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Table 3 Aetiological classification and incidence rates in children with progressive encephalopathy

From: Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study

 

n

%

95% CI

IR per 100,000 person yearsa

95% CI

I. Metabolic

55

65.5

54.3–75.5

4.21

3.23–5.49

A. Subcellular organelles

28

33.3

23.4–44.5

2.14

1.48–3.11

   Lysosomal

23

27.4

18.2–38.2

1.76

1.17–2.65

   Mitochondrial

3

3.6

0.7–10.1

0.23

0.07–0.71

   Peroxisomal

2

2.4

0.3–8.3

0.15

0.04–0.61

B. Intermediate metabolism

27

32.1

22.4–43.2

2.07

1.42–3.02

   Organic aciduria

11

13.1

6.7–22.2

0.84

0.47–1.52

   Fatty acid oxidation defect

6

7.1

2.7–14.9

0.46

0.21–1.02

   Urea cycle disorders

4

4.8

1.3–11.7

0.31

0.12–0.82

   Galactosemia

4

4.8

1.3–11.7

0.31

0.12–0.82

   Unspecified

2

2.4

0.3–8.3

0.15

0.04–0.61

II. Neurodegenerative

27

32.1

22.4–43.2

2.10

1.42–3.02

A. Specified

10

11.9

5.9–20.8

0.77

0.41–1.42

B. Unspecified

17

20.2

12.3–30.4

1.30

0.81–2.09

III. Infectious

2

2.4

0.3–8.3

0.15

0.04–0.61

Total

84

100

-

6.43

5.19–7.97

  1. CI: confidence interval; IR: incidence rates
  2. aNot adjusted for time periods