Arteriovenous Malformations Clinical Presentation

Updated: Dec 04, 2016
  • Author: Souvik Sen, MD, MPH, MS, FAHA; Chief Editor: Helmi L Lutsep, MD  more...
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Presentation

History

AVMs tend to be clinically silent until the presenting event occurs. Therefore, the diagnosis usually is made at the time of the first seizure or hemorrhage.

A history of minor learning disability is present in as many as two thirds of patients; such dysfunction is rarely apparent in adult life.

A history of headaches is present in as many as half of patients with cerebral AVM. The headaches subsequently may take the form of classic migraine or more generalized headache.

If seizures have occurred, a careful seizure history should be obtained. Seizures are simple, partial, or secondarily generalized.

The effectiveness of anticonvulsant therapy should be observed carefully and monitored before and during treatment.

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Physical

Focal neurologic findings are rare in the absence of seizure or hemorrhage in patients with cerebral AVMs. They are more common in brainstem and deeply located AVMs. They are associated with patient’s age and are more common among women.

Detailed neuropsychological testing may disclose subtle right or left hemisphere dysfunction.

If parenchymal hemorrhage is present, the physical findings are indistinguishable from those due to intracranial hemorrhage of other causes.

Intraventricular hemorrhage generally produces a less severe neurological deficit than hemorrhage into other areas of the brain.

In the rare patients in whom focal neurological deficits are present, the deficit may reflect the location of the AVM.

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Causes

No genetic, demographic, or environmental risk factors for cerebral AVM have been identified clearly.

Families with cerebral AVMs are rare, and such pedigrees have been too small to enable linkage studies. From the few family cases reported, the inheritance appears to be autosomal dominant.

In a small minority of cases, cerebral AVMs are associated with other inherited disorders, such as the Osler-Weber-Rendu syndrome (ie, hereditary hemorrhagic telangiectasia), Sturge-Weber disease, neurofibromatosis, and von Hippel-Lindau syndrome.

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