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Diagnosis and treatment of adult Moyamoya disease

Article information

J Korean Med Assoc. 2019;62(11):577-585
Publication date (electronic) : 2019 November 12
doi : https://doi.org/10.5124/jkma.2019.62.11.577
Jeong Eun Kim, MDorcid_icon, Chang Hwan Pang, MDorcid_icon
Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Korea.
Corresponding author: Jeong Eun Kim. eunkim@snu.ac.kr
Received 2019 September 27; Accepted 2019 October 20.

Abstract

Moyamoya disease (MMD) refers to a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery with abnormal collateral vessel formation of unknown etiology. The definite diagnosis of MMD requires cerebral angiography or magnetic resonance angiography and/or magnetic resonance imaging after excluding other underlying diseases, particularly in adult patients. The treatment aims to improve regional cerebral blood flow to prevent cerebral ischemic events and alleviate hemodynamic instability that can provoke cerebral hemorrhage. Although various surgical revascularization methods have been introduced, combined revascularization surgery including direct revascularization is preferred over indirect revascularization only in adult MMD patients. Several recent studies have shown that surgical treatment has better outcomes and prognosis for symptomatic hemodynamically unstable MMD patients with both ischemic and hemorrhagic presentations. For asymptomatic patients, follow up with appropriate imaging is recommended. Surgery should be considered when new symptoms emerge with hemodynamic aggravation.

Keywords: Diagnosis; Therapy; Adult; Moyamoya disease

Notes

Conflict of Interest

No potential conflict of interest relevant to this article was reported.

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This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Figure 1

Figure 1

Diagnostic diagram for adult Moyamoya disease (MMD) in Seoul National University Hospital. The diagnostic criteria for adult Moyamoya disease are based on the exclusion principle. Reproduced from Kim JE et al. Neurol Med Chir (Tokyo) 2012;52:311-317, according to the Creative Commons license [5].

Figure 2

Figure 2

The characteristics of Moyamoya disease in magnetic resonance (MR) imaging. (A) The MR imaging shows that the signal voids of the major vessels in the Circle of Willis are missing (arrows), and that there are multiple small punctate holes in the basal ganglia (arrow heads) that represents typical Moyamoya vessels. (B) FLAIR MR imaging shows multiple high signal intensities at cortical sulci (arrows), called the ‘Ivy sign.’

Figure 3

Figure 3

The typical cerebral angiographic features of Moyamoya disease. Digital subtraction angiography shows bilateral steno-occlusive change of the terminal internal carotid artery (arrows), and characteristic collateral vessels, named as ‘Moyamoya (puff of smoke)’ (arrow heads). (A) Anteroposterior view of internal carotid angiography and (B) lateral view of internal carotid angiography.

Figure 4

Figure 4

The characteristic features of a hemodynamic study in Moyamoya disease. (A) The single photon emission computed tomography and (B) magnetic resonance arterial spin labeling imaging shows marked decreases in both the cerebrovascular reserve capacity and tissue perfusion in the right cerebral hemisphere (arrows).

Figure 5

Figure 5

Management flow sheet for adult Moyamoya disease in Seoul National University Hospital. TFCA, transfemoral cerebral angiography; D-SPECT, Diamox-challenged single photon emission computed tomography; CT, computed tomography; MR, magnetic resonance image; FU, follow-up; CTA, computed tomographic angiography; MRA, magnetic resonance angiography.

Table 1

Associated factors for pathophysiology of Moyamoya disease

Table 1

Table 2

Diagnostic criteria for Moyamoya disease

Table 2

Adapted from Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis; Health Labour Sciences Research Grant for Research on Measures for Infractable Diseases. Neurol Med Chir (Tokyo) 2012;52:245-266 [11].

MRI, magnetic resonance imaging; MRA, magnetic resonance angiography.

Table 3

Various revascularization surgical methods

Table 3

MCA, middle cerebral artery; STA, superficial temporal artery; PCA, posterior cerebral artery; OA, occipital artery.