Background
Quasi-moyamoya disease (MMD) or moyamoya syndrome is based on various underlying diseases
and radiologically simulates MMD, but its disease entity is still unclear. Recent
studies have proven specific shrinkage of the involved arteries in MMD. Using 3-dimensional
constructive interference in steady state (3D-CISS), therefore, this study aimed to
analyze the outer diameter of the involved arteries in quasi-MMD.
Methods
This study included 9 patients with quasi-MMD (unilateral type, n = 2; bilateral type,
n = 7). Using 3D-CISS, the outer diameter was quantified in the internal carotid artery
distal to the posterior communicating artery (C1), the horizontal portion of the middle
and anterior cerebral arteries (M1 and A1, respectively), and the basilar artery.
Control values were obtained from 17 healthy subjects.
Results
In 7 of 9 patients, the outer diameters of C1, M1, and A1 were significantly smaller
than those of the controls. On the other hand, the values were normal in other 2 patients.
There was no significant difference in the underlying disorders between the 2 groups.
All 3 pediatric patients are categorized into the arterial shrinkage group, but 2
of 6 adult patients were not.
Conclusions
These findings strongly suggest that quasi-MMD is not a uniform disease entity and
includes at least 2 pathophysiologically different groups: the arterial shrinkage
group and the nonarterial shrinkage group. A certain subgroup of MMD patients may
be misdiagnosed as quasi-MMD because of the patients' comorbid disorders and mixed
up with the patients who present angiographic findings similar to MMD in spite of
the lack of arterial shrinkage.
Key Words
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Article info
Publication history
Published online: March 30, 2016
Accepted:
February 20,
2016
Received in revised form:
February 16,
2016
Received:
February 4,
2016
Footnotes
Source of funding: This study was supported by a grant from the Research Committee on Moyamoya Disease, sponsored by the Ministry of Health, Labor, and Welfare of Japan. The grant number is H26-078. Satoshi Kuroda received the funding.
Identification
DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2016.02.029
Copyright
© 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.
