Developmental And Epileptic Encephalopathy 64 C4693899
A genetic condition involving severe epilepsy and developmental impairment beginning in infancy. Management includes seizure control and comprehensive supportive care.
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
Focal impaired awareness seizures
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe intellectual disability
(neurologic)Spastic quadriplegia
(neurologic)Progressive microcephaly
(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
Anti-seizure medications (ASMs)
First-line pharmacological management using age-appropriate anti-seizure medications selected based on seizure type and EEG findings. Treatment requires careful titration and monitoring for efficacy and side effects.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Ketogenic diet therapy
High-fat, low-carbohydrate dietary intervention that can reduce seizure frequency in refractory cases. Requires supervision by a metabolic specialist and regular monitoring of metabolic parameters.
Additional treatment options
Vagus nerve stimulation (VNS)
Implantable device that delivers electrical stimulation to the vagus nerve, reducing seizure frequency and severity. Typically considered when medications fail.
procedureNeurodevelopmental physical therapy
Specialized therapy focusing on motor development, balance, and functional mobility. Helps address hypotonia and movement disorders commonly associated with DEE.
physical_therapyContinuous EEG monitoring and developmental assessment
Regular electroencephalographic monitoring to track seizure burden and guide treatment adjustments, combined with periodic developmental evaluations.
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 64 C4693899?
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