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Case Reports
. 2018 Oct 9:11:14-17.
doi: 10.1016/j.ebcr.2018.09.008. eCollection 2019.

Down syndrome associated moyamoya may worsen epilepsy control and can benefit from surgical revascularization

Sarah R Garson  1 , Stephen J Monteith  2 , Sheila D Smith  3 , Bart P Keogh  4 , Ryder P Gwinn  2   3 , Michael J Doherty  1   3
Affiliations
Case Reports

Down syndrome associated moyamoya may worsen epilepsy control and can benefit from surgical revascularization

Sarah R Garson et al. Epilepsy Behav Case Rep. .

Abstract

Objectives: To examine outcome of bilateral extracranial to intracranial (EC-IC) bypass surgeries for a Down syndrome patient with hard-to-treat epilepsy and moyamoya.

Materials and methods: Superficial temporal arteries were anastamosed using an indirect bypass technique to middle cerebral arteries bilaterally to help limit perfusion deficits and seizure controls.

Results: Two superficial temporal to middle cerebral artery indirect bypass surgeries were performed within 3 months. Post-revascularization improvements included seizure control, gait, perfusion, wakefulness, language and quality of life.

Conclusion: In patients with Down syndrome and moyamoya, improvements in seizure control and quality of life may occur with EC-IC bypass procedures.

Keywords: Bypass; Perfusion; Stenosis.

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Figures

Fig. 1
Fig. 1
Cerebral angiography with sequential preoperative lateral views of internal left internal carotid artery show narrowing beyond ICA terminus and minimal filling of what should be middle and anterior cerebral artery territories.
Fig. 2
Fig. 2
Axial T2 MRI shows an absence of ischemic deficits (A). Representative images from parametric mapping of Tmax (transit time) perfusion imaging results before (B) and after (C) reperfusion, showing interval decrease in hypoperfused brain parenchyma. Automated quantification of perfusion imaging (RAPID) gives a reduction in tissue meeting threshold for hypoperfusion (Tmax > 6 s) from 150.7 cm3 to 14 cm3 (whole brain). Y-axis color units displayed are perfusion times in seconds.
See this image and copyright information in PMC

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