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In the present study there was no significantly higher number of anomalies per patient in females than males. The most frequent CNS types of anomalies were seizures, motor impairment, mental retardation, and microcephaly. The most frequently registered CNS lesions found using brain imaging methods were brain infarcts or necrosis, brain atrophies, and corpus callosum lesions. <jats:italic>IKBKG<\/jats:italic> exon 4\u201310 deletion was present in 86.00% of genetically confirmed IP patients. The frequency of CNS anomalies, similar to the frequency of retinal anomalies in IP patients, concurrent with their severity, supports their recognition in the list of IP minor criteria.<\/jats:p>","DOI":"10.1186\/1750-1172-8-25","type":"journal-article","created":{"date-parts":[[2013,2,13]],"date-time":"2013-02-13T19:15:54Z","timestamp":1360782954000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":92,"title":["Systematic review of central nervous system anomalies in incontinentia pigmenti"],"prefix":"10.1186","volume":"8","author":[{"given":"Sne\u017eana","family":"Mini\u0107","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Du\u0161an","family":"Trpinac","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miljana","family":"Obradovi\u0107","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,2,13]]},"reference":[{"key":"515_CR1","unstructured":"Orphanet Report Series - Prevalence of rare diseases: Bibliographic data -. 2012, [http:\/\/www.orpha.net\/orphacom\/cahiers\/docs\/GB\/Prevalence_of_rare_diseases_by_alphabetical_list.pdf], May \u2013 Number 1,"},{"key":"515_CR2","unstructured":"Scheuerle A, Ursini MV, et al: Incontinentia pigmenti (Bloch-Sulzberger syndrome). 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