Recurrent Stroke in Young Adult as a Manifestation of Moyamoya Disease: Case Report

Abstract

Background: Moyamoya disease is a chronic cerebrovascular disorder characterized by progressive narrowing of the intracranial portions of the distal internal carotid artery, the initial proximal components of the middle cerebral artery, anterior cerebral artery, and posterior cerebral artery. Moyamoya disease can manifest as a stroke in young adult. Methods: This report presents a young female patient with Moyamoya disease in Siloam Hospital Denpasar, Bali, Indonesia, diagnosed using MRI and Digital Subtraction Angiography. Case Report: A 29-year-old woman with recurrent stroke, presented with right-side hemiparesis, urinary incontinence, disinhibition syndrome, hypertension, and a history of left hemiparesis from a previous stroke. The MRI brain showed hyperintensity throughout the sulci (ivy sign) in the bilateral frontoparietal with acute infarct in the left frontomedial region, chronic infarct in right frontomedial, occlusion of bilateral carotid arteries (supraclinoid segment), bilateral MCA and ACA. The cerebral DSA study showed stenosis in the right ICA, stenosis in the M1-M2 junction with poor collateral to ACA territory with a puff of smoke appearance, occlusion in the left ICA (supraclinoid segment), and severe stenosis in M1. The patient had undergone direct bypass in the right MCA and right superficial temporal artery. Unfortunately, the bypass did not succeed because of infection of the bypass site and the patient got oral medication with a single antiplatelet (asetosal) and angiotensin receptor blocker (valsartan). Discussion: Moyamoya disease is a rare chronic cerebrovascular disorder that can manifest as an ischaemic stroke in young adult. This patient had a recurrent ischaemic stroke due to Moyamoya disease. According to a global multicenter stroke database, 90% of Moyamoya disease patients initially presented with an ischemic stroke, 7.5% with a transient ischemic attack, and 2.5% with a hemorrhagic stroke. DSA showed that the intracranial collateral system failed to fulfill the demand for brain circulation when the stroke occurred. The patient had failure to grow collateral after direct bypass surgery from the right superficial temporal artery to MCA and single oral antiplatelet as a secondary prevention for ischaemic stroke. Conclusion: Moyamoya disease is a rare disease that needs further studies for its treatment to halt the progression and prevent secondary complications. Young patients with ischaemic stroke needed to be asses with brain MRI, MRA, and DSA to evaluate the etiology of ischaemic stroke. Keywords: Moyamoya disease, Digital Substraction Angiography,ischaemic stroke