Description of a Cohort of Patients with the Moyamoya Syndrome in a Rehabilitation Hospital

Background Moyamoya disease (MMD) is a chronic steno-occlusive cerebrovascular condition initially described in 1957 in a Japanese patient. The classic angiographic findings include bilateral stenosis or occlusion at the terminal portion of the internal carotid artery, with an abnormal vascular network at the base of the brain. There are few epidemiological descriptions of non-Asian cases. Objectives This study aimed at evaluating a cohort of patients with the MMD admitted in a quaternary, open-access rehabilitation hospital in central Brazil. Methods This was a retrospective, observational study. Two neurologists reviewed data from electronic records of patients with MMD admitted to SARAH Hospital in Brasilia in the past sixteen years. Data on etiology, associated conditions and general characteristics were collected. The modified Rankim scale (mRS) was used to determine morbidity. Results Twenty-one patients were identified and selected for this research. The average age at presentation was 20.5 years (range: 0 - 69 years), 62% were females, and 43% reported Asian ancestry. Ischemic stroke (IS) occurred in 95%, hemorrhagic stroke (HS) in 19%, and both IS and HS in 14%. Stroke recurred in 76% during follow-up; of these, 87% had IS. Because no other associated condition was found, 80% of the patients could be classified as MMD; 15% had the Moyamoya syndrome, with the presence of other conditions associated with the disease. Our hospital-based study showed a lower average age of onset than that in the literature, a much higher incidence of IS, and similar rates of HS. Patients with HS scored worse on an initial and final mRS, and surgical procedures did not significantly alter the prognosis. In comparison with those reported in international literature, we did not find significant differences in disability rates between younger and older patients. The average mRS score at admission was 3.4 (range: 2 - 4), with 2.9 (range: 1 - 4) at discharge. Conclusions Rehabilitation was able to improve the quality of life, as shown by the mRS at admission and discharge. Patients with HS tended to have worse outcomes.