Lissencephaly With Cerebellar Hypoplasia Type D C4274991
A severe developmental brain disorder affecting both brain surface formation and cerebellar development. It manifests early in life with profound impairment. Treatment is primarily supportive and symptomatic.
Key facts at a glance
- ICD-10
- G43.909
- 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
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Developmental delay
(neurologic)Ataxia
(neurologic)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
Adaptive Motor Skills Training
Structured program focusing on developing adaptive strategies for movement, posture control, and functional mobility appropriate to the patient's abilities and limitations.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Seizure Control and EEG Monitoring
Regular electroencephalogram monitoring combined with seizure diary review and medication management to achieve optimal seizure control and minimize impact on development.
Additional treatment options
Vagus Nerve Stimulation Evaluation
Surgical evaluation for vagus nerve stimulator placement in patients with drug-resistant epilepsy associated with lissencephaly and cerebellar hypoplasia.
procedureKetogenic Diet Assessment
Evaluation for ketogenic diet therapy as an adjunctive treatment for seizure control, particularly in patients with difficult-to-control seizures.
dietaryCognitive and Behavioral Support Services
Psychological assessment and ongoing support services addressing cognitive challenges, behavioral concerns, and emotional well-being for patients and caregivers.
psychologicalWhen 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 Lissencephaly With Cerebellar Hypoplasia Type D C4274991?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Lissencephaly With Cerebellar Hypoplasia Type D C4274991. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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