Table 2

Abnormal ¹H-MRSI and clinical rating scale scores (CRSS) findings in patients with Niemann-Pick disease type C (NP-C) and controls

NPC	CRSS	FC	TC	OC	PC	CG	CSO	TH	CD
NA/Cre
1	1	2.22↓	NS	NS	—	NS	1.72↓	—	NS
2	0	NS	—	NS	NS	—	NS	NS	NS
3	3	1.64↓	—	NS	—	—	NS	NS	1.65↓
4	3	1.73↓	NS	NS	NS	—	2.22↓	—	—
5	3	1.63↓	1.83↓	—	NS	1.50↓	1.54↓	2.03↓	1.28↓
9	3	1.58↓	1.85↓	—	—	NS	1.96↓	—	—
7	2	NS	NS	NS	NS	1.63↓	2.10↓	—	—
8	3	1.60↓	—	NS	—	—	NS	NS	NS
9	2	2.18↓	—	—	NS	—	NS	NS	—
10	4	1.31↓	—	—	—	—	1.08↓	—	—
Controls	Mean (SD)	2.83 (0.30)	2.65 (0.17)	2.59 (0.43)	2.52 (0.25)	2.39 (0.21)	2.81 (0.26)	2.54 (0.22)	2.53 (0.23)
	Range	(2.55–3.70)	(2.20–4.40)	(1.50–3.10)	(1.65–2.84)	(2.11–3.79)	(2.58–4.12)	(1.98–3.31)	(1.68–2.87)
Cho/Cre
1	1	NS	NS	NS	—	NS	1.87↑	—	NS
2	0	NS	—	NS	NS	—	NS	NS	NS
3	3	1.80↑	—	NS	—	—	2.00↑	NS	NS
4	3	1.79↑	NS	NS	NS	NS	NS	—	—
5	3	1.73↑	1.60↑	—	NS	NS	1.83↑	1.75↑	NS
6	3	1.64↑	NS	—	—	NS	1.99↑	—	—
7	2	NS	NS	NS	NS	NS	NS	—	—
8	3	1.82↑	—	NS	—	—	2.09↑	NS	—
9	2	1.64↑	—	—	NS	—	2.28↑	NS	NS
10	4	2.15↑	—	—	—	—	2.49↑	—	—
Controls	Mean (SD)	1.08 (0.22)	1.11 (0.18)	.95 (0.12)	1.20 (0.24)	1.09 (0.17)	1.24 (0.20)	1.24 (0.20)	1.32 (0.30)
	Range	(0.86–1.50)	(0.81–1.57)	(0.61–1.29)	(1.01–1.72)	(0.82–1.81)	(1.05–1.69)	(1.06–1.67)	(1.12–1.75)

For definition of abnormal ¹H-MRSI values and abbreviations see methods.
—=Not detected in ¹H-MRSI data.
FC=frontal cortex; TC=temporal cortex; OC=occipital cortex; PC=parietal cortex; CG=cingulate gyrus; CSO=centrum semiovale; TH=thalamus; CD=caudate.