What is a brain AVM?

Normally, arteries carry blood containing oxygen from the heart to the brain, and veins carry
blood with less oxygen away from the brain and back to the heart. When an arteriovenous
malformation (AVM) occurs, a tangle of blood vessels in the brain or on its surface bypasses
normal brain tissue and directly diverts blood from the arteries to the veins.

How common are brain AVMs?

Brain AVMs occur in less than 1 percent of the general population. It’s estimated that about
one in 2,000–5,000 people may have an AVM. AVMs are more common in males than in
females.

Why do brain AVMs occur?

We don’t know why AVMs occur. Brain AVMs are usually congenital, meaning someone is
born with one. But they’re usually not hereditary. People probably don’t inherit an AVM
from their parents, and they probably won’t pass one on to their children.

Where do brain AVMs occur?

Brain AVMs can occur anywhere within the brain or on its covering. This includes the four
major lobes of the front part of the brain (frontal, parietal, temporal, occipital), the back part
of the brain (cerebellum), the brainstem, or the ventricles (deep spaces within the brain that
produce and circulate the cerebrospinal fluid).

Do brain AVMs change or grow?

Most AVMs don’t grow or change much, although the vessels involved may dilate (widen).
Some AVMs may shrink due to clots in part of the AVM. Some may enlarge to redirect blood
in adjacent vessels toward an AVM.

What are the symptoms of a brain AVM?

Symptoms may vary depending on where the AVM is located:

More than 50 percent of patients with an AVM have an intracranial hemorrhage.

Among AVM patients, 20 percent to 25 percent have focal or generalized seizures.
Patients may have localized pain in the head due to increased blood flow around an AVM.
Fifteen percent may have difficulty with movement, speech and vision.
What causes brain AVMs to bleed?

A brain AVM contains abnormal and, therefore, “weakened” blood vessels that direct blood
away from normal brain tissue. These abnormal and weak blood vessels dilate over time.
Eventually they may burst from the high pressure of blood flow from the arteries, causing
bleeding into the brain.

What are the chances of a brain AVM bleeding?

The chance of a brain AVM bleeding is 1 percent to 3 percent per year. Over 15 years, the
total chance of an AVM bleeding into the brain — causing brain damage and stroke — is 25
percent.

Does one bleed increase the chance of a second bleed?

The risk of recurrent intracranial bleeding is slightly higher for a short time after the first
bleed. In two studies, the risk during the first year after initial bleeding was 6 percent and
then dropped to the baseline rate. In another study, the risk of recurrence during the first year
was 17.9 percent. The risk of recurrent bleeding may be even higher in the first year after the
second bleed and has been reported to be 25 percent during that year. People who are
between 11 to 35 years old and who have an AVM are at a slightly higher risk of bleeding.

What can happen if a brain AVM causes a bleed?

The risk of death related to each bleed is 10 percent to 15 percent. The chance of permanent
brain damage is 20 percent to 30 percent. Each time blood leaks into the brain, normal brain
tissue is damaged. This results in loss of normal function, which may be temporary or
permanent. Some possible symptoms include arm or leg weakness/paralysis, or difficulty
with speech, vision or memory. The amount of brain damage depends on how much blood
has leaked from the AVM.

What functions does an AVM affect?

The functions of the lobes of the brain is very complicated as all lobes have some overlapping
functions so that there is communication between them in order for all processes to integrate
information. At a basic level, the main functions of the brain lobes are below:

The frontal lobe functions to process motor (movements), and frontal eye fields, regulates
personality and articulation (and other aspects) of speech.
The parietal lobe functions to process sensory information, such as interpretation of pain and
temperature, light touch, vibration and more.
The temporal lobe functions to process things related to hearing, memory, learning and
receptive speech.
The occipital lobe functions to process things related to vision.
Are there different types of brain AVMs?

All blood vessel malformations involving the brain and its surrounding structures are
commonly referred to as AVMs. But several types exist:

True arteriovenous malformation (AVM). This is the most common brain vascular
malformation. It consists of a tangle of abnormal vessels connecting arteries and veins with
no normal intervening brain tissue.
Occult or cryptic AVM or cavernous malformations. This is a vascular malformation in the
brain that doesn’t actively divert large amounts of blood. It may bleed and often produce
seizures.
Venous malformation. This is an abnormality only of the veins. The veins are either enlarged
or appear in abnormal locations within the brain.
Hemangioma. These are abnormal blood vessel structures usually found at the surface of the
brain and on the skin or facial structures. These represent large and abnormal pockets of
blood within normal tissue planes of the body.
Dural fistula. The covering of the brain is called the “dura mater.” An abnormal connection
between blood vessels that involve only this covering is called a dural fistula. Dural fistulas
can occur in any part of the brain covering. Three kinds of dural fistulas are:
- Dural carotid cavernous sinus fistula. These occur behind the eye and usually cause
symptoms because they divert too much blood toward the eye. Patients have eye swelling,
decreased vision, redness and congestion of the eye. They often can hear a “swishing” noise.
- Transverse-Sigmoid sinus dural fistula. These occur behind the ear. Patients usually
complain of hearing a continuous noise (bruit) that occurs with each heartbeat, local pain
behind the ear, headaches and neck pain.
- Sagittal sinus and scalp dural fistula. These occur toward the top of the head. Patients
complain of noise (bruit), headaches, and pain near the top of the head; they may have
prominent blood vessels on the scalp and above the ear.
What is the best treatment for a dural fistula?

The best treatment is usually endovascular surgical blocking of the abnormal connections that
have caused the fistula. This involves guiding small tubes (catheters) inside the blood vessel
with X-ray guidance and blocking off the abnormal connections. Depending on the location
and size, many of these can be treated and cured by these less invasive endovascular
techniques.

How are AVMs diagnosed?

Most AVMs are detected with either a computed tomography (CT) brain scan or a magnetic
resonance imaging (MRI) brain scan. These tests are very good at detecting brain AVMs.
They also provide information about the location and size of the AVM and whether it may
have bled. A doctor may also perform a cerebral angiogram. This test involves inserting a
catheter (small tube) through an artery in the leg (groin). Then it’s guided into each of the
vessels in the neck going to the brain, and a contrast material (dye) is injected and pictures
are taken of all the blood vessels in the brain. For any type of treatment involving an AVM,
an angiogram may be needed to better identify the type of AVM.

What factors influence whether an AVM should be treated?

In general, an AVM may be considered for treatment if it has bled, if it’s in an area of the
brain that can be easily treated and if it’s not too large.

What is the best treatment for an AVM?

It depends on what type it is, the symptoms it may be causing and its location and size.
What different types of treatment are available?

Medical therapy. If there are no symptoms or almost none, or if an AVM is in an area of the
brain that can’t be easily treated, conservative medical management may be indicated. If
possible, a person with an AVM should avoid any activities that may excessively elevate
blood pressure, such as heavy lifting or straining, and avoid blood thinners like warfarin. A
person with an AVM should have regular checkups with a neurologist or neurosurgeon.
Surgery. If an AVM has bled and/or is in an area that can be easily operated upon, then
surgical removal may be recommended. The patient is put to sleep with anesthesia, a portion
of the skull is removed, and the AVM is surgically removed. When the AVM is completely
taken out, the possibility of any further bleeding should be eliminated.
Stereotactic radiosurgery. An AVM that’s not too large, but is in an area that’s difficult to
reach by regular surgery, may be treated with stereotactic radiosurgery. In this procedure, a
cerebral angiogram is done to localize the AVM. Focused-beam high energy sources are then
concentrated on the brain AVM to produce direct damage to the vessels that will cause a scar
and allow the AVM to “clot off.”
Interventional neuroradiology/endovascular neurosurgery. It may be possible to treat part or
all of the AVM by placing a catheter (small tube) inside the blood vessels that supply the
AVM and blocking off the abnormal blood vessels with various materials. These include
liquid tissue adhesives (glues), micro coils, particles and other materials used to stop blood
flowing to the AVM. The best treatment depends on the symptoms the patient is having, what
type of AVM is present and the AVM’s size and location.
What doctors specialize in treating brain AVMs?

Vascular neurosurgeons specialize in surgically removing brain AVMs.

Radiation therapists/neurosurgeons specialize in the stereotactic radiosurgery treatment of
brain AVMs.
Interventional neuroradiologists/endovascular neurosurgeons specialize in the endovascular
therapy of brain AVMs.
Stroke neurologists specialize in the medical management of brain AVMs.
Neuroradiologists specialize in the diagnosis and imaging of the head, neck, brain and spinal
cord. They perform and interpret the CT, MRI, and cerebral angiograms necessary for
evaluation, management and treatment. Each of these specialists has had advanced training
and is highly skilled at treating complex brain vascular malformations

When Survivors Are Hurting: Understanding Post-Stroke Pain

The stroke recovery journey is often filled with challenges. The physical, emotional,
behavioral and communication changes caused by stroke change the lives of not only the
survivor, but those who care for them as well. Post-stroke pain can further complicate the
situation.

Up to half of stroke survivors may experience some type of pain after their stroke. Neuro-
scientists and therapists distinguish four types of post-stroke pain: central pain syndrome
(CPS); complex regional pain syndrome (CRPS); spasticity; and shoulder pain.

Pain Terminology — Central & Neuropathic Pain

Dr. Richard Harvey

Let’s start with some basic definitions. Central pain is due to an injury in the central nervous
system (brain or spinal cord). Any pain that occurs in the peripheral nervous system
(anywhere along the nerves) is peripheral pain. “If you crush your arm in an accident, you are
at risk of developing severe peripheral neuropathic or nerve pain,” said Richard Harvey,
medical director of the Center for Stroke Rehabilitation at the Shirley Ryan AbilityLab
(formerly Rehabilitation Institute of Chicago). “If you have a stroke or a spinal cord injury,
you may develop central pain.”

Another pertinent distinction is between nociceptive pain and neuropathic pain. Nociceptive
occurs because of tissue damage; neuropathic is the result of nerve damage, regardless of
whether tissue is damaged. “Some people with diabetes have peripheral neuropathy caused
by the small nerve endings in their feet being damaged because of poor blood flow,” Harvey
said. That foot pain is called diabetic neuropathy. “That is a form of neuropathic pain because
it’s due to damage to small nerve endings, not to tissue — skin, muscle or bone — damage.
It’s the nerve being dysfunctional.”

Central Pain Syndrome

Central pain syndrome occurs when there is damage to an area of the brain that carries lots of
sensory pathways. It affects 8 percent to 10 percent of survivors. No particular type of stroke
causes CPS, rather strokes in particular areas of the brain do. For instance, the brainstem is
full of such sensory pathways, so a stroke involving those pathways puts a survivor at risk of
CPS. The same is true of a stroke in the thalamus, which is also rich in nerves. (For many
years, CPS was called thalamic pain syndrome because that was where it was often identified.
It was also known as Dejerine-Roussy syndrome after the two neuroscientists that first
identified it in the early 20th century.) “Starting in the 1960s, scientists determined that
essentially any injury along the sensory pathway from anywhere in the spinal cord up through
the brain to the cortex can cause CPS,” Harvey said. “But it has to injure the sensory
pathway, basically, the nerves that carry pain and temperature sensation. So, if you injure that
pathway — anywhere in the brainstem, the thalamus or between the thalamus and the cortex
— you can get CPS.”

Experimental Treatment for CPS

Motor cortex stimulation is an experimental treatment that is currently being tested. In it, a
stimulator is surgically implanted over the motor cortex, and it gives a continuous stimulation
that doesn’t result in movement but can result in pain reduction. “In some small studies, that
pain reduction has been as much as 90 percent and lasts as long as two years,” Harvey said.
But he cautions that it has not been well studied and since there are risks to doing such
surgeries, it’s not yet recommended as the standard of care. “But it’s very intriguing,
especially because it’s the motor cortex and not the sensory cortex.”

Typically, CPS doesn’t start right away, but usually shows up in the first 60 days after the
stroke. Neuroplasticity during that time results in inappropriate signaling within the pain
pathways that results in a perception of pain that isn’t due to any peripheral cause. Harvey
referred to this as “aberrant neuroplasticity,” changes to the brain that do not produce a
beneficial outcome. “One of the things that people can misconstrue about neuroplasticity is
that it’s always good, but it’s not always good,” Harvey said.

Spasticity itself is not typically painful, though it often foretells pain syndromes — nearly
three-quarters of survivors with spasticity developed pain.
And once CPS shows up, in all likelihood it will continue to be there and will require medical
management, which is typically medications. “Of all the medications we have available to
treat CPS, only about half the patients treated with medications will have any significant pain
reduction,” Harvey said. “Of those patients who have significant pain reduction, that pain
reduction at best will be only 50 percent. Once CPS starts, it can be very, very difficult to
treat.”

Pain and Depression

“There is a relationship between pain and depressed mood,” Harvey said. “People who have
CPS are more likely to have depressed mood, and some antidepressants have been shown to
help reduce pain. Some say you’re treating the depression which helps reduce the effect of
the pain; others say that you’re reducing the pain and therefore, they’re not as depressed.”

Depression may affect how a person perceives the severity of their pain. “Depression can
certainly lead to a catastrophizing of the pain,” he said. “‘Oh my God, this is the worst thing
in the world. I can’t do anything. I might as well just die.’ A person’s personality, their mood,
all of that can have an impact on how they perceive pain,” Harvey said.
Treating depression is generally a part of pain management.

Patti Gilstrap shares her story and experience as a CPS survivor.

A helpful resource for survivors with CPS is the Central Pain Syndrome Foundation.
Advice for Caregivers
Chronic pain can be stressful for the caregiver as well as the survivor. It reduces activity and
engagement in life and with others and may lead to isolation, which tends to increase
caregiver burden. “It’s emotionally stressful to see your loved one in pain,” Harvey said.

Early on when the survivor is starting to have pain and the pain is becoming significant, the
caregiver should advocate, be supportive and do what the doctor says. Encourage the patient
that there’s hope for treatment. “If it becomes a chronic pain problem, then the pain becomes
a lifestyle and the caregiver needs to learn how not to reinforce pain behavior,” Harvey said.

The caregiver must be watchful to not be manipulated: “I’m having pain so I need my pills.
Give them to me.” Harvey says caregivers must not respond to those kinds of things. “You
don’t reward pain behavior,” he said. “You reward behavior where the person is not letting
pain control their life. But that’s a process most people have to learn, perhaps through a
comprehensive pain management program.”

Complex Regional Pain Syndrome

Complex regional pain syndrome (CRPS) is a phenomenon that happens not only in stroke
but can also happen in peripheral nerve damage. Its symptoms are extreme neuropathic pain,
swelling or inflammation of the joints and skin, loss of range of motion, eventual atrophy of
the muscle and loss of the hair in the areas involved. “In stroke patients, it has a tendency to
affect the shoulder and the hand,” Harvey said. “In fact, CPRS for stroke has also been called
shoulder-hand syndrome because it seems to affect the shoulder and the hand more often than
other regions of the body.”

At this time, its cause is unknown, but the treatment is no mystery — early mobilization of
the affected limb. “In reality, we don’t see it that often in modern rehabilitation care because
we tend to mobilize patients fairly early even in acute hospitals and in doing so, it seems to
prevent it,” Harvey said.

Patients who do get CRPS are those who tend to be immobilized for an extended period,
perhaps because they’re comatose or they’re on a ventilator for a long time.
Getting survivors to put weight on the affected limb can be difficult. Harvey says he
sometimes helps the process by giving high-dose steroids to help reduce the inflammation
and allow the survivor to be mobilized and then as they get mobilized, the CRPS begins to
resolve over time.

“The key thing is when you discover it, to hit it hard and early. We want to prevent patients
from losing range of motion because as the tissue swells up with all the inflammation,
affected patients will begin to lose range of motion in their joints. If you don’t treat that early
on, eventually the CRPS sort of burns out and you have this atrophic limb with loss of range
of motion. At that point, it’s almost impossible to ever get that range of motion back. But you
don’t let that happen. You prevent it from the get-go. Prevention is the treatment,” Harvey
said.

Comprehensive Pain Management Centers

Today there are comprehensive pain management centers that teach emotional coping skills
for both survivors and caregivers as well as meditation and mindfulness techniques. “They
show them non-pharmacological ways of managing pain like relaxation techniques, massage
and good sleep hygiene and reduction of certain stimulants like caffeine, all these things.

“They also teach them how to exercise without fear that they’re going to injure themselves,”
he said. People in pain often do less, and because of that, they get deconditioned and lose
strength and endurance.

“One of the methods in comprehensive pain management programs is to show patients how
to exercise without making the pain worse. As they become more physically fit, the pain
actually reduces a little bit over time, or at least if it’s not reduced, it’s not as bothersome.”

Spasticity-Related Pain
Spasticity, an abnormal activation pattern of muscles, occurs to some degree within a week in
about a quarter of survivors. While it may look painful, spasticity itself is not typically
painful, though it often foretells pain syndromes — nearly three-quarters of survivors with
spasticity developed pain.

Spasticity may cause nociceptive pain because of injury to tissue. “Depending on how severe
their spasticity is and where it’s located, in some people, it may cause stress on the joints and
tendons,” said Harvey. Although the mechanism is not clearly understood, spasticity may
cause inflammation of those joints and tendons, which is painful. “The treatment is to treat
the spasticity with medications and Botox® injections. We also use medications like
Tylenol® or anti-inflammatory drugs as appropriate and that generally will take care of the
spasticity-associated pain. That one is pretty straightforward: Treat the spasticity.”

Non-pharmacological treatment for spasticity is stretching, maintaining good range of

motion, staying active and moving a lot. “Some would recommend splinting but it’s not yet
clear whether splinting really helps prevent spasticity,” Harvey said.

Shoulder Subluxation
The shoulder is the most complex joint in our bodies, perhaps because it has the greatest
range of motion. It is a ball-and-socket joint like the hip, but the socket is shallow and the ball
at the end of the arm is held in place by rotator cuff muscles. “When a survivor has paralysis
of the muscles including the rotator cuff, they will tend to have instability of the shoulder,”
Harvey said. The shoulder pain usually develops as the survivor starts getting muscle tone
back, perhaps because of poor mechanics around the shoulder or because of spasticity, which
also leads to poor mechanics around the shoulder that can lead to shoulder pain. “But that
shoulder pain is due to inflammation of the joints, or the biceps and tendons — nociceptive
pain, not neuropathic,” Harvey said.

But this is not a subluxation, where the ball and socket dislocate. “The only way that
subluxation can cause shoulder pain is if you don’t properly support the arm early on and the
rotator cuff gets torn because you just let the arm hang and the tissue gets torn,” Harvey said.
That, of course, is a tissue injury (nociceptive pain) and inflammation, but the subluxation
itself is not the cause of pain.

“People who have CPS are more likely to have depressed mood, and some antidepressants
have been shown to help reduce pain.”
Another cause of shoulder pain is when hypertonia (abnormally intense muscle tension that
makes it more difficult for a muscle to stretch) develops as a result of some abnormal pulse
around the shoulder that can cause a lot of inflammation and pain. If you do not treat this
tissue inflammation and pain early on, “the patient can develop a chronic inflammatory
problem around the shoulder and all chronic inflammatory problems end up becoming central
pain if not properly managed,” Harvey said. “If I let an inflamed shoulder go and it remains
inflamed over three to six months, it’s going to become a chronic neuropathic pain problem.
So, the treatment of hemiplegic shoulder pain is to nip it in the bud.”

This may involve x-rays and ultrasounds to determine the source of the pain — a fracture,
tissue damage or inflammation. Harvey treats inflammation with a steroid injection. “After
the injection, the patient can then work in therapy, develop proper mechanics and get their
range of motion back and often the pain will suppress and go away,” he said.
In those cases where it doesn’t go away and doesn’t respond to anti-inflammatory
medications, the hemiplegic shoulder pain may become chronic neuropathic pain, localized in
the shoulder. “At that point, I tend to use the usual medications that we use for central pain to
see if that helps, and again, the success rate is about 50 percent,” Harvey said.

For those who don’t respond, there is a new non-drug treatment for shoulder pain —
intramuscular nerve stimulators. With these devices, a single wire electrode is inserted in the
deltoid muscle, and then the muscle is stimulated at a low level for about six hours a day for
30 days. “You don’t stimulate the skin so the patient doesn’t feel much,” Harvey said. “They
may feel something but it’s not like you get this big old stimulator on your skin. It’s in the
muscle, and we believe it has a neuromodulation effect and sort of resets the central
hypersensitivity back towards normal so that the pain is reduced, at least during treatment.
And for some, there seems to be pain reduction for some time after the four-week treatment.”