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      Moyamoya Disease: Epidemiology, Clinical Features, and Diagnosis

      review-article
      Journal of Stroke
      Korean Stroke Society
      Moyamoya disease, Epidemiology, Clinical features, Diagnosis

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          Abstract

          Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology remains unknown, recent genetic studies identified RNF213 in the 17q25-ter region as an important susceptibility gene of MMD among East Asian populations. Possibly because of genetic differences, MMD is relatively common in people living in East Asian countries such as Korea and Japan, compared to those in the Western Hemisphere. The prevalence of MMD appears to be slightly lower among Chinese, compared to Koreans or Japanese. There are two peaks of incidence with different clinical presentations, at around 10 years and 30-40 years. The peak appears to occur later in women than men. In children, ischemic symptoms, especially transient ischemic attacks, are predominant. Intellectual decline, seizures, and involuntary movements are also more common in this age group. In contrast, adult patients present with intracranial hemorrhage more often than pediatric patients. In patients with MMD, intracerebral hemorrhage is more often accompanied by intraventricular hemorrhage than in patients with hypertensive intracerebral hemorrhage. These different age peaks and different clinical presentations in each age group are also observed in MMD patients in the USA. Catheter angiography is the diagnostic method of choice. Magnetic resonance (MR) angiography and computed tomographic angiography are noninvasive diagnostic methods. High-resolution vessel wall MR imaging also helps diagnose MMD by revealing concentric vessel wall narrowing with basal collaterals.

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          Moyamoya disease: current concepts and future perspectives.

          Moyamoya disease is an uncommon cerebrovascular disease that is characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. The disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed moyamoya vessels. The incidence of moyamoya disease is high in east Asia, and familial forms account for about 15% of patients with this disease. Moyamoya disease has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years. Most paediatric patients have ischaemic attacks, whereas adult patients can have ischaemic attacks, intracranial bleeding, or both. Extracranial-intracranial arterial bypass, including anastomosis of the superficial temporal artery to the middle cerebral artery and indirect bypass, can help prevent further ischaemic attacks, although the beneficial effect on haemorrhagic stroke is still not clear. In this Review, we summarise the epidemiology, aetiology, clinical features, diagnosis, surgical treatment, and outcomes of moyamoya disease. Recent updates and future perspectives for moyamoya disease will also be discussed.
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            Novel epidemiological features of moyamoya disease.

            Many clinical features that are specific to moyamoya disease have been reported and cited in textbooks based on previous data. The purpose of this study is to investigate the present epidemiological features of moyamoya disease based on recently obtained regional all-inclusive data. The authors performed an all-inclusive survey of moyamoya disease in Hokkaido, one of the major islands in Japan that has a population of 5.63 million. The epidemiological features were analysed based on the data from 267 newly registered patients with moyamoya disease in Hokkaido from 2002 to 2006. These analysed data were adjusted to the whole Japanese population at 2005. The detection rate of the disease per year was 0.94 patients per 100,000 people, and prevalence was 10.5 patients per 100,000 people. The incidence of ischaemia concerned with the disease was 0.53 patients per 100,000 people-years and haemorrhage was 0.2 patients per 100,000 people-years. The ratio of female to male patients was 2.18. The ratio of patients aged 10 years and above to under 10 years of age at onset was 6.18. Two peaks for age of onset were seen: the highest was observed between 45 and 49 years, and the second between 5 and 9 years. Asymptomatic patients comprised 17.8% of the total number of patients. The epidemiological features of moyamoya disease determined by this survey varied considerably from previous data. The detection rate and prevalence of the disease were higher than those reported previously. The highest peak of onset age was older than those reported previously. In addition, it was revealed that asymptomatic moyamoya patients are not always rare in Japan.
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              Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey.

              The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease. The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey. In 2003, the total number of patients treated in Japan was estimated at 7700 (95% confidence interval, 6300 to 9300). Sex ratio (women to men) of the patients was 1.8. For men, the peak of moyamoya disease was observed in patients aged 10 to 14 years and for women aged 20 to 24 years. Annual rate of newly diagnosed cases in 2003 was 0.54 per 100,000 population. Family history of moyamoya disease was found in 12.1% of the patients. The majority (77.9%) were treated as outpatients. Although the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones, the estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade (3900 in 1994 and 7700 in 2003). The increase could partly be explained by the increase in newly diagnosed cases (0.35 in 1994 and 0.54 in 2003 per 100,000 population).
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                Author and article information

                Journal
                J Stroke
                J Stroke
                JOS
                Journal of Stroke
                Korean Stroke Society
                2287-6391
                2287-6405
                January 2016
                29 January 2016
                : 18
                : 1
                : 2-11
                Affiliations
                Stroke Center and Department of Neurology, Asan Medical Center, University of Ulsan, Seoul, Korea
                Author notes
                Correspondence: Jong S. Kim  Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea  Tel: +82-2-3010-3440 Fax: +82-2-474-4691 E-mail: jongskim@ 123456amc.seoul.kr
                Article
                jos-2015-01627
                10.5853/jos.2015.01627
                4747069
                26846755
                eca32110-ae5c-40e4-a96a-b36afac69869
                Copyright © 2016 Korean Stroke Society

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 November 2015
                : 24 December 2015
                : 28 December 2015
                Funding
                Funded by: Welfare and Family Affairs
                Award ID: HI14C1985
                Categories
                Special Review

                moyamoya disease,epidemiology,clinical features,diagnosis

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