Last modified: 2018-09-09
Abstract
Introduction: Sub acute sclerosing pan encephalitis (SSPE) is a devastating, acquired neurodegenerative disorder occurring after measles infection. SSPE has pathogonomic clinical and electroencephalography (EEG) features, but unusual presentations and electro graphical variability can pose a diagnostic challenge for the clinicians. Methods: The encephalographic characteristics of children diagnosed with SSPE in the last eight years (2010-2018) were reviewed and recorded in a predesigned proforma.Results: A total of 34 children (M: F-1.8:1) in the age range of 2-13 years (median: 8yrs) were diagnosed with SSPE, based on positive CSF measles antibodies, in a span of eight years. There was a past history of measles in 31 patients (90%). Majority of patients presented to the hospital in stage II (n=27,80%) followed by Stage I (n=6,18%) and stage III (n=1,3%). Major seizures types were slow myoclonus (n=30, 88%) and drop attacks (n=14, 41%) followed by generalized seizures (n=15, 44%) and focal seizures (n=2, 6%). EEG findings had slow background and super imposable delta bursts (n=25, 74%) and electro-decrement response (n=14, 41%). Atypical EEG observations noted were focal (n=17,frontal:15, central:3, occipital:4,temporal:1and parietal:1), multifocal epileptiform discharges (n=5,15%), generalized spike and slow wave discharges (n=7,20%), generalized sharp and slow wave (n=11,32%). The mean duration of follow up was two years, six patients (18%) expired and remaining patients were either bedbound or caregiver dependent. Conclusion: Diagnosis of SSPE is usually not difficult if both the EEG and clinical findings are considered. But, knowledge of atypical electrophysiological findings is necessary for the early diagnosis and management.