (3,146)	0.133	0.61 (3,135)	0.609
		c	27	0.73	0.25	2.89	0.008			125	1.62	0.64	2.53	0.013
		d	27	−0.43	0.42	−1.03	0.314			125	−1.13	0.35	−3.27	0.001
				Dystonic (Dys.) patients							Asymptomatic (Asymp.) patients						Dys. vs. Asymp.		Dys. vs. Controls
SRT	Log Mean	b	13	2.43	0.06	39.30	<0.001	0.131	−12	17	2.36	0.08	30.36	<0.001	0.218	−16	0.30 (3,24)	0.824	4.67 (3,203)	0.004
		c	13	0.90	0.38	2.39	<0.038			17	0.80	0.27	3.01	0.009
		d	13	−0.75	0.43	−1.75	0.112			17	−0.65	0.39	−1.65	0.122
CP	Log Mean	b	11	0.85	0.05	17.21	<0.001	0.076	−12	16	0.84	0.15	5.61	<0.001	0.382	−4	0.75 (3,21)	0.533	4.85 (3,188)	0.003
		c	11	1.39	0.50	2.76	0.025			16	0.59	0.22	2.68	0.019
		d	11	−0.95	0.37	−2.59	0.032			16	−0.46	0.50	−0.92	0.377
Track-ing	Log Mean	b	13	1.41	0.06	21.81	<0.001	0.158	−10	17	1.29	0.09	15.01	<0.001	0.231	−15	1.14 (3,24)	0.351	0.08 (3,126)	0.969
		c	13	1.00	0.47	2.15	0.057			17	0.70	0.24	2.92	0.011
		d	13	−0.80	0.47	−1.69	0.123			17	−0.60	0.42	−1.43	0.175
VS 3	Log Mean L3	b	8	2.50	10.02	0.25	0.813	0.192	7	13	3.00	0.09	31.97	<0.001	0.356	1	n.d.	n.d.	n.d.	n.d.
		c	8	0.78	9.59	0.08	0.939			13	1.72	0.82	2.10	0.062
		d	8	−0.06	0.97	−0.06	0.956			13	−0.90	0.47	−1.91	0.085
VWM	Mean Contrast	b	11	0.64	0.34	1.88	0.100	0.652	12	16	0.79	0.10	8.10	<0.001	0.845	8	0.49 (3,21)	0.690	2.09 (3,130)	0.105
		c	11	0.91	0.50	1.80	0.110			16	6.35	47.34	0.13	0.895
		d	11	−0.36	0.53	−0.69	0.513			16	−2.81	7.57	−0.37	0.717

F-test according to Mead and Cournow 1983. Data of all psychological test scores show good fits to the model (R² _adj ≥ 0.442), both for GA-I patients (n = 30; asymptomatic: n = 17; dystonic: n = 13) and controls (n = 196), with the exception of VS3 for dystonic patients. For definition of model parameters b,c and d see Fig. 1. R² _adj: adjusted explanatory power of the regression model. SE: standard error

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