Background
Several coexisting diseases have been reported in patients with moyamoya vasculopathy
(MMV), but studies of quasi-moyamoya disease (quasi-MMD) are rare. This study aims
to investigate the frequency of known coexisting diseases in patients with quasi-MMD
and to compare quasi-MMD with moyamoya disease (MMD).
Methods
Between 2000 and 2011, we retrospectively screened patients with International Classification of Diseases, Ninth Revision, code of 4375 (MMD) in the Health Information System of our hospital. The vascular
images of each patient were confirmed by 2 neurologists and 1 neuroradiologist based
on the diagnostic criteria of Japan Ministry of Health and Welfare. We excluded the
patients with missing images and erroneous diagnosis. Demographics, coexisting diseases,
laboratory data, treatment, and recurrent strokes were recorded. The eligible patients
were divided into quasi-MMD and MMD groups according to the presence or absence of
coexisting diseases.
Results
MMV was found in 90 patients including 37 (41.1%) quasi-MMD and 53 (58.9%) MMD. Atherosclerosis
(32.4%) and thyroid disease (29.7%) were the leading coexisting diseases in quasi-MMD.
Patients with MMD became symptomatic in a bimodal age distribution, whereas patients
with quasi-MMD became symptomatic in a single-peak distribution. The prognosis of
recurrent strokes was similar between quasi-MMD and MMD based on Kaplan–Meier analysis.
Conclusions
A bimodal distribution of onset age was noted in MMD, whereas a single-peak distribution
was found in quasi-MMD. Coexisting diseases were usually underevaluated but were more
common than expected in patients with MMV. Atherosclerosis and thyroid diseases were
the leading coexisting diseases in different preferential age.
Key Words
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Article info
Publication history
Published online: January 27, 2014
Accepted:
November 11,
2013
Received in revised form:
November 10,
2013
Received:
October 3,
2013
Footnotes
Y.-C.W and C.-H.L are co-first authors.
Disclosure: All the authors declare that they have no conflicts of interest.
Identification
DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2013.11.008
Copyright
© 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.