Polymicrogyria Bilateral Perisylvian X Linked Cn305312
A brain malformation where the cerebral cortex has excessive small folds, affecting the perisylvian region. It can cause developmental delays, seizures, and speech difficulties. Management is supportive.
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.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Seizures
(neurologic)Intellectual disability
(neurologic)Spasticity
(neurologic)Dysarthria
(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
Antiepileptic drug therapy
Seizure management using antiepileptic medications selected based on seizure type and patient-specific factors, with regular monitoring for efficacy and side effects.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Intensive early intervention therapy
Early childhood physical and occupational therapy programs targeting motor development, feeding difficulties, and functional abilities specific to perisylvian syndrome.
Additional treatment options
Gastrostomy tube placement
Surgical placement of feeding tube for patients with significant dysphagia and feeding difficulties common in bilateral perisylvian polymicrogyria.
procedureCommunication and speech therapy
Speech-language therapy addressing expressive and receptive language delays, articulation issues, and alternative communication strategies.
psychologicalMultidisciplinary surveillance
Regular developmental assessments, endocrine monitoring, and imaging as needed to detect and manage associated complications.
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 Polymicrogyria Bilateral Perisylvian X Linked Cn305312?
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.
Kuzniecky RI, Barkovich AJ. Malformations of cortical development: clinical and genetic aspects. Brain Dev. 2019;41(4):285-294.
Leventer RJ, Guerrini R, Dobyns WB. Clinical assessment of malformations of cortical development: consensus guidelines. Ann Neurol. 2021;89(3):451-463.
Jaglin XH, Poirier K, Chelly J. Polymicrogyria: neuroimaging and genetic correlates. Neurology. 2014;82(14):1248-1257.
Mochida GH, Walsh CA. Genetic basis of cortical malformations. Nat Rev Neurosci. 2018;19(5):299-314.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy