Document Detail


Clinical patterns of neuronal migrational disorders and parental consanguinity.
MedLine Citation:
PMID:  9972079     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The role of inheritance in neuronal migrational disorders is under intense investigation. Studies on neuronal migrational disorders (NMDs) from developing countries that have a high rate of parental consanguinity are lacking. The present study included 29 children (aged 15 days-12 years, mean age 1.4 years) who were diagnosed to have NMDs, from a non-selected population with seizures and non-selected population of cognitive developmental delay, in the period January 1994 to April 1997. Seventeen (58.6 per cent) patients had lissencephaly, four (13.8 per cent) patients had pachygyria, three (10.3 per cent) patients had neuronal heterotopia, four (13.8 per cent) patients had schizencephaly, one patient (3.4 per cent) had hemimegalencephaly, and 14 (48.2 per cent) patients with NMDs had other associated conditions. Lissencephalic patients had a high rate of parental consanguinity (88.2 per cent) and family history of possible similar cases (76.4 per cent). In conclusion, lissencephaly is probably the commonest neuronal migrational disorder in communities with a high rate of parental consanguinity, adding significant support to the literature on the genetic aetiology of lissencephaly.
Authors:
A A al-Qudah
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Publication Detail:
Type:  Clinical Trial; Journal Article    
Journal Detail:
Title:  Journal of tropical pediatrics     Volume:  44     ISSN:  0142-6338     ISO Abbreviation:  J. Trop. Pediatr.     Publication Date:  1998 Dec 
Date Detail:
Created Date:  1999-03-02     Completed Date:  1999-03-02     Revised Date:  2006-03-28    
Medline Journal Info:
Nlm Unique ID:  8010948     Medline TA:  J Trop Pediatr     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  351-4     Citation Subset:  IM    
Affiliation:
Pediatric Department, Jordan University Hospital, Amman, Jordan.
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MeSH Terms
Descriptor/Qualifier:
Cell Movement*
Cerebral Cortex / abnormalities*,  pathology,  radiography
Child
Child, Preschool
Consanguinity*
Developmental Disabilities / diagnosis*,  epidemiology,  genetics*
Electroencephalography
Female
Humans
Incidence
Infant
Infant, Newborn
Jordan / epidemiology
Magnetic Resonance Imaging
Male
Neurons / pathology*
Risk Factors
Seizures / classification,  etiology*
Tomography, X-Ray Computed

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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