Systematic review of central nervous system anomalies in incontinentia pigmenti

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

	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

* For six patients, data concerning sex were not available in the literature.
** 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.

ISSN: 1750-1172