Moyamoya disease manifested as multiple simultaneous intracerebral hemorrhages: A case report and literature review

Abstract

Multiple simultaneous intracerebral hemorrhages (MSIH) caused by Moyamoya disease (MMD) is extremely rare. To date, the clinical manifestations, imaging characteristics and mechanism of MMD-induced MSIH have not yet been elucidated. In order to improve the understanding on such cases, the present study described a rare case of MSIH caused by MMD. A 40-year-old female patient with no history of hypertension or diabetes mellitus experienced a sudden headache followed by coma. Cranial computed tomography (CT) examination revealed MSIH in the left frontal area, temporal lobe and basal ganglia. CT angiography and digital subtraction angiography examinations revealed typical characteristics of MMD. Subsequent to excluding disorders of the blood system and blood coagulation, we concluded that the present case of MSIH was caused by MMD. Hematoma evacuation and decompressive craniectomy were performed with satisfactory results. In addition, after reviewing previous MSIH cases in the literature, potential mechanisms of MMD-mediated MSIH were considered. In conclusion, MMD should be considered as a possible cause of MSIH during diagnosis and treatment. MMD can lead to pathological changes in the fragility of small arteries; therefore, rupture and hemorrhage at one site may induce a transient increase in blood pressure, causing the rupture of small arteries at other sites, and thus leading to MSIH. Hematoma evacuation and decompression should be conducted in selective cases of MMD-induced MSIH in order to achieve a good prognosis.

Analysis of the clinical characteristics of hemorrhagic moyamoya disease in the Jilin province of northeastern China: A single-center study of 212 cases

“…Previous studies have also demonstrated that a notable proportion of adult patients exhibited minor hemorrhage with or without symptoms, which significantly predicted the deterioration of MMD and increased the hemorrhage risk ( 3 , 50 ). In the current study, the rate of mild disability, namely prognostic GOS scores of 5 or 4, was markedly lower in the MMD patients with a history of hemorrhage than in the MMD patients without a history of hemorrhage, which may be due to the MMD patients with a history of hemorrhage undergoing repeated progression, and thus demonstrating rapid progression that resulted in irreversible damage.…”