CLINICAL EVALUATION OF CHILDRENS WITH SUBACUTE SCLEROSING PANENCEPHALITIS

Objective: The purpose of this study was to investigate retrospectively the clinical, electrophysiological and neuro radiological features of patients with subacute sclerosing panencephalitis (SSPE). Material and Method: A total of 16 children with SSPE admitted to our clinic were retrospectively evaluated in terms of clinical, electrophysiological and neuroradiological features. The clinical stage of patients at admission was determined based on Risk & Haddad classification. The clinical stages and findings of patients were studied at the follow-up period. Results: Of all the patients twelve of them were male (75%) and four of them were female (25%). The average age was 8.25 years old at the time of diagnosis. All of the patients had measles before. Most patients (87.5%) had history of measles infection before three years and some patients (18.8%) have a history of measles infection before one years of age. The symptoms began between 3-12 years of age. Stereotypic attacks and behavorial changes were the common initial symptoms. At the time of diagnosis 10 patients were grade 2A, 5 patients were grade 2B an one patient was grade 2C. In 10 (62.5%) of cases, electroencephalography showed the characteristics periodic complexes with high slow wave activity of subacute sclerosing panencephalitis. Four of patients had periodic latelarized discharges. In nine (56.2%) of cases, the clinical status was progressive in five months, four (25%) patients were comatose and they were died in this period. Conclusion: SSPE has a gradual progressive course leading to death within one to three years. Most patients have a history of natural measles infection before three years of age. Seven of the patients had had active measles infection despite vaccination. This may be related to primary or secondary failure vaccination or immune responses of host.