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Table 1 The main findings of IP patients and CNS anomalies by sex for the period 1993-2012

From: Systematic review of central nervous system anomalies in incontinentia pigmenti

 

Number of

Distribution of CNS anomaly types in numbers **

Number of

Sex

IP patients

Neurologically investigated IP patients

Neurologically investigated IP patients with CNS anomalies

CNS anomalies

CNS anomaly types per patient

Seizures

Mental retardation

Motor impairment

Microcephaly

CNS ** anomalies found in small number or unspecified

Neurologically investigated IP patients with mild CNS anomalies

Neurologically investigated IP patients with severe CNS anomalies

Total*

1,393

795

242

393

1.62

165

80

101

16

31

92

150

Female

1,295

719

221

361

1.63

152

73

94

15

27

84

137

Male

92

76

21

32

1.52

13

7

7

1

4

8

13

  1. * For six patients, data concerning sex were not available in the literature.
  2. ** The task of counting and identifying anomalies in some references was challenging because there were only lists of observed anomalies with no exact numbers. These lists included frequent types of CNS anomalies such as seizures, motor impairment, mental retardation, and microcephaly. These anomalies were classified as unspecified anomalies together with different anomalies presented in small numbers. In this investigation, of 31 registered rare or unspecified CNS anomalies, 15 were unspecified but frequent types of CNS anomalies (seizures, motor impairment, mental retardation, and microcephaly). Because of these difficulties, the exact number of frequent types of CNS anomalies such as seizures, motor impairment, mental retardation, and microcephaly was actually higher than those presented in the tables.