Table 2 Distribution of clinical forms of NP-C disease in large cohorts
From: Consensus clinical management guidelines for Niemann-Pick disease type C
| Â | Neonatal systemic fatal (%) | Early infantile neurological onset (%) | Late infantile neurological onset (%) | Juvenile neurological onset (%) | Adolescent/adult neurological onset (%) | Total no. |
|---|---|---|---|---|---|---|
Early studies | ||||||
 France+ European countries [70] | 12 | 30 | 23 | 30 | 5 | 125 |
7 | 37 | 21 | 25 | 11 | 57 | |
7 | 26 | 32 | 23 | 12 | 43 | |
 France [11] | 9 | 26 | 22 | 26 | 16 | 107 |
Recent studies | ||||||
 Germany [22] | 3 | 3 | 35 | 54 | 5 | 37 |
 Czech Republic [12] | 6 | 17 | 24 | 37 | 17 | 54 |
 UK [13] | 5 | 6 | 39 | 32 | 19 | 132 |
 European countries [20] | – | 11 | 31 | 31 | 27 | 145 |