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Inducing Post-Traumatic Epilepsy in a Mouse Model of Repetitive Diffuse Traumatic Brain Injury
Published on: February 10, 2020
[Devastating epileptic encephalopathy-pseudoencephalitis: the new type of catastrophe epilepsy in our department]
Magdolna Neuwirth1, Eva Paraicz, Zoltán Liptai
1Magyar Református Egyház Bethesda Gyermekkórháza, Neurol6giai Osztály, Budapest. neuwirth.magda@bethesda.hu
Insights
This study highlights devastating epileptic encephalopathy in children, initiated by prolonged status epilepticus (SE) and fever. These cases demonstrate severe neurological decline despite treatment, emphasizing the aggressive nature of this condition.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroscience
Context:
- Intractable epilepsy in children presents significant management challenges.
- Status epilepticus (SE) is a medical emergency with potential long-term neurological sequelae.
- Fever of unknown cause can precede severe neurological conditions.
Purpose:
- To analyze the clinical history of five pediatric patients with intractable epilepsy.
- To investigate the onset of epilepsy following prolonged status epilepticus (SE) and high-grade fever.
- To characterize the progression and outcomes of devastating epileptic encephalopathy.
Summary:
- Five children with normal development experienced intractable epilepsy after prolonged SE and unexplained fever.
- Epilepsy onset was immediate post-SE, with seizures unresponsive to antiepileptic drugs.
- Neurological imaging revealed temporal and frontal lobe damage, cognitive decline, and behavioral issues, with one patient succumbing to the condition.
Impact:
- This case series underscores the severe prognosis of epileptic encephalopathy following SE in children.
- Findings suggest a distinct clinical entity requiring further investigation and specialized care.
- Highlights the need for early recognition and aggressive management strategies for SE in pediatric populations.
Purpose:
Analysis of history of our five patients with intractable epilepsy whose illness have begun with prolonged status epilepticus (SE) and high-grade fever of unknown cause.
Methods:
Retrospective study analysis of selected five intractable epileptic patients at a median age of 11.5 (8-14) years.
Results:
All children had normal development before epilepsy begun. Intractable SE lasted 3-10 (median seven) days by four patients and three months by one patient. The cause of illness was unknown at the beginning and the MRI were normal. Intractable epilepsy followed the SE in all cases without any latent period. Follow-up of the children was 3-15 (median 9.5) years. The seizures came continually with few-day-long breaks, antiepileptic drugs were ineffective. Semiology of seizures, EEG, and functional imaging examinations (PET, SPECT) referred to temporal and frontal lobe damages. Later on, the MR images showed hippocampal sclerosis in one patient and mild generalized brain atrophy in the others. During the years, cognitive deterioration and behavioral problems have been realized. The most severe patient developed tetraparesis, fell in vigil coma and died after five years.
Conclusions:
The symptoms of our patients fulfilled the criteria of devastating epileptic encephalopathy in school-aged children.
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