Developmental and epileptic encephalopathy

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Developmental and epileptic encephalopathy with spike-wave activation in sleep

ORPHA:725Disease

Also called Epileptic encephalopathy with continuous spike-and-wave during slow sleep · Epileptic encephalopathy with spike-and wave activation in sleep · CSWS · CSWSS syndrome · Continuous spikes and waves during sleep · Continuous spikes and waves during slow-wave sleep · DEE-SWAS

What it is

Continuous spikes and waves during sleep (CSWS) is a rare epileptic encephalopathy of childhood characterized by seizures, an electroencephalographic (EEG) pattern of electrical status epilepticus in sleep (ESES) and neurocognitive regression in at least 2 domains of development.

Key facts

Age of onset
Childhood
Inheritance
Autosomal dominant, Not applicable
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

GRIN2ADisease-causing germline mutation(s)

ICD-10 codes

G40.4filed under a broader ICD-10 category — shared with 28 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Benefit programs to look at

Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.

Cross-references

GARD 1513MEDDRA 10078827MONDO 0019123OMIM 245570UMLS C5552731

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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