Depdc5 Related Hemimegalencephaly Cn381427
A rare brain malformation where one hemisphere is enlarged, causing seizures and developmental challenges. Treatment involves seizure control and, in some cases, surgical intervention.
Key facts at a glance
- ICD-10
- Q04.5
- ICD-11
- LA00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Refractory focal seizures
Hemiparesis
Increased intracranial pressure
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe developmental delay
(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
Hemispherectomy or hemispherotomy
Surgical disconnection or removal of the enlarged hemisphere to eliminate seizure onset in severe, medication-resistant cases of hemimegalencephaly.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Aggressive antiepileptic drug regimen
Combination therapy with multiple antiepileptic medications to achieve seizure control, often necessary pre-operatively and when surgery is not feasible.
Additional treatment options
mTOR pathway inhibition
Everolimus or sirolimus therapy to potentially reduce abnormal brain growth and seizure burden by modulating cellular proliferation pathways.
medicationIntensive early intervention therapy
Comprehensive rehabilitation program addressing hemiparesis, developmental delays, and functional limitations through targeted exercises and adaptive techniques.
physical_therapySerial neuroimaging and EEG surveillance
Regular MRI imaging to monitor progression of megalencephaly and EEG monitoring to characterize seizure patterns and treatment response.
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 Depdc5 Related Hemimegalencephaly Cn381427?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy