Like many young adults her age, Savannah Cawood is looking forward to starting the rest of her life. She’s going to college, participating in an internship this summer, and planning to pursue a graduate degree in health policy so she can research methods of care delivery and help create more effective and equitable laws around healthcare.
Unlike most young adults her age, however, Savannah, 21, must navigate a rare, progressive disorder called moyamoya, which means “puff of smoke” in Japanese.
In patients with moyamoya, the vessels that supply blood to the brain (called carotid arteries) narrow, limiting the flow of blood and increasing the risk for stroke. To compensate for this reduced blood flow, the brain grows new blood vessels, which can temporarily help increase blood supply but eventually stop working. The “puff of smoke” name comes from how these new blood vessels appear on imaging tests: as tangled and smoke-like.
Symptoms That Didn’t Add Up
Savannah wasn’t diagnosed with moyamoya until she was 18 years old, but she received the first warning sign that something might be wrong at a young age.
Born in California, shortly after her family immigrated to the United States from South Africa, Savannah developed bacterial meningitis when she was only a few months old. She recovered from the infection and, when she was 2, Savannah and her family moved to Cincinnati.
During her freshman year of high school, Savannah began noticing what she called random symptoms. “I’d lose feeling in my hand,” she recalled, “or the side of my face would go numb so I couldn’t really talk.” Eventually, her pediatrician referred her to a rheumatologist at Cincinnati Children’s, who diagnosed Savannah with juvenile fibromyalgia—a chronic condition that causes pain in the muscles and soft tissues of the body—and enrolled her in a patient study.
Oddly, though, Savannah was also complaining of headaches, which wasn’t consistent with a fibromyalgia diagnosis. When her symptoms began interfering with her participation in the study, Savannah’s rheumatologist referred her to Cincinnati Children’s Cerebrovascular Disease and Stroke Center.
Finally Putting the Pieces into Place
In 2020, Savannah began seeing J. Michael Taylor, MD, a pediatric stroke and neurocritical care specialist in our Division of Neurology. An MRI for evaluation of Savannah's headaches revealed a prior brain injury and an abnormality of a brain artery. Changes like this can be seen in patients with a history of bacterial meningitis, said Dr. Taylor. Savannah's symptoms over the years suggested that she was having periodic problems of inadequate blood flow to her brain, commonplace in patients with moyamoya.
With moyamoya as the primary suspect, Dr. Taylor recommended that Savannah undergo further testing with a cerebral angiogram, which showed the appearance of moyamoya. With the moyamoya diagnosis in hand and the evidence of a past stroke, Dr. Taylor was confident they’d finally uncovered the culprit of Savannah’s symptoms.
“We’re often able to pinpoint a suspicious time in a patient’s past that we can draw the line back to and say, ‘I bet that’s where this started,’” Dr. Taylor said. “For Savannah, all signs pointed to her having had a stroke when she was a baby with bacterial meningitis.”