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Bilateral perysilvian polymicrogyria in Chiari I malformation
Authors:Alberto Spalice  Pasquale Parisi  Mario Mastrangelo  Francesca De Luca  Alberto Verrotti  Paola Iannetti
Affiliation:(1) Child Neurology Division, Department of Pediatrics, University “La Sapienza”, Viale Regina Elena, 324, 00161 Rome, Italy;(2) Child Neurology Section, Department of Pediatrics, University “La Sapienza 2”, Roma, Italy;(3) Section of Pediatrics, Department of Medicine, University of Chieti, Chieti, Italy
Abstract:Case report We report on a 7-year-old girl with generalized epilepsy and mental retardation. Discussion Neurologic examination was normal and only facial dysmorphic features, compatible with frontonasal dysplasia, were observed. However, magnetic resonance imaging (MRI) revealed Chiari I malformation together with bilateral opercular polymicrogyria. To our knowledge, this is the first report of the association of these developmental disorders. This condition raises questions regarding the ethiopathogenetic classification of Chiari spectrum and as to whether embryologic and genetic causes could be potentially interconnected.
Keywords:Polymicrogyria  Chiari I  Magnetic resonance imaging
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