Developmental And Epileptic Encephalopathy 66 C4748070
A genetic encephalopathy causing severe epilepsy and developmental delay in early childhood. Treatment focuses on seizure control and developmental support.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Myoclonic-atonic seizures
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Absence seizures
(neurologic)Limb hypertonia
(neurologic)Dysphagia requiring tube feeding
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Broad-spectrum anti-seizure medication regimen
Initial treatment with broad-spectrum ASMs such as valproate, levetiracetam, or clobazam depending on seizure presentation. Polytherapy may be necessary.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Early intensive motor and sensory therapy
Aggressive early intervention program combining physical and occupational therapy to maximize developmental potential during critical periods.
Additional treatment options
Responsive neurostimulation (RNS)
Closed-loop neurostimulation system that detects seizures and delivers targeted electrical stimulation. Considered for medication-resistant cases with focal onset.
procedureLow glycemic index treatment (LGIT)
Dietary approach using foods with low glycemic index to maintain stable glucose levels, offering moderate seizure control with fewer restrictions than classic ketogenic diet.
dietaryMultidisciplinary developmental surveillance
Regular coordinated assessments by neurology, developmental pediatrics, speech therapy, and nutrition to monitor disease progression and adjust interventions.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Developmental And Epileptic Encephalopathy 66 C4748070?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy