Lisa Deck lay on her basement couch with the lights and music off, but still, the room seemed too bright and the sounds seemed too loud. The left side of her body felt numb, and the rest of it — especially her head — throbbed. “What in the world is happening?” thought Deck, who was a 21-year-old college senior in the District of Columbia at the time.
At the hospital, doctors called it a stress-related migraine and sent her home to rest. A few days later, other doctors called it a stroke. “I was shocked,” says Deck, who had assumed she was too young and healthy to have a stroke. When another one struck six months later, doctors diagnosed her with a serious brain disease. “I had a very hard time in my 20s,” says Deck, who underwent chemotherapy and other intensive treatments until being declared in remission 2001.
But after many stroke-free years of moving on with her life — including getting married and having kids — Deck had another stroke. This time, a neurointerventional radiologist told her she had likely been suffering from moyamoya disease all along and would need brain surgery to treat it.
“I remember thinking about where are my kids, and how are they getting home from school?” Deck says. “At 21, it was all about me, and at 39, it was not about me.”
[See: 10 Seemingly Innocent Symptoms You Shouldn’t Ignore.]
Moyamoya disease is a rare condition meaning “puff of smoke” in Japanese to describe the cloud of narrowed blood vessels at the base of the brain that make patients vulnerable to strokes, transient ischemic attacks (aka “ministrokes”) and other ischemic events. “I had heard the word [moyamoya] before,” Deck says, “but I didn’t know what it meant.”
Unlike more common and well-publicized precursors of stroke, moyamoya — which is estimated to affect less than 1 in 100,000 people in the U.S. — seems to have little to do with heart health and more to do with genetics and unfortunate brain anatomy. “It is not associated with high blood pressure, it’s not associated with cholesterol — it has nothing to do with conventional vascular risk factors with atherosclerosis,” says Dr. Jose Biller, chair of the department of neurology at the Loyola University Chicago Stritch School of Medicine and a fellow of the American Academy of Neurology.
Rather, it’s associated with having East Asian ancestry, as well as a host of medical conditions including sickle cell anemia, Grave’s disease and Down syndrome. “There’s probably a dozen or so different conditions that all end up acting like moyamoya,” says Dr. Edward Smith, director of pediatric cerebrovascular neurosurgery at Boston Children’s Hospital who specializes in moyamoya disease.
The disease, which tends to strike either in childhood or in people’s 30s or 40s, can be understood like a traffic jam, Smith explains. Just imagine that the carotid artery is a highway that forks into two roads: one that delivers blood to the brain and one that delivers blood to the rest of the head outside of the brain. In moyamoya, the road to the brain is jammed at the on ramp. “Once you get past the accident, the traffic is fine,” Smith says. “The downstream blood vessels are normal, they just don’t have any good blood getting into them.”
Left untreated or misdiagnosed, “these roads get narrower and narrower, and blood flow to the brain is reduced,” leading to a 66 to 90 percent risk of severe or fatal stroke over five years, Smith says. Events that lower blood pressure — such as dehydration from childhood ailments like the flu, vomiting or diarrhea — can increase the risk of stroke by potentially reducing blood flow to the brain.
“Crying or blowing an instrument like a harmonica or … eating hot noodles or a child celebrating his or her birthday and blowing a candle” can all affect blood pressure, Biller says.
Fortunately, treatments for moyamoya are increasingly effective, and many patients — especially those who are treated young and at a high-volume medical center — go on to live largely normal, happy and productive lives. “We don’t make the disease go away, but if you have it, the good news is there are treatments,” Smith says.
Namely, surgery. In the procedure invented by Smith’s mentor and colleague, Dr. R. Michael Scott, for example, surgeons redirect blood flow to the brain by building a blood vessel bridge with a donor artery. “We can’t reopen the damaged part of the road,” Smith says, “[but] we can build those bridges with surgery.” The procedure, he says, makes stroke about 20 times less likely.
His team’s research has found that even without surgery, up to 50 percent of kids’ brains will attempt to reroute traffic themselves by growing blood vessels through natural cracks in their skulls, or those caused by earlier surgeries. “The blood vessels will grow throughout those cracks like grass through the sidewalk,” Smith says. In those cases, surgery is especially effective because surgeons have a better road map of where to direct traffic without disrupting the brain’s natural rebuilding efforts.
“[We’ve made] modifications over the years to make it faster, safer, and to understand the biology better,” says Smith, who has treated kids who are now doctors, nurses, lawyers and parents. “Intellectually, they do better after surgery because of increased blood flow,” Smith says. “They live full, healthy lives.”
Screening efforts and awareness of the disease are improving, too. The American Heart Association, for instance, has recommendations about screening kids for strokes, and radiologists are getting better at identifying moyamoya in kids who undergo brain scans for unrelated problems like concussions. Such early intervention is key since how well people fare doesn’t depend on whether they have moyamoya as much as it depends on whether they’ve suffered strokes before being treated, Smith says. “We’re slowly getting smarter at who to screen with MRIs,” Smith says.
[See: 10 Lessons From Empowered Patients.]
That’s good news for Deck, who’s become an advocate for moyamoya, stroke and heart disease. “I want people to know about [moyamoya] because people don’t think stroke can happen to young people, and people don’t know what moyamoya is,” she says.
Had she been treated before suffering four strokes, she may not have had to deal with the level of fatigue and other side effects she has now. Still, Deck, who underwent successful double brain bypass surgeries at Stanford in 2015, considers herself lucky; other folks with moyamoya can have debilitating lifelong physical and cognitive disabilities due to the brain damage from stroke. “I’m a believer that things happen for a reason,” she says. “My life is more fulfilling now had those things not happened to me.”
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What Is Moyamoya Disease? originally appeared on usnews.com
