The Role of Dual Antiplatelet Therapy (DAPT) vs Surgery in a Case of Moyamoya Disease: A Case Report and Review of the Literature

Abstract

Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by non-atherosclerotic and non-inflammatory progressive narrowing of the intracranial part of the carotid artery and its proximal branches. The disease process is commonly associated with the development of weak, dilated collateral blood vessels at the base of the brain. This gives it a classic smoky appearance on cerebral angiograms and hence the name “Moyamoya” which means “puff of smoke” in Japanese. When a patient has similar vasculopathy in the setting of another disease then it is known as Moyamoya syndrome (MMS). The associated diseases are sickle cell anemia, neurofibromatosis, long-standing diabetes, uncontrolled hypertension, or chemotherapy. Despite being known as a disease of the East Asian population, the disease is no longer exclusive to Asians, as evidenced by the rising incidence among non-Asian groups such as Caucasians, Hispanics, and African Americans. Patients can remain asymptomatic or present with ischemic or hemorrhagic stroke, headache, seizures, or recurrent transient ischemic attacks. Conventional cerebral angiography is considered the gold standard for diagnosing MMD. Treatment may be supportive, medical, or surgical. We present the case of a 42-year-old African American woman with several comorbidities who presented with sudden onset of ischemic stroke and upon further workup was found to have MMD. Equally important is to identify the most effective therapeutic approaches based on individual patients to achieve better clinical outcomes. Our case report highlights the importance of surgery in symptomatic MMD with a lack of supporting evidence indicating the benefits of dual antiplatelet therapy (DAPT).

Is medical management useful in Moyamoya disease?

“…Given the amplified risk of intracranial hemorrhage, such a regimen might be unsuitable for patients with MMD, even in scenarios where a single regimen proves ineffectual. Nonetheless, there have been documented instances wherein a dual antiplatelet regimen was implemented for patients who either refused revascularization surgery or were awaiting the procedure[ 39 , 40 ]. The most recent Japanese management guidelines for MMD advocate for the employment of antiplatelet therapy as a secondary preventive measure against cerebral infarction, albeit with a grade C recommendation level, signifying a potential consideration in the absence of substantial scientific justification[ 41 , 42 ].…”