Histopathological Characteristics of Distal Middle Cerebral Artery in Adult and Pediatric Patients with Moyamoya Disease

Abstract

Moyamoya disease (MMD) is a unique progressive steno-occlusive disease of the distal ends of bilateral internal arteries and their proximal branches. The difference in clinical symptoms between adult and children MMD patients has been well recognized. In this study, we sought to investigate the phenomenon through histopathological study. Fifty-one patients underwent surgical procedures for treatment of standard indications of MMD at Kyoto University Hospital. Fifty-nine specimens of MCA were obtained from MMD patients during the surgical procedures. Five MCA samples were also obtained in the same way from control patients. The samples were analyzed by histopathological methods. In this study, MCA specimens from MMD patients had significantly thinner media and thicker intima than control specimens. In subsequent analysis, adult (≥ 20 years) patients had thicker intima of MCA compared to pediatric (< 20 years) patients. There is no difference in internal elastic lamina pathology between adult and pediatric patients. Our results indicated that the pathological feature of MMD in tunica media occurs in both adult and pediatric patients. However, the MMD feature in tunica intima of MCA is more prominent in adult patients. Further analysis from MCA specimens and other researches are necessary to elucidate the pathophysiology of MMD.

Clinical and angiographic outcomes after combined direct and indirect bypass in adult patients with moyamoya disease: A retrospective study of 76 procedures

“…In addition, patients with direct revascularization are prone to developing cerebral hemodynamic disturbances and perioperative complications (16). In addition, recipient vessels of MMD patients always have a small diameter and thin wall, which are difficult to be anastomosed by inexperienced surgeons or hospitals (17,18). By contrast, indirect revascularization facilitates the formation of new blood vessels on the surface of the brain through the STA, the dura, the temporal muscle and the periosteum anastomosis (19)(20)(21).…”

Transcriptomic Profile of Distal Middle Cerebral Artery from Moyamoya Disease Patients Reveals a Potential Unique Pathway

“…This study was performed under our institution guidelines provided by the ethics committee in our center as being described in our previous reports [3], [10], [11], [22]. Six patients underwent superficial temporal artery to MCA (STA-MCA) bypass procedures for the treatment of MMD at our center.…”

“…Moyamoya disease (MMD) is a peculiar disease, characterized by progressive steno-occlusion of the distal ends of bilateral internal carotid arteries (ICAs) and their proal vascular network at the base of the brain (moyamoya vessels) is formed as a result of progressive ischemic changes in the brain [1], [2], [3]. The high incidence of MMD in Japanese population and high proportion of familial cases suggest the involvement of a genetic factor, particularly polymorphism in RNF213 gene [4], [5], [6].…”

“…Pathological evaluations on autopsy subjects have demonstrated that the main vascular lesion in MMD is steno-occlusion of distal ICA caused by a fibrocellular intimal thickening with a multilayered elastic lamina and some lipid deposits [3], [7], [8], [9]. Despite there is an intimal proliferation, the media layer thickness is thinner [2], [10], and the smooth muscle cells undergo apoptosis [11].…”

Prospective evaluation of the diagnostic value of plasma apelin 12 levels for differentiating patients with moyamoya and intracranial atherosclerotic diseases

“…Although the prevalence of MMD is higher in East Asian countries, including Japan, Korea and China 2, 3 , MMD has been observed in various populations worldwide 4 . The frequency of transient ischaemic attacks in patients with MMD and chronic cerebral ischaemia is similar to that in patients with other aetiologies.…”