Head 'Cigarette smoke shape'Vascular 'Moya molya disease' If left unattended, there's a possibility of a stroke
Dec 10, 2024
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The name Moyamoya disease was given because the abnormal microvessels created to supply insufficient blood flow as normal blood vessels narrowed are similar to 'the shape of cigarette smoke rising up'. In 1969, Professor Suzuki of Japan named it after the Japanese word 'Moyamoya(もやもや)' which means 'Moyamoya'.
Professor Jang Dong-kyu of Neurosurgery at the Catholic University of Korea's Incheon St. Mary's Hospital said, `Moya moya disease is relatively common in Korea and Japan and rare in the West, especially in children around the age of 10 and adults in their 40s and 50s.'
◇The main cause of childhood stroke... 10-15% of patients have a family history
The number of patients with moya moya disease is increasing rapidly. According to data from the Health Insurance Review and Assessment Service, the number of patients with moya moya disease in Korea increased from 11,860 in 2018 to 17,459 last year (2023), an increase of 47.2% over five years. It is about twice as many in women. Moyamoya disease is considered a major cause of childhood stroke, and the frequency of cerebral hemorrhage increases in adults.
Symptoms include △ headache △ symptoms of paralysis △ decreased sensory function △ speech impairment △ visual impairment △ cramps △ decreased cognitive function. In the case of children, when eating hot soup or food, they suspect if they breathe and rest or show neurological abnormalities. It may be a symptom caused by decreased cerebral blood flow due to hyperventilation. In addition, even when you are dehydrated after sweating a lot, such as suddenly exercising heavily or being exposed to sudden changes in temperature such as heat or sauna, your brain blood flow may change and symptoms may appear.
Pediatric moyamoya disease progresses relatively quickly, and adult moyamoya disease progresses slowly, which is very close to genetic predisposition. Most patients with symptoms of moya moya disease have a higher risk of stroke and a higher recurrence rate of stroke than the general population. In particular, about 34% of adults have cerebral hemorrhage and 50% have cerebral ischemia symptoms.
Recent genomic studies have found several suspected genes, but the exact cause of Moyamoya's disease has not yet been identified. Professor Jang Dong-gyu said, `10-15% of patients have a family history, especially toward mothers.' `It is estimated that several different genes, not just one gene, work in combination and are expressed as environmental factors influence.'
◇ Risk of stroke and cerebral ischemia if left unattended... treatment by surgery with cerebrovascular bypass
In order to establish a confirmation and treatment plan for moya moya disease, a thorough examination is required. Moyamoya's disease is diagnosed by brain magnetic resonance angiography (Brain MRA), brain computed tomography (Brain CTA), and catheter cerebral angiography. The diagnosis is made through the characteristic findings of the growth of moyamoya blood vessels due to the gradual closure of both internal arteries in the cranial or major branch arteries, and the hemodynamic stress level is evaluated through a cerebral hemodynamic test (SPECT), and the treatment policy is determined accordingly.
Treatment is divided into medical treatment and surgical treatment. In the case of ischemic moyamoya disease, antiplatelet drugs (anti-thrombotic drugs) are prescribed to prevent cerebral infarction in the acute phase. Recently, a domestic study reported that the prescription of such antiplatelet drugs can reduce mortality in patients with moyamoya disease.
Professor Jang Dong-gyu said, "However, if there is a cerebral hemorrhage, there is a disagreement on the use of antithrombotic drugs, and there are few research results yet that drug treatment can prevent cerebral hemorrhage."
In surgical treatment, 'Vascular bypass surgery', which is an operation that directly or indirectly connects blood vessels in the outside of the brain to relieve hemodynamic instability caused by obstruction or stenosis of intracranial cerebrovascular vessels. In addition, embolization treatment is performed for intracranial aneurysms or pseudoaneurysms caused by hemodynamic instability.
Clinical trials of vascular bypass surgery and non-surgical treatment in patients with hemorrhagic moyamoya disease published in Japan in 2014 have shown that vascular bypass surgery has the potential to reduce mortality and recurrence of cerebral hemorrhage. In addition, a paper published by Korean researchers, including Professor Jang Dong-gyu, showed that surgery by cerebrovascular bypass reduces the incidence of cerebral hemorrhage and reduces the mortality rate.
While most children are operated on vascular bypass, which is a surgical treatment, adults are treated with many surgical treatments along with medication in the case of ischemic moyamoya disease, and hemorrhagic moyamoya disease is on the rise recently. In the case of asymptomatic cases, follow-up observation was often performed without treatment in the past, but surgery is also considered prophylactically if there is a decrease in blood flow or a high risk of cerebral hemorrhage.
Professor Jang Dong-gyu said, "Moya moya disease is a scary disease in which the frequency of stroke is much higher than that of the general population, and the mortality rate is very high in the event of a cerebral hemorrhage." "If you have a family history of moya moya disease or are diagnosed, you need active preventive management even if you are asymptomatic, and preventive early examination is more important than anything else, especially if you have a family history."
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This article was translated by Naver AI translator.
