Moyamoya disease and moyamoya syndrome: A case series from multicentre private hospitals in Indonesia.

Abstract

Background: Moyamoya disease (MMD) and moyamoya syndrome (MMS) are considered rare in Indonesia, without any proper epidemiological data backing this claim. Hence, this case series aims to assess all MMD and MMS cases from the perspective of a multicentre private hospital in Indonesia.

Methods: This is a descriptive analysis using data from the picture archiving and communication system (PACS) from January 2019 to December 2023. The inclusion criteria included all patients who fulfilled the radiological criteria for MMD and/or MMS, while patients who only underwent brain non-contrast computed tomography (CT) scans were excluded.

Results: There are 58,905 unique MRI scans from 2019 to 2023. The cohort comprises 8 females and 2 males, with a median age of 37 (7-65) years old. Three cases are probable MMD. with six MMD cases and one MMS case. Therefore, the prevalence rate for the four years is approximately 11.9 cases per 100,000 scans. Out of the confirmed Moya-Moya cases, the majority (4/7) are ischemic subtypes, followed by epileptic (electroencephalography shows slowing brain waves) and TIA in one case each. The most common presenting symptom is weakness in the extremity (N = 7), followed by headache (N = 5). Four patients underwent operative procedures, with three of them being superior temporal artery to middle cerebral artery (STA-MCA) bypass procedures and one of them being encephalo-duro-myo-arterio-pericraniosynangiosis (EDMAPS).

Conclusion: The paucity of confirmed cases either points towards the low prevalence of MMD and MMS in Indonesia or the underdiagnosis of these cases.