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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.