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Clinical features of probable moyamoya disease in Japan
K Ikezaki1, T Inamura, T Kawano
1Department of Neurosurgery, Kyushu University Faculty of Medicine, Fukuoka, Japan. nobu@ns.med.kyushu-u.ac.jp
Abstract:
To clarify the current clinical features of probable Moyamoya disease in Japan, 180 cases were analyzed based on the cases collected by the Research Committee on Spontaneous Occlusion of the Circle of Willis of the Ministry of Health and Welfare, Japan. Although the age distribution patterns of probable Moyamoya disease showed two peaks similar to that of definite cases, the pattern shifted to the right thus indicating a reversed children versus adult ratio. The female/male ratio was 1.65, which was not significantly different from that observed in definite cases. The family occurrence rate was 6.7%. The incidence of cerebral infarction and bleeding in probable cases was higher than that in definite cases. The incidence of hemorrhage was higher in females than in males. Angiographically probable cases were at earlier stages in comparison to definite cases. The rebuild-up phenomenon in electroencephalograms was less detectable than in definite cases. Surgical procedures were performed in 63.3% of all cases and approximately 81% of them underwent bypass surgery. Single encephalo-duro-arterio-synangiosis, direct bypass and combined bypass procedures were performed in 35, 40 and 25% of all surgical cases, respectively. Although the incidence of bleeding was higher in the probable cases, the outcomes of the patients were similar to that of the definite cases except for mortality. Approximately 7% of the probable cases developed into definite type within an average 6.6 year follow-up period. The majority of probable Moyamoya disease cases thus seems to have somewhat different clinical characteristics from the definite disease cases.
Insights
Probable Moyamoya disease in Japan presents distinct clinical features, including an altered age distribution and higher rates of stroke compared to definite cases. Some probable cases progress to the definite type over time.
Area of Science:
- Neuroscience
- Neurology
- Vascular Neurology
Background:
- Moyamoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries and their branches.
- Understanding the clinical spectrum of probable Moyamoya disease is crucial for accurate diagnosis and management.
Purpose of the Study:
- To elucidate the current clinical characteristics of probable Moyamoya disease in Japan.
- To compare the clinical features, progression, and outcomes of probable Moyamoya disease with definite cases.
Main Methods:
- Analysis of 180 probable Moyamoya disease cases collected by a Japanese research committee.
- Comparison of demographic data, clinical presentation, angiographic findings, electroencephalogram (EEG) results, surgical interventions, and patient outcomes with definite Moyamoya disease cases.
- Longitudinal follow-up to assess disease progression.
Main Results:
- Probable Moyamoya disease shows a reversed children versus adult ratio compared to definite cases, with a female/male ratio of 1.65.
- Higher incidence of cerebral infarction and hemorrhage observed in probable cases, particularly in females.
- Angiographically, probable cases represent earlier disease stages; surgical bypass procedures were performed in 63.3% of cases.
Conclusions:
- Probable Moyamoya disease exhibits distinct clinical features from definite Moyamoya disease, including differences in age distribution and stroke incidence.
- Despite a higher bleeding risk, outcomes are similar to definite cases, barring mortality.
- Approximately 7% of probable cases evolve into the definite type, underscoring the need for continued monitoring.