SUN-134 The Frequency of Type 1 Diabetes among Adolescent Patients with Moyamoya Disease

Background: Moyamoya disease is a rare cerebrovascular condition characterized by stenosis or occlusion of the anterior circulation vessels of the brain, with development of the collateral vessels. The pathogenesis of moyamoya is not well known. The role of immunology as well as the role of genetics has been suggested, and recent reports have noted an association between moyamoya and type 1 diabetes mellitus (T1DM). In this study, we evaluated the frequency of T1DM in children and adolescents with moyamoya disease. Methods: All children and adolescents who were confirmed moyamoya disease by magnetic resonance angiography (MRA) before the age of 20 years in Severance Hospital were included. Retrospectively chart review was conducted and we evaluated the frequency of endocrine autoimmune disease among patients with moyamoya disease. In addition, the clinical characteristics of patients with both of T1DM and moyamoya disease are described. Results: Seven hundreds and two patients (current mean age, 14.7 ± 4.8 years) were included. They were diagnosed moyamoya disease at an average age of 9.0 years. Female patients were 57.3%. Five of them (0.71%, mean age 13.3 years, one male) were noted T1DM. This prevalence was significantly higher than that in general Korean adolescent (0.081%). The incidence of T1DM in Moyamoya disease was 125 per 100,000 per year and this was also significantly higher than that in general Korean adolescent population (3.19 per 100,000 per year). One of them had been diagnosed both of T1DM and Grave’s disease. Two patients with T1DM were diagnosed with moyamoya disease earlier than T1DM, and at the time of diagnosis with diabetes, their C-peptide levels were not definitely low. However, their C-peptide levels decreased gradually in a short period of time and they also had GAD antibody. All patients had Moyamoya disease, bilaterally. Conclusion: In the present study, not a few patients have both of moyamoya disease and T1DM. This supports a possible autoimmune component to the pathogenesis of moyamoya disease. Because stroke-like symptoms in moyamoya syndrome are similar to hypoglycemic symptoms, we recommend that clinicians consider the T1DM when evaluating patients with moyamoya disease.