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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
PubMed

Insights

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.
Abstract

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