Developmental And Epileptic Encephalopathy 88 C5394553
A rare genetic epilepsy causing early-onset seizures and significant developmental delays. Seizure management with anti-epileptic drugs and supportive care are the main treatments. Outlook depends on the specific genetic cause.
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
Seizures
Refractory epilepsy
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Spastic quadriplegia
(neurologic)Cortical atrophy
(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)
Comprehensive seizure management using ASMs selected based on seizure classification, genetic findings when available, and individual response patterns.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Early intervention services
Coordinated early intervention program including physical, occupational, and speech therapy to address global developmental delays.
Additional treatment options
Low glycemic index treatment (LGIT)
Dietary therapy maintaining stable blood glucose levels through low glycemic index foods, offering a less restrictive alternative to ketogenic diet.
dietaryFamily-centered behavioral support
Psychological support and behavioral interventions for the patient and family to address challenges associated with severe developmental encephalopathy.
psychologicalResponsive neurostimulation (RNS)
Implantable device that detects seizures and delivers targeted electrical stimulation to reduce seizure frequency.
procedureWhen 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 88 C5394553?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy