Epidemiological Features of Moyamoya Disease in Japan

Hoshino, Haruhiko; Izawa, Yoshikane; Suzuki, Norihiro

doi:10.2176/nmc.52.295

Cited by 69 publications

(48 citation statements)

References 8 publications

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“…The majority of pediatric patients present with ischemic attacks, whereas adults present with both hemorrhagic and ischemic events at almost equal proportion. 1,2,20 The present study showed that both children and adult patients have more ischemic strokes than hemorrhagic events, although the ratio of hemorrhagic stroke to ischemic stroke was greater in adults. We also found that children were more likely to have comorbid epilepsy.…”

Section: May 2014mentioning

confidence: 47%

Epidemiology of Moyamoya Disease in Taiwan

Chen

Yang

Chien

et al. 2014

Stroke

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Background and Purpose— Previous studies have shown regional and temporal variations in epidemiological features of moyamoya disease, but population-based studies in regions other than Japan are limited. We investigated the incidence and patients characteristics of moyamoya disease during 12 years in Taiwan using claims databases of a universal health insurance system. Methods— From the inpatient databases of the Taiwan National Health Insurance program, we identified subjects who had an initial hospitalization with moyamoya disease and had been underwent cerebral angiography as incidence cases during 2000 to 2011. The incidence and the patient characteristics were described by age and time periods of the hospitalization. Results— During the 12-year period, 422 patients were identified, representing an annual incidence of 0.15 per 100 000 person-years. Adults exhibited an upward trend in incidence with an incidence rate ratio of 1.74 (95% confidence interval [1.17–2.58]) in years 2010 to 2011 comparing with years 2000 to 2001. However, children had a decreased incidence except a slightly increase in the last 2 years. Compared with patients hospitalized during 2000 to 2005, patients identified during 2006 to 2011 had greater women-to-men ratio (1.7 versus 1.1, P =0.048). Children were more likely to have comorbid epilepsy than were adult patients (25.0% versus 3.4%, P =0.002). Hemorrhagic stroke was rare among pediatric patients but presented more frequently in adults. However, ischemic stroke was more prevalent in both groups. Conclusions— The incidence of moyamoya disease has increased in adults but not in children from 2000 to 2011 in Taiwan. Sex ratio and comorbid conditions differed by age and study period.

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Section: May 2014mentioning

confidence: 47%

Epidemiology of Moyamoya Disease in Taiwan

Chen

Yang

Chien

et al. 2014

Stroke

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“…However, previous studies in Japan demonstrated a ratio of approximately 2:1 [25,26]. This dissimilar sex ratio of MMD in different populations may be a reflection of genetic discrepancies.…”

Section: Discussionmentioning

confidence: 90%

Asymptomatic Cerebral Microbleeds in Adult Patients with Moyamoya Disease: A Prospective Cohort Study with 2 Years of Follow-Up

et al. 2013

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Objective: Risk factors for cerebral microbleeds (CMBs) in moyamoya disease (MMD) remain unknown, and whether the presence or distribution of CMBs is related to the subsequent hemorrhagic events needs to be confirmed. This study aimed to investigate the relationship between intracranial vascular morphology and the incidence/distribution patterns of CMBs as well as to determine the outcome in adult patients with MMD. Methods: Eighty-five consecutive adult patients with MMD were prospectively evaluated by 3.0-tesla high-intensity susceptibility-weighted magnetic resonance imaging and catheter-based digital subtraction angiography from June 2009 to January 2012. The differences in intracranial angiography (Suzuki stages, posterior cerebral artery stages, and angiographic features of the anterior choroidal and posterior communicating arteries) and clinical parameters (age, gender, hemorrhagic onset, antiplatelet treatment, systolic/diastolic blood pressure, diabetes, and plasma fibrinogen) among various CMB distribution patterns were analyzed with multinomial logistic regression. Moreover, after a median follow-up of 23 months, the relationship between CMB distribution patterns and subsequent intraventricular hemorrhage was also analyzed with Kaplan-Meier survival analysis and a Cox proportional hazards model. Results: Forty-five female and 40 male patients were finally enrolled in the study with an average age of 41.4 ± 12.7 years. Thirty-one CMB foci were detected in 24 (28.2%) patients. Most of the lesions (n = 22, 71.0%) were located in the region of deep and periventricular white matter (DPWM). Dilation and extension of anterior choroidal and posterior communicating arteries (AChA-PComA) was found to be an independent risk factor associated with the CMBs located in DPWM (p = 0.045; odds ratio 3.39, 95% CI 1.03-11.19). Patients with DPWM CMBs showed a statistically higher likelihood of subsequent intraventricular hemorrhage compared to patients without CMB foci and with CMBs in other regions (p = 0.014) during the follow-up period. Multivariable analysis suggested that DPWM CMBs were independent predictors of subsequent intraventricular hemorrhage (p = 0.028; hazard ratio 5.53, 95% CI 1.20-25.41). Conclusions: Dilation and extension of AChA-PComA is a determinant of CMBs involved in DPWM. CMBs in DPWM are probably predictors of subsequent intraventricular hemorrhage.

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“…This importance is highlighted in the 10-15% of moyamoya disease cases that are familial. 10 The proposed mechanism of inheritance is autosomal dominant with incomplete penetrance but it is likely there are polygenic interactions as well. 11 Currently, screening is not recommended for moyamoya disease.…”

Section: Etiologymentioning

confidence: 99%