Introduction: Moyamoya disease is a progressive occlusive disease of the cerebral vasculature, with abnormal collateral networks bypassing stenotic vessels. Though first described as a bilateral phenomenon affecting East Asians, similar angiographic features are evident in patients with other medical conditions, known as moyamoya syndrome. Most conditions in the literature referencing moyamoya syndrome exist only as case reports. This study reports both new and previously identified conditions associated with moyamoya syndrome within a Kentucky regional population.
Methods: Inclusion criteria for this retrospective chart review were patients evaluated at University of Kentucky Medical Center, from June 1, 2011 to June 1, 2015, diagnosed with moyamoya and treated by the investigators. Data collected include patient demographics, presenting manifestation, vessels involved, co-morbid conditions present, abnormal laboratory values, and treatment administered. Data storage and analysis were performed using REDCap hosted at the University of Kentucky; the protocol was IRB-approved.
Results: Twenty patients with moyamoya were enrolled (6 males, 14 females). Of these, 16 presented with ischemic stroke, 2 with hemorrhagic stroke, and 2 with other (headache, TIA). 65% had involvement of the ICA, 70% of the MCA, and 20% of the ACA, with 40% having multi-vessel involvement. The most common co-morbid condition was hypertension in 60% of patients. Co-existing autoimmune conditions were present in 20% of patients, including autoimmune polyglandular syndrome 1, autoimmune hepatitis, Addison’s disease, psoriasis, ITP, hypothyroidism, Crohn’s disease, and multiple sclerosis. Another 20% had co-existing pro-thrombotic disorders, including protein S deficiency, Factor V Leiden, hemoglobin G trait, and prothrombin G20210A mutation. Seventeen patients were treated with indirect encephalo-duro-arterio-synangiosis bypass. All twelve receiving angiogram after bypass showed evidence of neovascularization.
Conclusions: Our retrospective study of moyamoya patients at UK Medical Center reveals multiple co-existing autoimmune and pro-thrombotic disorders. Our experience with indirect bypass demonstrates excellent evidence of re-vascularization within one year of treatment.
Patient Care: Numerous associated conditions have been reported in the literature in reference to moyamoya syndrome, but most occur as case reports. Few publications have analyzed the prevalence of such associations, or given a comprehensive overview of associated conditions within a regional population. The goal of this study is therefore to report both new and previously identified conditions in patients with moyamoya syndrome within a Kentucky population treated at the University of Kentucky Medical Center from 2011 to 2015. The identification of these coexisting conditions will hopefully serve to allow better conceptualization of the pathophysiology involved in moyamoya syndrome, as well as more timely and accurate diagnosis of moyamoya syndrome in patients with no other known risk factors for stroke. The study also evaluates outcomes associated with indirect encephalo-duro-arterio-synangiosis bypass as a treatment modality.
Learning Objectives: By the conclusion of this session, participants should be able to:
1) Differentiate between moyamoya disease and moyamoya syndrome
2) Describe some co-existing medical conditions found in association with moyamoya syndrome
3) Identify an effective treatment for revascularization in moyamoya patients