Successful multiple burr hole openings for limb-shaking transient ischemic attack due to moyamoya disease: illustrative case

Abstract

Background: Limb-shaking transient ischemic attacks (LS-TIAs) are a rare form of TIAs that present as involuntary movements of the limbs and indicate severe cerebral hypoperfusion. LS-TIAs are often reported in patients with carotid artery stenosis but can also affect patients with intracranial artery stenosis and moyamoya disease (MMD).

Observations: A 72-year-old woman presented with repeated episodes of involuntary shaking movements of the right upper limb. Cerebral angiography revealed complete occlusion of the M1 segment of the left middle cerebral artery (MCA), and the left hemisphere was supplied by moyamoya vessels. She was treated with left direct revascularization without complications, and her involuntary movements subsided. However, she demonstrated involuntary shaking movements of the right lower limb 2 months postoperatively. Cerebral angiography revealed complete occlusion of the A1 segment of the left anterior cerebral artery (ACA). The multiple burr hole opening (MBHO) procedure was performed to improve perfusion in the left ACA territory and after 3 months, the patient's symptoms resolved.

Lessons: This case demonstrated that LS-TIAs can also develop as ischemic symptoms due to MMD. Moreover, instances of LS-TIA of the upper and lower limbs developed separately in the same patient. The patient's symptoms improved with direct revascularization and MBHO.

Keywords: ACA = anterior cerebral artery; CEA = carotid endarterectomy; DSA = digital subtraction angiography; EDAS = encephalo-duro-arterio-synangiosis; EDMAPS = encephalo-duro-myo-arterio-pericranial synangiosis; LS-TIA = limb-shaking transient ischemic attacks; MBHO = multiple burr hole opening; MCA = middle cerebral artery; MMD = moyamoya disease; MRA = magnetic resonance angiography; MRI = MR imaging; SPECT = single photon emission computed tomography; STA = superficial temporal artery; TIA = transient ischemic attack; limb-shaking; moyamoya disease; multiple burr hole opening.

FIG. 1.

A: Preoperative fluid-attenuated inversion-recovery MRI shows old infarctions in the bilateral deep white matter. B: Preoperative common carotid artery angiography (frontal view) reveals occlusion of the left M1 segment and moyamoya-like vessels. C: Preoperative external carotid artery angiography (lateral view). D: MRA shows good bypass patency (white arrow) on the next day of surgery. E: Postoperative common carotid artery angiography (frontal view) shows complete occlusion of the A1 segment of the left ACA and good patency of the STA-MCA graft and EDMAPS. F: External carotid artery angiography (lateral view) shows good patency of the STA-MCA graft (black arrow) and EDMAPS.

FIG. 2.

SPECT before and after cerebral revascularization. A: SPECT before STA-MCA bypass and EDMAPS. B: SPECT after STA-MCA bypass and EDMAPS and before MBHO. White arrow indicates reduced cerebral vasoreactivity in the ACA territory compared with panel A. C: SPECT after MBHO. White arrowhead indicates improved cerebral vasoreactivity in the ACA territory compared with panel B. Rainbow displaying cerebral blood flow from 0 to 60 mL/100 g per minute appears on the right.

FIG. 3.

A: Sagittal view of post-MBHO. Four burr holes are pierced in the left frontal region. B and C: A follow-up DSA 10 months after MBHO revealing blood flow through multiple burr holes to the left ACA territory from both the left (B, frontal view; C, lateral view) external carotid arteries (black arrows). No blood flow in ACA territory in Fig. 1E compared with positive blood flow in Fig. 3B.