J Cerebrovasc Endovasc Neurosurg.  2014 Sep;16(3):241-246. 10.7461/jcen.2014.16.3.241.

Clinicoepidemiological Features of Asymptomatic Moyamoya Disease in Adult Patients

• Yang J
• Hong JC
• Oh CW
• Kwon OK
• Hwang G
• Kim JE
• Kang HS
• Cho WS
• Kim T
• Moon JU
• Ahn SY
• Kim JH
• Bang JS

• KMID: 1963162
• DOI: http://doi.org/10.7461/jcen.2014.16.3.241

Abstract

OBJECTIVE
The aim of this study was to document the natural course of asymptomatic adult moyamoya disease (MMD) and the factors related to disease progression to aid in treatment decisions.
MATERIALS AND METHODS
Among 459 adult MMD patients (aged > or = 20 years), 42 patients were included in this retrospective cohort study. Clinical records of adult asymptomatic MMD patients (n = 42) and follow-up data from September 2013 were reviewed to determine the factors related to disease progression.
RESULTS
The mean age of patients at the time of diagnosis was 41.2 years (range, 23-64 years), and the mean follow-up period was 37.3 months (range, 7.4-108.7 months). Of the 42 patients and 75 hemispheres, there were 12 patients (28.6%) and 13 hemispheres (17.3%) with disease progression. There were four hemispheres (5.3%) with symptomatic progression (three hemorrhage, one transient ischemic attack) and nine hemispheres (12.0%) with asymptomatic radiographic progression. There were no relationships with sex, diabetes, hypertension, thyroid disease, family history of MMD, or family history of stroke. However, reduced initial cerebrovascular reserve capacity was observed in seven hemispheres (9.3%) in patients with disease progression. A relationship was found between disease progression and initial cerebrovascular reserve capacity (p = 0.05). None of the patients underwent bypass surgery during the follow-up period.
CONCLUSION
It appears that asymptomatic adult MMD is not a permanent stable disease. In particular, reduced cerebrovascular reserve capacity is an indication of MMD progression, so close regular observation is needed.

Keyword

Asymptomatic disease; Cerebrovascular insufficiency; Moyamoya disease

MeSH Terms

Adult*
Asymptomatic Diseases
Cerebrovascular Disorders
Cohort Studies
Diagnosis
Disease Progression
Follow-Up Studies
Hemorrhage
Humans
Hypertension
Moyamoya Disease*
Retrospective Studies
Stroke
Thyroid Diseases

Figure

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  Fig. 1 Cumulative event free survival rate in patients with asymptomatic moyamoya disease who experienced asymptomatic or symptomatic disease progression (n = 12) CVR = cerebrovascular reserve

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  Fig. 2 Cumulative event free survival rate in patients with asymptomatic moyamoya disease who experienced symptomatic disease progression (n = 4) CVR = cerebrovascular reserve

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