Table 3

Patient characteristics of moyamoya disease according to region

Author, yearRegionNo of patients*Female:male ratioPositive family historyAge peaksMode of presentation, % (95% CI)
Childhood peakAdulthood peakIschaemiaICHAsymptomaticOther
Waka 19974Japan1176 (1055/121/0)1.8 (1.6–2.0)10%10–1440–49
Kuriyama 20085Japan1240 (1170/70/0)1.8 (1.6–2.1)12%♀ 20–24 ♂ 10–14♀ 50–54 ♂ 35–39
Baba 200714Japan: Hokkaido267 (233/34/0) A: 227§§ C: 402.2 (1.7–2.8)15%5–945–49T 57 (51–63) A 53 (46–60) C 78 (62–90)T 21 (16–26) A 24 (18–29) C 3 (0–13)T 18 (13–23) A 19 (14–24) C 15 (5–30)T 4 (2–7) A 4 (2–7) C 5 (1–17)
Hung 200715Taiwan92 A: 68** C: 241.3 (0.9–2.0)0%31–40T 48 (37–59) A 35 (24–48) C 83 (63–95)T 52 (42–63) A 65 (52–76) C 17 (5–37)T 0T 0
Miao 201016China: Nanjing202 (202/0/0) A: 180§§ C: 221.1 (0.9–1.5)1.48%5–935–45T 40 (33–47) A 34 (27–41) C 86 (65–97)T 56 (50–63) A 62 (55–69) C 14 (3–35)T 4 (2–8) A 4 (2–8) C 0T 0
Wetjen 199818USA: Iowa30 (26/3/1) A: 26†† C: 42.8 (1.2–6.1)§1–1031–45T 77 (58–90) A 80 (61–94) C 75 (19–99)§T 10 (2–27) A 12 (2–30) C 0§T 0§T 13 (3–31) A 8 (1–25)C 25 (1–81)§
Uchino 200517USA: California, Washington2982.1 (1.7–2.7)§5–9§55–59§T 26T 21T 22
Graham 199719USA: Hawaii21 (21/0/0) A: 17†† C: 41.6 (0.7–3.8)40–49T 52 (30–74) A 53 (28–77) C 75 (19–99)T 29 (13–52) A 29 (10–56) C 25 (1–81)T 0T 19 (5–42) A 18 (4–43) C 0
  • * Between brackets are the numbers of patients with definite moyamoya disease (MMD), probable MMD and moyamoya syndrome (MMS), if specified in the article. If available, the number of patients with adult and childhood onset is shown.

  • Characteristics reported on all cases with MMD in the investigated period, not only newly diagnosed cases.

  • Results include both probable and definite cases of MMD.

  • § Results include probable and definite cases of MMD and cases of MMS.

  • §§ Adulthood defined as ≥10 years.

  • ** Adulthood defined as >15 years.

  • †† Article provides age at initial presentation to a medical facility, not age at onset.

  • Data were available for 53% of patients.

  • A, adulthood onset; C, childhood onset; ICH, intracerebral haemorrhage; T, total.