Document Detail


Unusual ribbon-like periventricular heterotopia with congenital cataracts in a Japanese girl.
MedLine Citation:
PMID:  22315185     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Periventricular heterotopia (PH), clumps of neurons mislocated beside the ventricle, is caused by failure to initiate migration during embryogenesis. We report on a 32-month-old Japanese girl with a unique subtype of PH, namely ribbon-like PH. The patient presented with severe psychomotor developmental delay, intractable epilepsy, and congenital cataracts and developed West syndrome phenotype. Brain magnetic resonance imaging revealed a unique undulating form of PH, categorized as ribbon-like PH, and other brain malformations including simplified gyri and dysgenesis of the corpus callosum. There was no evidence of prenatal TORCH infection or associated syndrome. Array-based comparative genomic hybridization revealed no chromosomal rearrangements. Genetic analyses of the FLNA, DCX, ARX, LIS1, and TUBA1A genes showed no mutations. Although little is known about ribbon-like PH, the clinical manifestations in our patient clearly differed from those in other reported patients. © 2012 Wiley Periodicals, Inc.
Authors:
Rie Tsuburaya; Mitsugu Uematsu; Atsuo Kikuchi; Naomi Hino-Fukuyo; Shinji Kunishima; Mitsuhiro Kato; Kazuhiro Haginoya; Shigeru Tsuchiya
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-2-7
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  -     ISSN:  1552-4833     ISO Abbreviation:  -     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-2-8     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Wiley Periodicals, Inc.
Affiliation:
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
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