(Health Korea News / Jang Dong-gyu) Moyamoya disease is A disease in which the terminal portion of the arterial blood vessel in the brain that supplies blood to the brain gradually narrows and eventually becomes blocked for no particular reason.am. When this happens, cerebral blood flow becomes insufficient, causing ischemic symptoms, or hemorrhagic stroke occurs due to the rupture of blood vessels that formed to compensate for the insufficient blood flow.
The disease name, Moyamoya disease, was given because the abnormal micro-blood vessels formed to supply insufficient blood flow as normal blood vessels narrow, resembling the ‘shape of cigarette smoke rising.’ In 1969, Japanese professor Suzuki named it after the Japanese word ‘moyamoya’, which means ‘everything’.
Moyamoya disease is relatively common in Korea and Japan, but rare in the West. A characteristic that occurs relatively frequently in children around the age of 10 and adults in their 40s and 50sIt looks like
◇Main cause of pediatric stroke… 10-15% of patients have a family history
The number of patients with moyamoya disease is rapidly increasing. According to data from the Health Insurance Review and Assessment Service, the number of patients increased from 11,860 in 2018 to 17,459 last year (2023), an increase of 47.2% over the past five years. It is about twice as common in women. Moyamoya disease is considered a major cause of stroke in children, and the frequency of cerebral hemorrhage appears to be increasing in adults.
Symptoms include △headache △paralysis △decrease in sensory function △speech impairment △visual impairment △convulsions △decrease in consciousness △decrease in cognitive function It appears in various ways. In children, it is suspected if they exhale heavily when eating hot soup or food or show neurological abnormalities. This may be a symptom caused by reduced cerebral blood flow due to hyperventilation. In addition, symptoms may appear due to changes in cerebral blood flow when dehydration occurs after excessive sweating, such as during sudden strenuous exercise or exposure to rapid temperature changes such as in the heat or sauna.
Moyamoya disease in children progresses relatively quickly, while moyamoya disease in adults progresses slowly, and research has shown that this is closely related to genetic predisposition. Most patients with moyamoya disease symptoms have a higher risk of stroke and a higher stroke recurrence rate than the general population. In particular, in adults, approximately 34% of cases manifest as cerebral hemorrhage and 50% manifest as cerebral ischemia symptoms.
Recent genome research has discovered several suspicious genes, but the exact cause of moyamoya disease has not yet been revealed. 10-15% of patients have a family history, especially More often than not, there is a family history on the mother’s side.. However, it is presumed that several different genes, rather than one gene, act in combination and are expressed while influenced by environmental factors.
◇Risk of stroke and cerebral ischemia if left unattended… Treatment with cerebrovascular bypass surgery
A detailed examination is necessary to confirm the diagnosis of moyamoya disease and establish a treatment plan. Moyamoya disease is diagnosed using brain magnetic resonance angiography (Brain MRA), brain computed tomography angiography (Brain CTA), or catheter cerebral angiography. The diagnosis is made through the characteristic finding that moyamoya blood vessels grow due to gradual occlusion of the distal ends of the internal carotid arteries or major branch arteries on both sides of the skull, and the degree of hemodynamic stress is evaluated through cerebral hemodynamic examination (SPECT) and treated accordingly. Set a policy.
Treatment is divided into medical treatment and surgical treatment.Do it. In the case of ischemic moyamoya disease, antiplatelet agents (antithrombotic agents) are prescribed to prevent cerebral infarction in the acute phase. Recently these Antiplatelet medication prescription can reduce mortality in patients with moyamoya disease.A domestic study was also reported. However, there are differing opinions on the use of antithrombotic drugs in cases of cerebral hemorrhage, and there are still few research results showing that cerebral hemorrhage can be prevented with drug treatment.
A representative surgical treatment is ‘vascular bypass surgery’, which is a surgery that directly or indirectly connects blood vessels outside the brain to cerebral blood vessels to relieve hemodynamic instability caused by occlusion or stenosis of intracranial cerebral blood vessels. Embolization treatment is performed for intracranial aneurysms or pseudoaneurysms that occur due to hemodynamic instability. Results of a clinical trial of treatment with vascular bypass surgery and non-surgical treatment in patients with hemorrhagic moyamoya disease published in Japan in 2014, Vascular bypass surgery has the potential to reduce mortality and recurrence of cerebral hemorrhage.There was a report that there was this. A paper published by the author and other domestic researchers at the end of last year also showed that cerebrovascular bypass surgery reduces the incidence of cerebral hemorrhage and reduces mortality.
In children, vascular bypass surgery, which is a surgical treatment, is mostly performed, while in adults, surgical treatment along with drug treatment is often performed for ischemic moyamoya disease, and surgical treatment for hemorrhagic moyamoya disease has recently been on the rise. In the past, asymptomatic cases were often observed without treatment, but in cases where blood flow is low or the risk of cerebral hemorrhage is high, surgery may be considered preventively.
Moyamoya disease has a much higher incidence of stroke than the general population. A scary disease with a very high mortality rate when cerebral hemorrhage occurs.am. If you have a family history of moyamoya disease or are diagnosed with it, active preventive management is necessary even if you are asymptomatic. Early preventive screening is especially important if you have a family history. (Written by / Professor Jang Dong-gyu, Neurosurgery, Brain Hospital, Incheon St. Mary’s Hospital, Catholic University)
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