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Long-term mortality in patients with moyamoya angiopathy according to stroke presentation type in South Korea

  • Original Article - Vascular Neurosurgery - Other
  • Published:
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Abstract

Background

Incidence, prevalence, and long-term survival outcomes in patients with moyamoya angiopathy (MMA) according to stroke presentation type and age group have not been clearly elucidated.

Methods

We investigated mortality in patients with MMA (moyamoya disease, probable moyamoya disease, moyamoya syndrome) of whose International Classification Disease 10 code was I67.5 from 2006 to 2015 using the Korean National Health Insurance database. MMA at diagnosis was classified into 3 types (ischemic, hemorrhagic, and asymptomatic or else) according to stroke presentation. Survival analysis was performed according to stroke presentation type and age group (< 15 years and ≥ 15 years) using the Kaplan–Meier method.

Results

There were 12,146 newly diagnosed moyamoya cases, with a female-to-male ratio of 1.81; the ischemic type was identified in 3671 (30.2%) patients, the hemorrhagic type in 2449 (20.2%) patients, and the asymptomatic or else type in 6026 (49.6%) patients. The mean age at diagnosis according to stroke presentation was 33.1 (± 14.8) years in asymptomatic or else type, 41.2 (± 17.3) years in ischemic type, and 45.4 (± 14.3) years in hemorrhagic type (P < 0.001). The 10-year survival rates in ischemic-, hemorrhagic-, and asymptomatic or else-type patients were 88.9%, 76.3%, and 94.3%, respectively (log-rank test; P < 0.001). Pediatric MMA (< 15 years) and adult MMA (≥ 15 years) showed different survival curves according to stroke presentation type (log-rank test; P = 0.017, P < 0.001, respectively).

Conclusions

Our study showed that moyamoya patients had different diagnosis ages and distinct survival courses according to stroke presentation type. Adult moyamoya patients with hemorrhagic presentation had the worst survival outcomes.

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References

  1. Ahn IM, Park DH, Hann HJ, Kim KH, Kim HJ, Ahn HS (2014) Incidence, prevalence, and survival of moyamoya disease in Korea: a nationwide, population-based study. Stroke 45:1090–1095. https://doi.org/10.1161/STROKEAHA.113.004273

    Article  PubMed  Google Scholar 

  2. Ahn SH, Lee J, Kim YJ, Kwon SU, Lee D, Jung SC, Kang DW, Kim JS (2015) Isolated MCA disease in patients without significant atherosclerotic risk factors: a high-resolution magnetic resonance imaging study. Stroke 46:697–703. https://doi.org/10.1161/STROKEAHA.114.008181

    Article  CAS  PubMed  Google Scholar 

  3. Baba T, Houkin K, Kuroda S (2008) Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 79:900–904. https://doi.org/10.1136/jnnp.2007.130666

    Article  CAS  PubMed  Google Scholar 

  4. Cheng JH, Ye Q, Ye ZS, Li ZQ, Xu AD (2016) Five-year experience of 101 adult patients with moyamoya disease at a single institution in Eastern China. J Clin Neurosci 32:30–34. https://doi.org/10.1016/j.jocn.2015.12.042

    Article  PubMed  Google Scholar 

  5. Ge P, Zhang Q, Ye X, Liu X, Deng X, Li H, Wang R, Zhang Y, Zhang D, Cao Y, Wang S, Zhao J (2017) Clinical features of hemorrhagic moyamoya disease in China. World Neurosurg 106:224–230. https://doi.org/10.1016/j.wneu.2017.06.145

    Article  PubMed  Google Scholar 

  6. Han DH, Kwon OK, Byun BJ, Choi BY, Choi CW, Choi JU, Choi SG, Doh JO, Han JW, Jung S, Kang SD, Kim DJ, Kim HI, Kim HD, Kim MC, Kim SC, Kim SC, Kim Y, Kwun BD, Lee BG, Lim YJ, Moon JG, Park HS, Shin MS, Song JH, Suk JS, Yim MB, Korean Society for Cerebrovascular D (2000) A co-operative study: clinical characteristics of 334 Korean patients with moyamoya disease treated at neurosurgical institutes (1976–1994) The Korean Society for Cerebrovascular Disease. Acta Neurochir (Wien) 142(1263):1273. https://doi.org/10.1007/s007010070024 (discussion 1273-1264)

    Article  Google Scholar 

  7. Hishikawa T, Tokunaga K, Sugiu K, Date I (2013) Clinical and radiographic features of moyamoya disease in patients with both cerebral ischaemia and haemorrhage. Br J Neurosurg 27:198–201. https://doi.org/10.3109/02688697.2012.717983

    Article  PubMed  Google Scholar 

  8. Jang DK, Lee KS, Rha HK, Huh PW, Yang JH, Park IS, Ahn JG, Sung JH, Han YM (2014) Clinical and angiographic features and stroke types in adult moyamoya disease. AJNR Am J Neuroradiol 35:1124–1131. https://doi.org/10.3174/ajnr.A3819

    Article  PubMed  PubMed Central  Google Scholar 

  9. Jang DK, Lee KS, Rha HK, Huh PW, Yang JH, Park IS, Ahn JG, Sung JH, Han YM (2017) Bypass surgery versus medical treatment for symptomatic moyamoya disease in adults. J Neurosurg 127:492–502. https://doi.org/10.3171/2016.8.JNS152875

    Article  PubMed  Google Scholar 

  10. Jo KI, Yeon JY, Hong SC, Kim JS (2014) Clinical course of asymptomatic adult moyamoya disease. Cerebrovasc Dis 37:94–101. https://doi.org/10.1159/000356350

    Article  PubMed  Google Scholar 

  11. Kim DS, Jang DK, Huh PW, Yoo DS, Han YM, Huh CW (2011) Ischaemic stroke after acute intracranial haemorrhage in patients with moyamoya disease: six new cases and a short literature review. Acta Neurochir (Wien) 153:1253–1261. https://doi.org/10.1007/s00701-010-0926-9

    Article  Google Scholar 

  12. Kim T, Lee H, Bang JS, Kwon OK, Hwang G, Oh CW (2015) Epidemiology of moyamoya disease in Korea: based on national health insurance service data. J Korean Neurosurg Soc 57:390–395. https://doi.org/10.3340/jkns.2015.57.6.390

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Kuroda S, Hashimoto N, Yoshimoto T, Iwasaki Y, Research Committee on Moyamoya Disease in J (2007) Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke 38:1430–1435. https://doi.org/10.1161/STROKEAHA.106.478297

    Article  Google Scholar 

  14. Miyatake S, Miyake N, Touho H, Nishimura-Tadaki A, Kondo Y, Okada I, Tsurusaki Y, Doi H, Sakai H, Saitsu H, Shimojima K, Yamamoto T, Higurashi M, Kawahara N, Kawauchi H, Nagasaka K, Okamoto N, Mori T, Koyano S, Kuroiwa Y, Taguri M, Morita S, Matsubara Y, Kure S, Matsumoto N (2012) Homozygous c.14576G>A variant of RNF213 predicts early-onset and severe form of moyamoya disease. Neurology 78:803–810. https://doi.org/10.1212/WNL.0b013e318249f71f

    Article  CAS  PubMed  Google Scholar 

  15. Nanba R, Kuroda S, Takeda M, Shichinohe H, Nakayama N, Ishikawa T, Houkin K, Iwasaki Y (2003) Clinical features and outcomes of 10 asymptomatic adult patients with moyamoya disease. No Shinkei Geka 31:1291–1295

    PubMed  Google Scholar 

  16. Research Committee on the P, Treatment of Spontaneous Occlusion of the Circle of W, Health Labour Sciences Research Grant for Research on Measures for Infractable D (2012) Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis). Neurol Med Chir (Tokyo) 52:245–266. https://doi.org/10.2176/nmc.52.245

    Article  Google Scholar 

  17. Sato Y, Kazumata K, Nakatani E, Houkin K, Kanatani Y (2019) Characteristics of moyamoya disease based on national registry data in Japan. Stroke 50:1973–1980. https://doi.org/10.1161/STROKEAHA.119.024689

    Article  PubMed  Google Scholar 

  18. Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299. https://doi.org/10.1001/archneur.1969.00480090076012

    Article  CAS  PubMed  Google Scholar 

  19. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, Kojima M, Lin Y, Ohno Y (1997) Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 99(Suppl 2):S1-5. https://doi.org/10.1016/s0303-8467(97)00031-0

    Article  PubMed  Google Scholar 

Download references

Funding

This research was supported by a Grant of Translational R&D Project through Institute for Bio-Medical convergence, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea.

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Authors and Affiliations

  1. Department of Neurosurgery, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea

    Sang-Hyuk Im

  2. Department of Neurosurgery, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea

    Dong-Kyu Jang

  3. Department of Neurosurgery, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 56, Dongsu-ro, Bupyeong-gu, Incheon, 21431, Republic of Korea

    Dong-Kyu Jang

  4. Department of Neurosurgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea

    Hoon Kim

  5. Department of Neurosurgery, Gangnam Severance Hospital, Yonsei University College of Medicine, 211, Eonju-ro, Gangnam-gu, Seoul, 06273, Republic of Korea

    Sang-Kyu Park

  6. Department of Biostatistics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea

    Kyung-Do Han

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Correspondence to Dong-Kyu Jang.

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Comments

The authors reported long-term mortality in patients with moyamoya angiopathy (MMA) using the Korean National Health Insurance database. They included 12,146 newly diagnosed MMA cases between 2006 and 2015. They classified into three groups according to their stroke presentation type: ischemic MMA (30.2%), hemorrhagic MMA (20.2%), and asymptomatic or else MMA (49.6%). They clearly showed that adult MMA patients with hemorrhagic presentation had the worst survival outcome in this study. This is important and informative message to the readers from South Korea. On the other hand, the caution should be taken because MMA differs according to patient demographic groups.

Kenji Sugiu,

Okayama city, Japan

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Im, SH., Jang, DK., Kim, H. et al. Long-term mortality in patients with moyamoya angiopathy according to stroke presentation type in South Korea. Acta Neurochir 163, 3473–3481 (2021). https://doi.org/10.1007/s00701-021-04959-0

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  • DOI: https://doi.org/10.1007/s00701-021-04959-0

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