The natural clinical course of hemodynamically stable adult moyamoya disease

Abstract

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.http://thejns.org/doi/abs/10.3171/2014.9.JNS132281 KEY WORDS asymptomatic; hemorrhage; ischemia; moyamoya disease; clinical course; vascular disorders aBBreViatiOnS KPS = Karnofsky Performance Scale; MMD = moyamoya disease; mRS = modified Rankin Scale; SAH = subarachnoid hemorrhage; SPECT = single photon emission computed tomography; TIA = transient ischemic attack.

Stroke prevention by direct revascularization for patients with adult-onset moyamoya disease presenting with ischemia

“…16,26 In a recent article, Cho et al reviewed publications that have reported stroke recurrence in patients who received nonsurgical treatment for MMD. 4 The authors found that the weighted average of annual stroke rates for a total of 470 patients receiving conservative treatment was 7.1%. However, to our knowledge, nonsurgical treatments have not yet been compared with surgical treatments on such a large scale.…”

“…While the annual stroke rate of nonsurgically treated adult patients with MMD has been reported to be 3.2%-13.3%, 3,4,11,13,15,18,23 the incidence of stroke after direct or combined revascularization surgery appears to be lower. Several retrospective cohort studies have investigated this abbreviatioNs ARR = absolute risk reduction; AS = any kind of stroke; DSA = digital subtraction angiography; D-SPECT = SPECT with acetazolamide (Diamox) challenge; HS = hemorrhagic stroke; IS = ischemic stroke; MMD = moyamoya disease; mRS = modified Rankin Scale; NNT = number needed to treat; OA = occipital artery; RRR = relative risk reduction; SPECT = single photon emission tomography; STA = superficial temporal artery; TIA = transient ischemic attack.…”

Long-Term Outcomes After Combined Revascularization Surgery in Adult Moyamoya Disease

“…The annual risks of symptomatic hemorrhage and infarction were 0.4% and 0.2%, respectively, and the 5-year stroke-free survival rate of hemorrhage and infarction was 98.7% after surgery in this study. In our recent report addressing the natural clinical course in hemodynamically stable patients, 29 the annual stroke risk in adult MMD with an ischemic presentation was 4.2% (3.0% ischemic stroke, 1.2% hemorrhagic stroke), and the 5-year stroke risk was 17%. Considering that the patients surgically treated were more hemodynamically unstable than those who were conservatively treated, the stroke risk in patients recruited in this study would have been higher if they had not undergone surgery.…”

The Efficacy of Surgical Treatment for the Secondary Prevention of Stroke in Symptomatic Moyamoya Disease

“…1 The most common clinical manifestation of MMD is ischemic or hemorrhagic stroke. 2 The diagnosis is confirmed by angiographic examination with typical characteristics, such as stenosis or occlusion at the main branch of the ICA, and an abnormal vascular network at the skull base. 3 Without intervention, MMD often leads to serious neurological defects or even death.…”