Abstract：
OBJECTIVE:We retrospectively reviewed six patients with incontinentia pigmenti (IP) who had encephalopathic manifestations during early infancy. METHODS:We enrolled six patients who met the following criteria from the mailing list of the Annual Zao Conference: (1) diagnosis of IP; (2) encephalopathic manifestations with reduced consciousness and clusters of seizures by 6 months of age; and (3) no evidence of central nervous system infection or metabolic derangement. RESULTS:The onset of the encephalopathic events was within the first 2 months of life in all but one patient. All had clusters of focal clonic seizures. The duration of seizures was typically 5 min. The seizures ceased within 5 days in all patients. Various degrees of reduced consciousness were observed in association with the frequent seizures. Diffusion-weighted imaging during the acute phase showed reduced water diffusion in the subcortical white matter, corpus callosum, basal ganglia, thalami, and internal capsule in two patients. Scattered subcortical white matter lesions were observed on fluid-attenuated inversion-recovery images in two patients. CONCLUSIONS:The encephalopathic manifestations in patients with incontinentia pigmenti were characterized by seizure clusters and reduced consciousness, albeit of relatively short duration. Magnetic resonance imaging abnormalities were predominant in the subcortical areas in most patients.
journal_name
Brain Devjournal_title
Brain & developmentauthors
Abe S,Okumura A,Hamano S,Tanaka M,Shiihara T,Aizaki K,Tsuru T,Toribe Y,Arai H,Shimizu Tdoi
10.1016/j.braindev.2010.04.002subject
Has Abstractpub_date
2011-01-01 00:00:00pages
28-34issue
1eissn
0387-7604issn
1872-7131pii
S0387-7604(10)00088-4journal_volume
33pub_type
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journal_title：Brain & development
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journal_title：Brain & development
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pub_type： 杂志文章
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journal_title：Brain & development
pub_type： 杂志文章
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更新日期：1991-11-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章
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journal_title：Brain & development
pub_type： 杂志文章
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更新日期：2011-08-01 00:00:00
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journal_title：Brain & development
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更新日期：2017-06-01 00:00:00
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pub_type： 杂志文章
doi：10.1016/0387-7604(94)90076-0
更新日期：1994-05-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(12)80054-4
更新日期：1991-07-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2011.12.009
更新日期：2012-10-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章,评审
doi：10.1016/j.braindev.2012.11.010
更新日期：2013-10-01 00:00:00
