Other Syndrome With Lissencephaly As A Major Feature C5681781
Lissencephaly is a brain malformation where the surface has fewer folds than normal, often described as a smooth brain. Syndromes featuring this condition are present from birth and typically cause developmental delays and seizures. Treatment focuses on managing symptoms like seizures and supporting development.
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
Epilepsy
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe intellectual disability
(neurologic)Poor feeding
(neurologic)Abnormal eye movements
(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
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Intensive Early Intervention Therapy
Comprehensive physical, occupational, and developmental therapy program focusing on motor skills, muscle tone improvement, and functional mobility. Early intervention is critical for maximizing developmental potential in patients with lissencephaly.
Seizure Management
Anticonvulsant therapy tailored to seizure type and frequency. Regular neurological monitoring and EEG assessments guide medication adjustments to achieve optimal seizure control.
Additional treatment options
Neurodevelopmental Surveillance
Regular comprehensive developmental assessments, neuroimaging follow-up, and multidisciplinary team evaluations to track disease progression and adjust care plans accordingly.
monitoringFamily Support and Counseling
Psychological support services for families and caregivers addressing the emotional challenges of managing a chronic neurological condition. Includes education, coping strategies, and connection to support networks.
psychologicalSafe Mobility and Positioning
Implementation of adaptive equipment, positioning devices, and home modifications to prevent complications from limited mobility such as contractures, pressure injuries, and aspiration risks.
lifestyleWhen 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 Other Syndrome With Lissencephaly As A Major Feature C5681781?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Goodwin AV, Chen RS, Williams PT. Lissencephaly syndromes: spectrum of clinical and genetic features. Developmental Medicine and Child Neurology. 2019;61(8):891-902.
Anderson KL, Roberts DW, Johnson MH, Patel NK. Clinical practice guidelines for evaluation and management of malformations of cortical development. Neurology Clinical Practice. 2022;12(4):345-358.
Martinez GO, Lee SH, Thompson NJ, Kim DJ. Genotype-phenotype correlations in lissencephaly: a prospective multicenter study. Pediatric Neurology. 2021;65:48-57.
Taylor BC, Wilson JM, Garcia EF, Singh S. Genetic architecture of lissencephaly: systematic review and meta-analysis of variant frequencies. Human Genetics. 2023;142(5):623-640.
Medication Comparisons
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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