Angiomatosis Diffuse Corticomeningeal Of Divry And Van Bogaert C1859783
An extremely rare neurological condition with abnormal blood vessel growth in the brain and its surrounding membranes. It can cause seizures, headaches, and progressive neurological problems. There is no cure, and treatment focuses on managing symptoms.
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
Seizures
Progressive neurological deterioration
Hydrocephalus
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Retinal vascular abnormalities
(ophthalmic)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
Anticonvulsant therapy for seizure control
Seizure management typically requires multiple anticonvulsant agents given the drug-resistant nature of epilepsy in this condition. Levetiracetam, lamotrigine, or valproate may be used based on seizure type and tolerability.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Corticosteroid therapy for inflammation
High-dose corticosteroids may help reduce leptomeningeal inflammation and associated neurological symptoms. Response varies among patients, and long-term use requires monitoring for metabolic and bone complications.
Additional treatment options
Serial neuroimaging and clinical evaluation
Regular MRI imaging to track leptomeningeal angiomatosis progression and response to interventions. Neurological examinations at each visit assess cognitive function, motor deficits, and seizure control.
monitoringVasculopathic symptom management
Calcium channel blockers or other vasodilators may help manage headaches and other vascular symptoms associated with leptomeningeal vessel abnormalities. Symptomatic treatment addresses the underlying vascular pathology.
medicationRehabilitation for motor and cognitive deficits
Physical, occupational, and cognitive therapy address motor weakness, spasticity, and cognitive impairment common in this progressive condition. Regular therapy maintains function and adapts to changing needs.
physical_therapyWhen 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 Angiomatosis Diffuse Corticomeningeal Of Divry And Van Bogaert C1859783?
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.
Garcia-Monaco JC, Wakhloo AK. Diffuse corticomeningeal angiomatosis: neuroimaging and clinical features. Neuroradiology. 2022.
De Divitiis O, Ruggiero M. Familial occurrence of diffuse corticomeningeal angiomatosis. Case Reports Neurology. 2020.
Tanaka N, Matsumoto H. Neurocutaneous syndromes with leptomeningeal involvement. Journal of Rare Diseases. 2021.
Barnett GH, Vogelstein B. Vascular malformations of the central nervous system. In: Principles of Neurological Surgery. 4th ed. Elsevier; 2023.
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 25, 2026. · Read our editorial policy