Link of Video Abstract: https://youtu.be/DWVi34rPNOg
Background: Moyamoya disease (MMD) is a non-atherosclerotic cerebrovascular disorder characterized by progressive stenosis of the distal part of the internal carotid artery (ICA), thus forming an abnormal vascular network in the base of the brain. MMD is often found in women. The common symptoms of MMD are migraine, epilepsy, recurrent transient ischemic attack (TIA), ischemic stroke, and intracerebral hemorrhage. Stroke at young onset has challenges in looking for risk factors and etiology. A detailed examination is needed to prevent recurrent strokes.
Case Presentation: Male 17-year-old complained of a throbbing headache on the right side for 5 months, with a numeric rating scale (NRS) 4. The headache duration was 24-72 hours, with a frequency of 1-2 times a month. Complaints are exacerbated by activity and improve when taking over medication. 2 months after the first onset, he felt severe headache with NRS 7, accompanied by weakness and tingling on the left side. All his laboratory results were within normal limits. Cerebral Digital Subtraction Angiography (DSA) results showed severe stenosis at the base of the right ICA bifurcation segment accompanied by slow flow at the right middle cerebral artery (MCA) and severe stenosis at the base of the left ICA accompanied by cross-filling to the contralateral anterior cerebral artery (ACA). Cortico-cortical collaterals were obtained from the posterior cerebral artery (PCA) branch covering the MCA area. The patient was diagnosed with MMD.
Conclusion: MMD is a rare cerebrovascular disease that can be a risk factor for stroke at a young age. Diagnosis and evaluation of the collateral system need to be carried out through cerebral angiography so that the selection and consideration of appropriate management of recurrent stroke attacks can be carried out.