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  • Author or Editor: Young Jae Kim x
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Won-Sang Cho, Young Seob Chung, Jeong Eun Kim, Jin Pyeong Jeon, Young Je Son, Jae Seung Bang, Hyun-Seung Kang, Chul-Ho Sohn and Chang Wan Oh

OBJECT

Moyamoya disease (MMD) is a rare cerebrovascular disease and its natural history is still unclear. The authors aimed to investigate the natural course of hemodynamically stable cases of adult MMD, with the analysis of stroke risk factors.

METHODS

Two hundred forty-one patients were included in this retrospective study. One hundred sixty-six (68.9%) were female, and mean age (± SD) at first visit was 41.3 ± 12.0 years (range 18–69 years). Unilateral involvement was identified in 33 patients, and 19 patients (7.9%) had a family history of MMD. According to the clinical presentations, patients were classified into hemorrhagic (n = 62, 25.7%), ischemic (n = 144, 59.8%), and asymptomatic (n = 35, 14.5%) groups. The mean duration of follow-up was 82.5 ± 62.9 months (range 7.3–347.0 months).

RESULTS

The annual stroke risk was 4.5%, and the annual risks of rebleeding in the hemorrhagic group and recurrent ischemic events in the ischemic group were 4.3% and 3.0%, respectively. There was no significant difference in cumulative stroke risk between the 3 groups (p = 0.461). Risk factors included thyroid disease for overall strokes (HR 2.56, 95% CI 1.16–5.67), initial hemorrhagic presentation for hemorrhagic strokes (HR 2.53, 95% CI 1.24–5.17), and initial ischemic presentation for ischemic strokes (HR 2.69, 95% CI 1.15–6.27). Familial MMD was a common risk factor for all types of stroke. Among the 3 clinical groups, the hemorrhagic group showed the worst clinical status at discharge and at most recent follow-up. Twenty-three patients (9.5%) eventually underwent revascularization surgery.

CONCLUSIONS

There was no statistically significant difference in the incidence of stroke in the different clinical groups; clinical status, however, was most severe in patients with hemorrhagic presentation. In patients who experienced stroke during the follow-up period, the stroke type tended to correspond to their initial presentation. Close follow-up is needed in patients with thyroid disease and a family history of MMD.

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Jin Pyeong Jeon, Jeong Eun Kim, Jun Hyong Ahn, Won-Sang Cho, Young Dae Cho, Young-Je Son, Jae Seung Bang, Hyun-Seung Kang, Chul-Ho Sohn, Hyun-Tai Chung, Chang Wan Oh and Dong Gyu Kim

OBJECT

Treatment strategies for venous-predominant arteriovenous malformation (vp-AVM) remain unclear due to the limited number of cases and a lack of long-term outcomes. The purpose of this study was to report the authors’ experience with treatment outcomes with a review of the pertinent literature in patients with vp-AVM.

METHODS

Medical and radiological data from 1998 to 2011 were retrospectively evaluated. The degree of the arteriovenous (AV) shunt was categorized into 2 groups, a high- and low-flow AV shunt based on the angiographic findings.

RESULTS

Sixteen patients with a mean age of 45.3 years (range 16–78 years) and a mean follow-up of 79.9 months (range 25–264 months) were examined. Symptomatic lesions were noted in 13 patients: intracranial hemorrhage (ICH) in 9, seizure in 1, and headache in 3. A high-flow shunt was observed on angiography in 13 patients. Among these 13 patients, 12 patients were symptomatic. Nine patients presenting with ICH underwent hematoma removal with additional Gamma Knife surgery (GKS; n = 4), GKS only (n = 2), or conservative treatment (n = 3). The 3 asymptomatic patients received conservative treatment, and 1 rebleeding episode was observed. Seven of 8 patients who underwent GKS as an initial or secondary treatment modality experienced a marked reduction in the AV shunt on follow-up angiography, but complete obliteration was not observed.

CONCLUSIONS

Poor lesion localization makes a vp-AVM challenging to treat. Symptomatic patients with a high-flow shunt are supposedly best treated with GKS, despite the fact that only 87.5% of the vp-AVMs treated this way showed a reduction in the malformation volume, and none were cured.