Published Online
on September 22, 2005

A more recent version of this article appeared on October 1, 2005

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Submitted on May 9, 2005
Accepted on June 2, 2005

Incidence and Clinical Features of Disease Progression in Adult Moyamoya Disease

Satoshi Kuroda MD, PhD^*; Tatsuya Ishikawa MD, PhD; Kiyohiro Houkin MD, PhD; Rina Nanba MD, PhD; Masaaki Hokari MD, PhD; and Yoshinobu Iwasaki MD, PhD

From the Department of Neurosurgery (S.K., T.I., R.N., M.H., Y.I.), Hokkaido University Graduate School of Medicine; and the Department of Neurosurgery (K.H.), Sapporo Medical University, Sapporo, Japan

^* To whom correspondence should be addressed. E-mail: skuroda{at}med.hokudai.ac.jp.

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.

Key words: adult • cerebral ischemia • disease progression • moyamoya disease

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