Developmental And Epileptic Encephalopathy 90 C5542345
A rare genetic epilepsy causing severe seizures and impaired development in early life. Treatment involves anti-epileptic medications and comprehensive supportive therapies. Care coordination among specialists is typically needed.
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
Early-onset seizures
Status epilepticus
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe developmental delay
(neurologic)Autonomic dysfunction
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Individualized antiseizure medication regimen
Customized pharmacological approach based on seizure semiology and EEG findings. Multiple medication trials may be necessary to achieve optimal seizure control.
Modified Atkins diet
Less restrictive alternative to ketogenic diet that may improve seizure control while allowing more dietary flexibility. Requires supervision by a metabolic specialist.
Additional treatment options
Responsive neurostimulation
Implantation of a device that detects seizure activity and delivers targeted electrical stimulation to abort seizures. Reserved for patients with focal-onset seizures.
surgicalNeuropsychological support services
Cognitive-behavioral therapy and neuropsychological interventions to address behavioral challenges, anxiety, and cognitive limitations associated with the condition.
psychologicalContinuous EEG surveillance
Regular electroencephalographic monitoring to assess seizure burden, evaluate treatment response, and identify epileptiform activity patterns.
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 90 C5542345?
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 20, 2026. · Read our editorial policy