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.
monitoringMedication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy