Table 3.

Motor milestones and neurological symptoms of NP-C in the untreated vs treated groups (in chronological order)

	No-miglustat group n = 16	Miglustat treated group n = 10	p value
Autonomous sitting position
Age at acquisition	0.75 (0.5–2) uk = 4	0.83 (0.7–1.4) na = 1, uk = 2	x
Age at loss	2.83 (2.25–5.9) uk = 6	3.1 (1.9–3.8) uk = 1	0.8
Standing position with help or walk with help
Age at acquisition	1.25 (0.75–2) uk = 5	1.25 (0.8–2.25) na = 3	x
Age at loss	2.5 (1.5–5.5) uk = 9	2.91 (2.4–3.4) uk = 1	x
Autonomous walk	1.7 (0.8–2) na = 10, uk = 2	na = 9, uk = 1	x
Delayed language development	1.63 (0.75–2.3) uk = 10	1.56 (0.75–2.4) uk = 2	x
Cerebellar signs	1.88 (1.25–3.5) uk = 7	2.21 (1.2–3.9) uk = 2	0.60
VSGP or oculomotor apraxia	2.33 (1.6–5.25) uk = 11	2.58 (0.7–4.6) uk = 1	x
Limb hypertonia	2.54 (2.1–4.4) uk = 6	2 .13 (0.7–3.9) uk = 2	0.20
Seizures	2.75 (2.4–4.5) uk = 8	3.37 (2.75–5.6) uk = 6	x
Neuropathic pain	2.83 (2.75–4) uk = 12	3.08 (0.7–4.8) uk = 5	x
Swallowing trouble	3.04 (0.7–4.6) uk = 8	2.37 (0.25–2.8) uk = 4	x
Gelastic cataplexy	3.5 (2.2–4.5) uk = 13	4.08 (2.5–4.6) uk = 6	x

The ages are reported as median (range) and expressed in years. Ataxia, dysmetria and/or impaired global or fine coordination were considered as cerebellar signs. Limb hypertonia reported here was spastic or dystonic. Abbreviations: x: no statistical test due to inadequate sample size; uk: data unknown (= n); na: never acquired (= n); VSGP = vertical supranuclear gaze palsy