Patients with moyamoya disease presenting with movement disorder

Report of 4 cases

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Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders.

Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed.

The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery–middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1.

Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery–middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.

Abbreviations used in this paper: ACA = anterior cerebral artery; EDAS = encephaloduroarteriosynangiosis; GSD = glycogen storage disease; ICA = internal carotid artery; MCA = middle cerebral artery; STA = superficial temporal artery; TIA = transient ischemic attack; Xe-CT = xenon-enhanced computed tomography; 99mTc-HMPAO = technetium-99m–labeled hexamethylpropyleneamine oxime.

Article Information

Address correspondence to: Gary K. Steinberg, M.D., Ph.D., Department of Neurosurgery, R281, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, California 94305-5325. email: gsteinberg@stanford.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 3. Preoperative MR images revealing basal ganglionic infarct (left) and bilateral frontal subcortical infarct (right).

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    Case 3. Preoperative SPECT scans showing bilateral cortical and subcortical hypoperfusion.

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    Case 3. Postoperative SPECT scans showing normal perfusion.

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    Case 3. Preoperative angiograms showing bilateral supraclinoid ICA occlusion with moyamoya vessels. LICA = left ICA injection; RICA = right ICA injection.

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    Case 3. Postoperative right (a) and left (b) external carotid artery (ECA) injections showing excellent revascularization.

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    Case 2. Images obtained in an 8-year-old girl. a: Axial FLAIR MR image showing bilateral watershed infarcts. b and c: Preoperative right and left ICA injection digital subtraction angiograms, lateral projection, showing bilateral supraclinoid ICA occlusion with moyamoya vessels. d and e: Follow-up right and left ECA digital subtraction angiograms, lateral projection, showing excellent revascularization.

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    Case 4. Images obtained in an 18-year-old man. a: Axial MR image showing no evidence of infarction or ischemia. b and c: Preoperative left and right ICA digital subtraction angiograms, anteroposterior projections, showing left supraclinoid ICA occlusion with moyamoya vessels. d and e: Follow-up left ECA injection digital subtraction angiograms, lateral and anteroposterior, projections showing excellent revascularization.

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