Subacute Sclerosing Panencephalitis: Results of the Canadian Paediatric Surveillance Program and review of the literature

BACKGROUND: Subacute Sclerosing Panencephalitis (SSPE) is so rare in developed countries with measles immunization programs that national active surveillance is now needed to capture sufficient number of cases for meaningful analysis of data. Through the Canadian Paediatric Surveillance Program (CPSP), the SSPE study was able to document a national incidence and determine the epidemiology of affected Canadian children. METHODS: Between 1997 and 2000, the CPSP surveyed monthly 1978 to 2294 Canadian pediatricians and sub-specialists for SSPE cases. The response rate varied from 82–86% over those years. RESULTS: Altogether, four SSPE cases were reported to the CPSP: one case before, two during and one after the study period. The incidence of SSPE in Canadian children was 0.06/million children/year. Of the four cases, diagnosed between ages four and 17 years, three children had measles infection in infancy. All children showed a progressive course of dementia, loss of motor skills and epilepsy. Two children were treated with isoprinosine and intraventricular interferon but died in less than three years from disease onset. One child did not have any treatment and died after seven years of illness. One child received intraventricular ribavirin and remains alive, but markedly impaired, nine years following diagnosis. CONCLUSION: The CPSP has demonstrated that Canadian paediatricians and paediatric neurologists may encounter cases of SSPE. This report highlights the clinical course of affected Canadian children and provides a review of the disease and its management

Diagnosis and management of nonconvulsive status epilepticus in children

Nonconvulsive status epilepticus (NCSE) encompasses a wide range of diagnoses with variable outcomes and treatment recommendations. In children, NCSE can be observed in various conditions, including acute neurological injuries, specific childhood epilepsy syndromes and other neurological conditions, and can also be observed in individuals with learning difficulties. NCSE in children is thought to be under-recognized, and further studies examining the electrographic characteristics of very young children in NCSE would aid the prompt recognition of additional patients. Some subtypes of NCSE are probably more harmful than others, and long-term prospective studies are needed to evaluate the damaging potential of NCSE itself as opposed to that of the underlying circumstances in which it occurs. Specific data in childhood are clearly lacking, but extrapolation from adult studies indicates that aggressive treatment is most warranted in comatose patients. By contrast, a cautious approach seems to be indicated for absence status epilepticus, complex partial status epilepticus and electrical status epilepticus during sleep