title: [Schizencephaly and upper limb malformation].
abstract: BACKGROUND
  Schizencephaly, a failure of the cerebral mantle to form is usually unilateral and not associated with anomalies of the controlateral limbs. We present two cases associated with such anomalies.
  
  
  METHODS
  Case no 1. A boy born with a lobster claw deformity in the right arm. His IQ was 60 and he developed generalized seizures at the age of 4 years with spastic diplegia. MRI examination showed unilateral left schizencephaly also lined with pachygyric cortex. Case no 2. A girl was born with a right ectromelic-hand and developed left spastic hemiparesy. Her IQ was normal. She had a generalized seizure at the age of 11 years. MRI showed unilateral right schizencephaly also lined with pachygyric cortex and heterotopic periventricular gray matter.
  
  
  CONCLUSIONS
  Schizencephaly seems to be due to localized ischemia in the periventricular germinal matrix during the 7th week of gestation. The limb deformity could be explained by the same vascular mechanism in the first case but not in the second one since it was homolateral to brain damage.
  
  
  CONCLUSIONS
  Description of further cases is necessary to understand this association. We suggest MRI examination of brain in patients with limb deformity associated with neurological damage.
authors: Service de neurologie pédiatrique, clinique Saint-Eloi, Montpellier, France.
authors: V
authors: V
authors: Humbertclaude
authors: J M
authors: JM
authors: Pedespan
authors: M
authors: M
authors: Azaïs
authors: D
authors: D
authors: Fontan
authors: B
authors: B
authors: Echenne
mesh_terms: D000015:Abnormalities, Multiple; D001132:Arm / Q000002:abnormalities*; D001921:Brain / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D004480:Ectromelia / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male
vernacular_title: Schizencéphalie et malformation du membre supérieur.