Document Detail


Parieto-occipital lobe epilepsy caused by a POLG1 compound heterozygous A467T/W748S genotype.
MedLine Citation:
PMID:  21515089     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
We describe a 16-year-old woman with a rare POLG1 A467T/W748S genotype, with a wide range of neurological manifestations, including focal parieto-occipital lobe seizures, migraine headaches, cerebellar ataxia, sensory-motor axonal neuropathy, and impairment of visual perception and cognitive function. Treatment of epilepsy in patients with a POLG1 compound heterozygous A467T/W748S genotype is very challenging; the epilepsy may preferentially respond to sodium channel blockers. The POLG1-related syndrome has a variable clinical course, and disease morbidity and mortality may be correlated with the genotype.
Authors:
David Roshal; David Glosser; Andro Zangaladze
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-22
Journal Detail:
Title:  Epilepsy & behavior : E&B     Volume:  -     ISSN:  1525-5069     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-4-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100892858     Medline TA:  Epilepsy Behav     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Elsevier Inc. All rights reserved.
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