Childhood convulsion and hemiparesis due to schizencephaly-a case report

Childhood convulsion with hemiparesis due to schizencephaly is a rare condition. It is a developmental disorder of neuronal migration, characterized by early focal destruction of the germinal matrix and surrounding brain before the cerebral hemispheres are fully formed at 1-5 months of gestation .The lesion is most likely related to multiple aetiologies including genetic, toxic, metabolic, vascular or infectious agents. This case is reported due to its worldwide rarity. INTRODUCTION Schizencephaly, a developmental disorder of neuronal migration is characterized by cleft in cerebral mantle, which communicates between the subarachnoid space laterally and ventricular system medially. This disorder was originally described by Wadsworth and Yakolev. 1 Their original work describes schizencephaly to result from failure of normal migration of primitive normoblasts resulting in cerebral cleft .The cardinal neuropathological features are і) Hemispheric cleft іі) communication of subarachnoid space with lateral ventricle medially ііі) Infolding of grey matter along the cleft iv) Multiple associated intracranial malformations including polymicrigyria, absent septum pellucidum, optic nerve hypoplasia. The presentation and outcome are variable i.e. hemiparesis, developmental delay, microcephaly, mental retardation and most patients have seizures. CASE REPORT A 2 year aged female child was admitted in paediatric neurosurgery ward of CM Hospital Dhaka with complain of repeated episode of convulsion since birth and weakness in right upper and lower limbs since two months of age. She was symptomatically treated with anticonvulsant sodium valproate. The child was delivered full term normally at home with unremarkable antenatal maternal history. She cried soon after birth and there were no postnatal complications. There is no history of consanguineous marriage. On physical examination child had normocephaly, no dysmorphism of face. Anterior fontanelle was normal. Sutures were widely normal. There was a global delay in the developmental milestones. Muscle tone of the right upper and lower limbs showed spasticity with 4/5 power grade with normal reflexes. Vitals were normal. Anthropometric measurements of weight and length were within normal limits. The child was admitted with provisional diagnosis of Cerebral palsy. Investigations done revealed normal Hb, Tc, Dc. EEG showed abnormal record with seizure pattern. CT scan showedSCHIZENCEPHALY. Figure 1 Fig: CT scan of brain to show schizenencephaly Thus the diagnosis was established with the help of CT scan report. The option of treatment with prognosis was explained to the parents. She eventually underwent cystoperitonreal shunt. Postoperative recovery was uneventful except an episode of seizure that was managed by sodium valproate. Childhood convulsion and hemiparesis due to schizencephaly-a case report 2 of 3 Counseling was given and physiotherapy was advised. Anticonvulsant was continued. DISCUSSION Schizencephaly is a rare developmental disorder of neuronal migration. It is characterized by CSF filled cleft, lined by grey matter and extends across the entire cerebral hemisphere from ependyma to the periphery of the brain 12. Types of Schizencephaly: Type I: Closed type (lips of cleft are fused) Type II: Open type (clefts are separated) Type II is more common than type I