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Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis
Y Mikaeloff1, I Jambaqué, L Hertz-Pannier
1Neuropediatric Department, University Hospital, Bicêtre, France. yann.mikaeloff@tnn.aphp.fr
Epilepsy Research
|February 14, 2006
Summary
We identified Devastating Epileptic encephalopathy in School-age Children (DESC), a severe epilepsy syndrome. It starts with prolonged seizures triggered by fever and leads to intractable epilepsy and cognitive decline.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Epileptic encephalopathies can present with severe neurological deficits.
- A specific subtype of epilepsy in children has been overlooked.
- Understanding new epilepsy syndromes is crucial for diagnosis and treatment.
Purpose of the Study:
- To characterize a previously unrecognized epileptic encephalopathy.
- To describe its presentation as intractable bilateral perisylvian epilepsy.
- To identify its onset with prolonged status epilepticus in school-aged children.
Main Methods:
- Retrospective study of normally developing children with prolonged status epilepticus (SE).
- Analysis of clinical data, EEG, CSF, and MRI findings over 7 years.
- Follow-up assessment of seizure control and cognitive sequelae.
Main Results:
- Fourteen children with a median age of 7.5 years were included.
- Prolonged SE (median 30 days) was followed by intractable epilepsy in all cases.
- All patients developed major cognitive sequelae, with fronto-temporal impairment suggested on neuropsychology.
Conclusions:
- A recognizable epilepsy pattern, Devastating Epileptic encephalopathy in School-age Children (DESC), was identified.
- DESC begins with prolonged SE triggered by fever of unknown cause.
- It persists as intractable perisylvian epilepsy with severe cognitive deterioration.
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