Size and Shape of the Corpus Callosum in Adult Niemann-Pick Type C Reflects State and Trait Illness Variables

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Fig 1.
Extraction of the corpus callosum from midsagittal image. Top shows midsagittal callosal image, middle shows binarized callosal image, and bottom shows midspline traversing callosum between 2 end points and callosal thicknesses orthogonal to equidistant points along the midspline.
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Fig 2.
Callosal metrics. Top left, total callosal area; top right, length of callosal midspline; bottom left, 39 individual thickness measures perpendicular to midcallosal spline; bottom right, callosal bending angle.
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Fig 3.
Boxplots (with quartiles) showing between-group comparisons of main callosal metrics: total area (top left), mean width (top right), total length (bottom left), and bending angle (bottom right).
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Fig 4.
Mean callosal thickness in patients with NPC and controls. Genu thickness (left) and splenium thickness (right).
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Fig 5.
Significance map showing regions of change across the callosum. Genu (left) and splenium (right).
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Fig 6.
Correlations between callosal area with duration of symptoms (left) and symptom score (right).

Table 1:

Clinical, biochemical, and ocular-motor variables for the NPC patient group

Demographic Variables			Illness Variables			Biochemical Variables		Ocular-Motor Variables
Patient	Age (yr)	Gender	Age at Onset (yr)	Duration (yr)	Illness Scale	Filipin (%)	Cholesterol Esterification (pmol/hr/mg)	V_max (°/s)	Reflexive Gain	α (ms/°)	β (ms)	Antisaccade Errors (%)	Self-Paced Saccades (30 s)
1	49	M	47	2	5	20	NA	538	0.985	1.053	15.64	66.67	59
2	23	M	17	6	13	75	2.2	NA	NA	NA	NA	NA	NA
3	33	F	29	4	9	25	1.5	456	0.785	1.67	14.47	53	43
4	32	F	26	6	7	60	5.3	411	0.797	1.383	21.03	75	65
5	43	F	41	2	6	15	2.3	355	0.895	2.233	15.18	60.5	72
6	20	M	19	1	5	90	2	522	0.962	1.639	15.46	53	64
7	32	M	25	7	11	70	2.9	321	0.735	1.596	29.37	92	36
8	31	M	19	12	15	95	0.3	125	0.517	8.817	2.77	100	26
9	18	M	14	4	7	70	1.4	495	0.909	1.538	16.12	93	97

Table 2:

Demographic and callosal variables across control and NPC patient groups

	NPC	Controls	Variable	P
Age (yr, mean ± SD)	31.22 ± 10.17	30.24 ± 9.78	t = 0.252	.805
Gender (M/F)	6/3	17/9	χ² = 0.040	.841
Total callosal area (mm²)	572.19 ± 67.33	702.74 ± 90.22	t = −3.96	<.0001
Bending angle (radians)	1.66 ± 0.13	1.69 ± 0.10	t = −0.75	.472
Total callosal length (mm)	97.80 ± 7.72	98.18 ± 5.29	t = −0.16	.894
Mean callosal width (mm)	6.19 ± 1.01	7.49 ± 0.79	t = −3.96	<.0001