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Epilepsia partialis continua in children with fulminant subacute sclerosing panencephalitis
Ruzica Kravljanac1, Nebojsa Jovic, Milena Djuric
1Institute for Mother and Child Health, University of Belgrade, 6-8 Radoje Dakic Street, 11070 Belgrade, Serbia. djruzica@eunet.rs
Insights
Epilepsia partialis continua can occur in the late stages of subacute sclerosing panencephalitis, a rare measles complication. This rare presentation in unvaccinated children indicates a rapidly fatal disease course.
Area of Science:
- Neurology
- Infectious Diseases
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder caused by persistent measles virus infection.
- Inflammatory central nervous system diseases, including SSPE, can manifest as epilepsia partialis continua (EPC).
Observation:
- Two unvaccinated boys presented with EPC during the terminal phase of atypical SSPE.
- Both cases exhibited rapid cognitive and neurological decline, with one experiencing complex partial seizures and myoclonic jerks.
Findings:
- Diagnosis of SSPE was confirmed by elevated antimeasles antibodies in serum and cerebrospinal fluid.
- EPC in the terminal SSPE phase responded to midazolam but lacked typical EEG epileptic discharges.
- The disease course was fulminant, with survival less than three months from onset to death.
Implications:
- EPC may be an underrecognized manifestation of advanced SSPE.
- This highlights the critical importance of measles vaccination to prevent SSPE.
- The rapid progression underscores the severity of SSPE, even in atypical presentations.
Abstract:
Various inflammatory diseases of central nervous system, including subacute sclerosing panencephalitis, could cause epilepsia partialis continua. Two boys with epilepsia partialis continua with onset in terminal phase of atypical subacute sclerosing panencephalitis have been reported. Children were not vaccinated against measles, and the second case had history of measles at an early age. In both cases, the onset of subacute sclerosing panencephalitis was characterized by altered behavior and cognitive decline with very fast mental and neurological deterioration. One boy was suffering from complex partial seizures and myoclonic jerks synchronous with periodic electroencephalographic pattern. Diagnosis was proved by increased titers of antimeasles antibodies in both serum and cerebrospinal fluid. In terminal phase of the disease, epilepsia partialis continua of localized group of the muscles was diagnosed, with good response to intravenous infusion of midazolam. Surface electroencephalographic recordings during epilepsia partialis continua did not show the epileptic discharges. During the terminal phase of the disease, no other type of seizures and movement disorders were recognized, except epilepsia partialis continua. In spite of the treatment, period from the onset of disease to death lasted less than 3 months, suggesting very fulminant course of subacute sclerosing panencephalitis.
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