Polymicrogyria in glycogenosis type III: An incidental finding?

The purpose of the present report is to document a 20-year-old woman with glycogenosis type III who presented a malformation of cortical development, in this case a polymicrogyria over bilateral perisylvian regions. Association of a malformation of cortical development in this type of glycogenosis h...

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Bibliographic Details
Published in:Pediatric neurology 2004-08, Vol.31 (2), p.143-145
Main Authors: Vincentiis, Silvia, Valente, Kette D., Valente, Marcelo
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Cerebral Cortex - abnormalities
Cerebral Cortex - pathology
Female
Glycogen Storage Disease Type III - pathology
Humans
Magnetic Resonance Imaging - methods
Medical sciences
Nervous System Malformations - pathology
Neurology
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Summary:The purpose of the present report is to document a 20-year-old woman with glycogenosis type III who presented a malformation of cortical development, in this case a polymicrogyria over bilateral perisylvian regions. Association of a malformation of cortical development in this type of glycogenosis has not been previously reported. The existence of previous cases of glycogenosis associated with malformations of cortical development led us to believe that glycogen storage during pregnancy may act as a harmful prenatal event. On the other hand, the presence of a lesion associated with severe neurologic deficits in one patient with a milder form of glycogenosis is in disagreement with the idea that there is a strong correlation between the severity of the central nervous system lesion and that of the disease.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2004.02.012