Incidence and Clinical Features of Disease Progression in Adult Moyamoya Disease

Abstract

Background and Purpose— The progression of occlusive lesions in the major intracranial arteries was believed to be very rare in adult patients with moyamoya disease. The present study aims to clarify the incidence and clinical features of disease progression in adult moyamoya disease. Methods— For the past 15 years, 120 adult Japanese patients were diagnosed with moyamoya disease. Of these, 63 patients were enrolled in this historical prospective cohort study on a total of 86 nonoperated hemispheres. All were followed up with a mean period of 73.6 months. MRI and magnetic resonance angiography were repeated every 6 to 12 months, and cerebral angiography was performed when disease progression was suspected on MRI and magnetic resonance angiography. Results— Disease progression occurred in 15 of 86 nonoperated hemispheres (17.4% per hemisphere) or in 15 of 63 patients (23.8% per patient) during the follow-up period. Occlusive arterial lesions progressed in both anterior and posterior circulations, in both symptomatic and asymptomatic patients, and in both bilateral and unilateral types. Eight of 15 patients developed ischemic or hemorrhagic events in relation to disease progression. Multivariate analysis revealed that the odds ratio conferred by a male patient was 0.20 (95% CI, 0.04 to 0.97). Conclusions— The incidence of disease progression in adult moyamoya disease is much higher than recognized before, and female patients may be at higher risk for it than male patients. Careful follow-up would be essential to prevent additional stroke occurrence in medically treated adult patients with moyamoya disease, even if they are asymptomatic or are diagnosed as having unilateral moyamoya disease.

Current Status of Revascularization Surgery for Moyamoya Disease: Special Consideration for Its ‘Internal Carotid-External Carotid (IC-EC) Conversion’ as the Physiological Reorganization System

“…It had been required that steno-occlusive change at ICA should be evident bilaterally for the definitive diagno- In light of the increasing number of the patients with unilateral involvement as well as the evidence that substantial number of unilateral cases could progress to the bilateral presentation (Kuroda et al 2005), diagnostic criteria of the definitive moyamoya disease was revised to include patients, demonstrating both bilateral and unilateral involvement of terminal ICA stenosis associated with abnormal vascular network at the base of the brain with unknown etiology, as stated by the Research Committee of Moyamoya Disease of the Japanese Ministry of Health, Labour, and Welfare in 2015. Diagnostic criteria also state that definitive diagnosis of moyamoya disease requires catheter angiography in unilateral cases while bilateral cases could be promptly diagnosed by either catheter angiography or magnetic resonance (MR) imaging/angiography.…”

Paradoxical Association of Moyamoya Syndrome With Large Middle Cerebral Artery Aneurysm and Subarachnoid Hemorrhage -Case Report-

“…10) The incidence of disease progression in adult moyamoya disease was proven to be higher than recognized before. 9) Atherosclerotic background of the disease might be involved in the disease progression in our case.…”

Headache attack followed by rapid disease progression in pediatric moyamoya disease—how should we manage it?

“…In addition, it is well known that functional and/or intellectual outcomes are poor in pediatric patients presenting with ischemic stroke due to moyamoya disease [13][14][15]. To prevent the occurrence of cerebral infarction, therefore, regular imaging studies should also be planned with shorter intervals in the conservatively treated children with moyamoya disease, because MRI and MRA studies enable it to accurately detect disease progression at outpatient clinic [16]. Surgical revascularization should also be indicated in the cases that show rapid disease progression in order to prevent permanent neurological and cognitive deficits.…”