title: Hemihydranencephaly syndrome: case report and review.
abstract: BACKGROUND
  Hemihydranencephaly (HHDNC) is a rare disorder with complete or almost complete unilateral absence of cerebral cortex.
  
  
  METHODS
  This study describes a 27 months-old girl presenting with developmental delay and generalized weakness more on the left side. Bilateral blindness was noted since the age of 6 months.
  
  
  RESULTS
  Her fundus examination revealed bilateral optic atrophy, dilated tortuous retinal veins with increased intra-ocular tension. She had polyuria and recurrent attacks of dehydration due to neurogenic diabetes insipidus. Her blood protein S was deficient. Her magnetic resonance imaging (MRI) demonstrated HHDNC with nearly complete absence of the right cerebral hemisphere. Her MR-Arteriography demonstrated total occlusion of right middle and anterior cerebral arteries and attenuated and beaded right posterior cerebral artery. Diffusion tensor MR imaging revealed complete absence of right cortico-spinal and optic tracts with deficient left sided tracts.
  
  
  CONCLUSIONS
  In contrast to the good outcome of the few reported cases of HHDNC, this case had severe global disabilities.
authors.affiliation: Department of Pediatrics, Ain Shams University, Cairo, Egypt. saharhassanein@yahoo.com
authors.forename: Sahar M A
authors.initials: SM
authors.lastname: Hassanein
authors.forename: Yasser A
authors.initials: YA
authors.lastname: Abbas
authors.forename: Ahmed M
authors.initials: AM
authors.lastname: Monib
authors.forename: Mohsen S
authors.initials: MS
authors.lastname: El Alfy
mesh_terms: D001921:Brain / Q000473:pathology* / Q000503:physiopathology; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D056324:Diffusion Tensor Imaging; D005260:Female; D006801:Humans; D006832:Hydranencephaly / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D009896:Optic Atrophy / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology