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Table 2 Classification of patients (n = 182) into NPC clinical forms

From: Clinical disease characteristics of patients with Niemann-Pick Disease Type C: findings from the International Niemann-Pick Disease Registry (INPDR)

Clinical form

N (%)

Mean age at diagnosis (SD) and range in years

Neonatal systemic rapidly fatal (< 28 days)

10 (5.6)

0.37 (0.25)—0.0–0.9

Early infantile neurological (< 2 years)

43 (24.2)

2.16 (2.75)—0.0–17

Late infantile neurological (2 to  < 6 years)

47 (26.4)

4.92 (3.25)—0.1–12

Juvenile neurological (6 to < 15 years)

41 (23.0)

9.89 (5.34)—0.1–22

Adult neurological (> 15 years)

37 (20.8)

34.12 (13.84)—1.4–68

No neurological manifestations

4

19 (17.95)—0.7–42