Patient characteristics of moyamoya disease according to region
| Author, year | Region | No of patients* | Female:male ratio | Positive family history | Age peaks | Mode of presentation, % (95% CI) | ||||
| Childhood peak | Adulthood peak | Ischaemia | ICH | Asymptomatic | Other | |||||
| Waka 19974 | Japan | 1176† (1055/121/0) | 1.8 (1.6–2.0)‡ | 10% | 10–14‡ | 40–49‡ | – | – | – | – |
| Kuriyama 20085 | Japan | 1240† (1170/70/0) | 1.8 (1.6–2.1)‡ | 12% | ♀ 20–24‡ ♂ 10–14‡ | ♀ 50–54‡ ♂ 35–39‡ | – | – | – | – |
| Baba 200714 | Japan: Hokkaido | 267 (233/34/0) A: 227§§ C: 40 | 2.2 (1.7–2.8)‡ | 15% | 5–9‡ | 45–49‡ | T 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 200715 | Taiwan | 92 A: 68** C: 24 | 1.3 (0.9–2.0) | 0% | – | 31–40 | T 48 (37–59) A 35 (24–48) C 83 (63–95) | T 52 (42–63) A 65 (52–76) C 17 (5–37) | T 0 | T 0 |
| Miao 201016 | China: Nanjing | 202 (202/0/0) A: 180§§ C: 22 | 1.1 (0.9–1.5) | 1.48% | 5–9 | 35–45 | T 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 0 | T 0 |
| Wetjen 199818 | USA: Iowa | 30 (26/3/1) A: 26†† C: 4 | 2.8 (1.2–6.1)§ | – | 1–10‡ | 31–45‡ | T 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 200517 | USA: California, Washington | 298 | 2.1 (1.7–2.7)§ | – | 5–9§ | 55–59§ | T 26¶ | T 21¶ | – | T 22¶ |
| Graham 199719 | USA: Hawaii | 21 (21/0/0) A: 17†† C: 4 | 1.6 (0.7–3.8) | – | – | 40–49 | T 52 (30–74) A 53 (28–77) C 75 (19–99) | T 29 (13–52) A 29 (10–56) C 25 (1–81) | T 0 | T 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.