Hindawi Publishing Corporation Case Reports in Pediatrics Volume 2012, Article ID 374232, 3 pages doi:10.1155/2012/374232 Case Report Nonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children Pratibha Singhi, Arushi Gahlot Saini, and Jitendra Kumar Sahu Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India Correspondence should be addressed to Pratibha Singhi, doctorpratibhasinghi@gmail.com Received 24 June 2012; Accepted 16 September 2012 Academic Editors: N.-C. Chiu, K. Kowal, W. B. Moskowitz, and E. Veneselli Copyright © 2012 Pratibha Singhi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis. 1. Introduction Subacute sclerosing panencephalitis (SSPE) is a progres- sive neurodegenerative disease caused by aberrant measles virus infection. Patients generally present with behavioral and cognitive changes, periodic myoclonus, and electroen- cephalogram typically shows periodic complexes [1, 2]. The diagnosis is confirmed by elevated measles antibodies in cerebrospinal fluid and serum. We report here two cases of SSPE with rare and atypical presentation of nonconvulsive status epilepticus (NCSE). 2. Case 1 A previously well, ten-year-old boy was admitted with pro- gressive cognitive decline, myoclonic jerks, gait unsteadiness, and frequent falls for the past four months. There was no history of measles, fever, or trauma. He had received measles vaccination at nine months of age. On examination, he appeared confused with intermittent lapses in consciousness and periodic myoclonic jerks every 5 to 6 seconds in form of sudden flexion of the neck, trunk, and arms. He had difficulty in sitting, swallowing, drooling of saliva, slurred speech, and poor interaction with the examiner. He had generalized spasticity, hyperreflexia, and bilateral extensor plantar response. Cranial nerves, fundus, and the rest of the physical examination were normal. A clinical diagnosis of SSPE was considered in view of periodic myoclonus and progressive cognitive decline. Scalp electroencephalogram showed disorganized back- ground with generalized spike-wave discharges 2–2.5/second occupying more than 80% of tracing, consistent with NCSE (Figure 1). Following intravenous diazepam (0.3 mg/kg) administration, transient normalization of background rhythm to 6–8/second along with intermittent, generalized high-voltage slow-wave discharges was seen, but typical periodic complexes were not seen. There was no change in the mental status of the child following diazepam. Magnetic resonance imaging of brain (fluid attenuated inversion recovery sequence) showed periventricular hyperintensities involving bilateral parieto-occipital white matter (Figure 2). Measles antibody titers by enzyme-immunoassay were raised in both cerebrospinal fluid and serum: 5.24 IU/mL (positive >1.1) and 5.11 IU/mL (positive >1.1), respectively. A diag- nosis of SSPE was made with clinical stage IIIA according to Jabbour’s criteria [1]. Child was started on valproic acid