Patient will present as → a 63-year-old illiterate female with a history of right motor partial seizures and generalization since the age of 23 years arrives at the emergency room due to acute right hemiparesis. Neurological examination reveals a right hemiparesis, right tactile and pain hypoesthesia, poor fluency, temporal and spatial disorientation, and a Mini-Mental State Examination score of 5 points (one for immediate memory, two for naming, one for repetition, and one for commands). MRI is performed demonstrating a massive AVM in the left hemisphere of the frontotemporoparietal region (9.2 × 6.0 cm) with parenchymal compression and microangiopathy.
Arteriovenous malformation is an abnormal connection between arteries and veins, bypassing the capillary system.
- Arteriovenous malformations (AVMs) are the most dangerous of the congenital vascular malformations with the potential to cause intracranial hemorrhage and epilepsy in many cases
- Brain AVMs occur in about 0.1 percent of the population, one-tenth the incidence of intracranial aneurysms
- Supratentorial lesions account for 90 percent of brain AVMs
- Brain AVMs underlie 1 to 2 percent of all strokes, 3 percent of strokes in young adults, and 9 percent of subarachnoid hemorrhages
Brain AVMs usually present between the ages of 10 and 40 years. In about half of all brain AVMs, intracranial hemorrhage is the first sign (41 to 79 percent)
In people without hemorrhage, signs, and symptoms of a brain AVM may include:
- Seizure: 11-13 percent of patients with AVM present with seizure
- Headache: 0.2 percent of patients with headache and normal neurologic examinations were found to have an AVM
- Focal neurologic deficit
Risk factors include being male and having a family history of AVM.
Angiography is the gold standard for the diagnosis, treatment planning, and follow-up after treatment of brain AVMs
- Brain AVMs are typically first identified on cross-sectional imaging (CT or MRI). MRI is more sensitive, particularly in the setting of an acute intracerebral hemorrhage.
- A combination of MRI and angiography is often used to assess the likely success and risks of surgical, endovascular, or radiosurgical therapy.
Surgical excision is the mainstay of treatment; radiosurgery is a useful option in lesions deemed at high risk for surgical therapy, and endovascular embolization can be a useful adjunct to these techniques
- Treatment for brain AVMs can be symptomatic, and patients should be followed by a neurologist for any seizures, headaches, or focal neurologic deficits.
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Subarachnoid hemorrhage (SAH) is a bleed in the subarachnoid space, which occurs most commonly due to rupture of a berry aneurysm. It can also occur from trauma and AVM. Patients often complain of the ‘worst headache of my life.
Play Video + QuizQuestion 1 |
Computed tomography with contrast Hint: See C for explanation | |
Magnetic resonance imaging (MRI) Hint: See C for explanation | |
Angiography | |
Carotid Doppler ultrasound Hint: Doppler ultrasound of the carotid arteries is not indicated in the diagnosis of AVM |
Question 2 |
Headache Hint: 0.2 percent of patients with a headache and normal neurologic examinations were found to have an AVM | |
Seizure Hint: 11-13 percent of patients with AVM | |
Intracranial hemorrhage | |
Focal neurologic deficit Hint: This presentation is fairly unusual for cerebral AVM. While a vascular steal syndrome has been hypothesized to cause this presentation, in most cases this is related to mass effect, hemorrhage, or seizure. |
Question 3 |
Trauma Hint: Trauma is more likely to cause epidural or subdural hematoma. | |
Ruptured aneurysm | |
Bleeding arteriovenous malformation Hint: Blood from a ruptured arteriovenous malformation can be intraparenchymal and cause focal neurologic symptoms. | |
Embolic stroke | |
Primary intracerebral hemorrhage |
List |
References: Merck Manual · UpToDate