Moyamoya Disease: Minimizing Risk, Maximizing Recovery
Introduction
In recognition of World Brain Day on July 22, the University of Illinois Chicago (UIC), part of UI Health, are bringing much-needed attention to their experience in treating one of the most difficult and underdiagnosed diseases of the brain: Moyamoya Disease.
For decades, the multidisciplinary team has been at the forefront of treating Moyamoya with advanced imaging, surgical revascularization, and ongoing neurological care. With expert knowledge of both the disease and its related disorders, this institute is a lifeline for patients and families in need of answers and support.
What is Moyamoya Disease?
With less oxygenated blood traveling, the brain is more prone to strokes. “Patients are over 60% more likely to have a stroke,” said Dr. Fady T. Charbel, an internationally recognized surgical expert and head of the Department of Neurosurgery at UIC, who has developed special surgical techniques for bypass surgery in Moyamoya.
In response, the brain activates the growth of new vessels to try to recirculate blood, but these vessels are poorly designed and cannot withstand the pressure of normal blood flow.
These delicate vessels can burst and cause intracerebral hemorrhage. These hemorrhages are usually present in deep brain structures such as the basal ganglia, contributing to their neurological impact.
The basal ganglia are a set of structures that primarily function in the transition from learning new movements and emotional and cognitive processes into long-term habits.
Since the Moyamoya causes internal carotid arteries to narrow, the entire brain hemisphere—including the basal ganglia—is at risk of stroke due to inadequate blood supply.
Unknown Causes
The exact causes of Moyamoya disease remain unknown, making it an especially challenging condition for patients and physicians to prevent. What is known is that the condition most typically affects children between the ages of 5 and 10, but young adults may also acquire the condition, typically with different patterns of symptoms.
Because the condition is rare and complex, coordinated care and early diagnosis are crucial—but not always accessible.
MRI/MRA
“Here, we have a specialized center to treat Moyamoya,” said Dr. Charbel. “We have expertise in evaluating patients, including special MRIs that create detailed images of the brain to determine how much blood is coming to the brain and whether that blood flow is sufficient.”
Functional Magnetic Resonance Imaging (fMRI) is an essential research and patient care tool, particularly in the field of neuroscience. In patients who have Moyamoya disease, fMRI offers a non-invasive imaging method for mapping critical brain function relating to how memory, movement, and speech are affected.
Additionally, the neurosurgery team utilizes a non-invasive Magnetic Resonance Angiography (MRA), called NOVA, to view quantitative blood flow measurements in the brain in real-time.
These clear imaging capabilities enable neurosurgeons to more precisely plan revascularization surgery, decreasing the possibility of damage to delicate areas and facilitating the preservation of long-term motor and cognitive function.
Bypass Procedure
UI Health is also one of the few academic medical centers in the Midwest that specializes in direct and indirect cerebral bypass procedures, offering advanced, targeted treatment to restore blood flow and reduce the risk of stroke in both children and adults.
“If there is a blockage in the arteries, we have to create alternative routes for the blood to flow to the brain,” said Dr. Charbel. “So, the blood will come through an artery that the surgeon will make via a small hole in the skull and attach it to the surface of the brain. By doing this, blood supply is brought into the brain.”
Over time, this newly re-routed blood flow helps reduce the risk of stroke and improves overall brain health, offering patients a safer and more stable long-term outcome.
A Valuable Refuge
Effective care for Moyamoya is just one reason why UIC has become a valuable refuge for families seeking treatment for this condition. One of the few centers in the region with dedicated expertise in Moyamoya, the team continues to deliver comprehensive care for patients with Moyamoya, with all the disciplines of neurosurgeons, neurologists, radiologists, and specialty nursing staff working together to maintain brain function at every stage.
“We’re proud of our role as one of the largest centers to treat Moyamoya disease,” said Dr. Charbel. “We have over 30 years of experience researching and treating this condition, some of the most experienced neurosurgeons in the world, and amazing success rates for bypass surgery.”
Visit UI Health’s Neurosurgery Stroke Program to learn more or call 312.996.3700 to request an appointment.