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Research paper

Encephalopathy with status epilepticus during sleep or continuous spikes and waves during slow sleep syndrome: a multicenter, long-term follow-up study of 117 patients

Multicenter long-term follow-up of 117 CSWS / ESES patients. Broad etiology distribution; persistent cognitive and/or behavioral sequelae in a majority despite EEG/seizure remission. Etiology-stratified prognosis: structural + surgery → better outcomes; unknown / genetic → worse outcomes.

Indexed context

Caraballo RH, et al.

esescswslong-term-outcomemulticenterprognosis

Markdown path

content/research/papers/2013-caraballo-csws-eses-117-patients.md

Findings

Multicenter long-term follow-up of 117 CSWS / ESES patients. Broad etiology distribution; persistent cognitive and/or behavioral sequelae in a majority despite EEG/seizure remission. Etiology-stratified prognosis: structural + surgery → better outcomes; unknown / genetic → worse outcomes.

Why it may matter for Levi

Quantitatively reinforces Liukkonen 2010: electrographic remission does not guarantee developmental recovery. Levi's structurally unremarkable MRI combined with three negative germline workups places him in the harder-prognosis population unless a mosaic or epigenetic diagnosis opens a targeted-therapy path. Underscores the priority of mosaic-sensitive PROS panel and methylation / episignature panel.

Paper text

Caraballo et al. (2013) — Multicenter long-term follow-up of 117 CSWS / ESES patients

Source

Why this paper is in the corpus

One of the largest multicenter long-term follow-up cohorts in CSWS / ESES. Confirms the pattern established in Liukkonen 2010 — EEG and seizure remission but persistent neurodevelopmental sequelae — across a larger patient population and with multicenter data. Provides a strong prognostic reference and etiological distribution anchor.

Key findings

  • 117 patients with ESES / CSWS from multiple centers followed long-term.
  • Broad etiological distribution including structural, genetic, and unknown causes.
  • Persistent cognitive and/or behavioral sequelae in a majority of patients despite EEG and seizure remission.
  • Prognosis influenced by etiology: structural cases with successful surgery often returned to baseline cognitive development, while unknown / genetic etiologies frequently retained intellectual disability.

Levi-relevant takeaways

  • Quantitatively reinforces the Liukkonen 2010 finding that electrographic remission does not guarantee developmental recovery.
  • The etiology-stratified prognosis (structural + surgery → better outcomes; unknown / genetic → worse outcomes) is relevant to Levi. His structurally unremarkable MRI, combined with three negative germline workups, places him in the harder-prognosis population unless a mosaic or epigenetic diagnosis opens a targeted-therapy path.
  • Underscores the priority of pursuing the mosaic-sensitive tissue-based PROS panel and the methylation / episignature panel — identifying an etiology is prognostically meaningful and may be therapeutically actionable.

Citation note

Referenced as [22] in the 2026-04-21 user-supplied comprehensive DEE-SWAS / ESES / CSWS research report.