Distinct presentation of Moyamoya disease in Iran

Moyamoya Disease (MMD) is a rare cerebrovascular disorder characterized by stenosis of the internal carotid arteries. To date, no studies have specifically described the characteristics of Moyamoya among Iranian populations. This study aims to examine the clinical characteristics of a case series of Iranian patients with MMD. This study prospectively identified all patients diagnosed with MMD at an institutional tertiary hospital in Tehran from 2010 to 2020. Data on demographic characteristics, disease presentations, past medical history, type of vascular lesion, treatment approaches, and outcomes during follow-up were collected. A total of 15 patients with MMD were included. Of these, 9 (60%) were female. Age distribution at diagnosis showed 6 patients were younger than 40 years. No familial patterns of MMD were observed. All patients experienced Cerebrovascular Accidents (CVA) at disease onset. A notable prevalence of comorbid conditions was observed, including hypertension (33.3%), diabetes mellitus (20%), and seizure disorders (20%). Vascular lesions were unilateral in 46.6% of patients and bilateral in 53.4%. Direct bypass surgery was performed in 40% of cases. The mean follow-up period was 16.3±1.2 months. During this time, 2 patients died and 1 experienced a new CVA. Residual symptoms were present in 13.3% of patients. This study shows distinct characteristics of MMD in an Iranian cohort, including a female predominance, frequent ischemic symptoms, and a high rate of bilateral occlusions. Further prospective studies are warranted to assess the efficacy of medical and surgical interventions in preventing recurrent symptoms in this population.