Abstracts

Rationale: Continuous Spike Wave in Sleep Syndrome (CSWS) is a rare but potentially treatable epilepsy syndrome of childhood. It is defined by developmental regression and electrical status epilepticus in slow wave sleep (ESES). The pathophysiology of CSWS is still unknown. The objective is to present 3 cases of CSWS preceded by neonatal hydrocephalus.Methods: We retrospectively identified 16 patients seen between 2007 and 2009 with ESES. Of these, 3 patients with CSWS had a history of neonatal hydrocephalus with ventriculo-peritoneal (VP) shunt placement. We then reviewed the charts of these patients to determine the clinical features, treatment and responsiveness of the CSWS. Results: Case 1 (PD) presented at age 3 days with grade 3 intraventricular hemorrhage (IVH) following an uneventful pregnancy and delivery, requiring placement of a VP shunt. She was developmentally delayed and began to have complex partial seizures at age 4 years. Her development regressed shortly afterward. She was treated for CSWS with many different anti-epileptic medications including diazepam, prednisone, and IVIG, but did not achieve remission. Case 2 (AB) had a large neonatal bilateral intraventricular hemorrhage extending into the basal ganglia and thalamus following a difficult term delivery, requiring VP shunt placement. She was developmentally delayed and had a right hemiparesis. She developed complex partial seizures at age 21 months and was hospitalized several times for status epilepticus. She began to regress developmentally at age 4.5 years. She was treated with diazepam, valproate, prednisone, zonisamide, ethosuxamide, IVIG, sulthiame and deep brain stimulation, but did not achieve remission of CSWS. Case 3 (CS) underwent placement of VP shunt for hydrocephalus at age 4 days following a difficult term delivery. He was developmentally delayed and developed generalized and complex partial seizures at age 4 years. Developmental regression began at age 6. He was diagnosed at age 8 with cerebral folate deficiency and was treated with leucovorin. His CSWS was treated successfully with diazepam and has been maintained in remission on zonisamide. Conclusions: Given the high prevalence of a history of neonatal hydrocephalus in this cohort of children with CSWS (3/16), neonatal hydrocephalus may be a risk factor for ESES and CSWS. Children with seizures and developmental regression should be screened for ESES, as regression is potentially reversible with treatment. However, ESES associated with neonatal hemorrhage is particularly refractory to treatment.