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Table 2 Distribution of clinical forms of NP-C disease in large cohorts

From: Consensus clinical management guidelines for Niemann-Pick disease type C

  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
 Spain [71,72,73] 7 37 21 25 11 57
 Italy [74, 75] 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
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