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VI. Diagnosis

*1. Clinical features

AVMs can be asymptomatic or symptomatic, but the great development and advances in imaging methods have made it possible to diagnose incidental AVMs earlier nowadays.
In cases of symptomatic AVMs, the main clinical manifestations are : intracranial hemorrhage, seizures, and headache. Approximately 50% of symptomatic patients (42 to 72% in the literature) present intracranial hemorrhage, the first event happening between 20 and 40 years of age, in both male and female [20]. AVM rupture may result in subarachnoid, intracerebral or intraventricular hemorrhage.

Epilepsy comes in second place, occurring in 34% of the cases, mainly simple partial seizures and complex partial seizures. Headaches is the first symptom in 31% of the cases, without any specific feature. Focal neurological signs are present in 40% of the patients [58]. Other manifestations should also be considered, such as focal motor and sensitive deficits, speech disorder, visual deficits, and so forth.

Part of the symptoms are related to the angio architecture : as patients with afferent meningeal arteries are more prone to headache ; as previously mentioned, more risk of hemorrhage in previous rupture, large AVM size, infratentorial and deep locations, intranidal aneurysm, etc. ; progressive neurologic deficit by flow steal phenomenon in large AVMs.

The clinical presentation of the Vein of Galen malformation varies according to age. Neonates tend to present with high-output cardiac failure, pulmonary hypertension, and, in more severe cases, multiorgan system failure. Infants commonly present with hydrocephalus, seizures, or neurocognitive delay. Older children and adults usually present with headaches or intracranial hemorrhage.

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