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Table 3 IP clinical data

From: Incontinentia pigmenti: report on data from 2000 to 2013

Type of defect

Number of cases**

Percentage of cases

CNS defects

97

 

Seizures

39

40.2

Mental retardation

29

29.9

Spastic paresis

16

16.5

Cerebral atrophy

13

13.4

Microcephaly

11

11.3

Hydrocephaly

5

5.1

Ischemic strokes*

5

5.1

White matter alterations*

4

4.1

Arachnoid cysts*

3

3.1

Cortico-subcortical atrophy*

3

3.1

Brain morphological alterations*

2

2.1

Teeth defects

134

 

Delayed primary dentition

46

34.3

Cone/peg shaped teeth

30

22.3

Delayed permanent dentition

30

22.3

Teeth dystrophy

23

17.2

Impactions

23

17.2

Ophthalmologic defects

94

 

Vision defects

16

17

Retinopathy

15

15.9

Retinal detachment

8

8.5

Microphthalmia

6

6.4

Retinal neuropathy

6

6.4

Retinal vascular visorders

5

5.3

Hair defects

82

 

Alopecia

8

9.7

Hypertrichosis

3

3.6

Fingernail defects

45

 

Nail dystrophy

29

64

Developmental evolution

30

 

Recurrent infections

36

11.7

Syndactyly of fingers or toes

4

1.3

  1. *Data obtained by Magnetic Resonance Imaging analysis in 17 IP cases.
  2. **IP patients can have more than one defect affecting different organ systems.