ICNC Abstracts, ICNC 2018

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Electrophysiological characteristics in children with Subacute sclerosing panencephalitis (SSPE): A study from a tertiary care hospital.
ANSHITA ARORA, SHILPA KULKARNI, MEENAL GARG, SONAM KOTHARI, ANAITA HEGDE, KRISHANKUMAR SHAH

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.

 


Keywords


Subacute sclerosing panencephalitis, measles antibodies, myoclonus, superimposable delta bursts

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