Clinical Management of Moyamoya Patients

Abstract

Moyamoya angiopathy (MMA) is a peculiar cerebrovascular condition characterized by progressive steno-occlusion of the terminal part of the internal carotid arteries (ICAs) and their proximal branches, associated with the development of a network of fragile collateral vessels at the base of the brain. The diagnosis is essentially made by radiological angiographic techniques. MMA is often idiopathic (moyamoya disease-MMD); conversely, it can be associated with acquired or hereditary conditions (moyamoya Syndrome-MMS); however, the pathophysiology underlying either MMD or MMS has not been fully elucidated to date, and this poor knowledge reflects uncertainties and heterogeneity in patient management. MMD and MMS also have similar clinical expressions, including, above all, ischemic and hemorrhagic strokes, then headaches, seizures, cognitive impairment, and movement disorders. The available treatment strategies are currently shared between idiopathic MMD and MMS, including pharmacological and surgical stroke prevention treatments and symptomatic drugs. No pharmacological treatment able to reverse the progressive disappearance of the ICAs has been found to date in both idiopathic and syndromic cases. Antithrombotic agents are usually prescribed in ischemic MMA, although the coexisting hemorrhagic risk should be considered. Surgical revascularization techniques, which are currently the best available treatment in symptomatic MMA, are associated with good long-term outcomes and reduced ischemic and hemorrhagic risks. Given the lack of dedicated randomized clinical trials, current treatment is mainly based on observational studies and physicians’ and surgeons’ expertise.

Vaso-Occlusive Pain Management in a Patient With Sickle Cell Disease Associated With Moyamoya Syndrome

“…However, as moyamoya patients are at risk for developing both ischemic and hemorrhagic strokes, the use of anticoagulation is often not preferred. The mainstay of treatment includes pharmacotherapy with antithrombotics and revascularization surgery [ 5 ].…”

“…The complications of moyamoya angiopathy, such as seizures and chronic headaches, should also be clinically managed with antiepileptics and analgesics [ 5 ]. Concerning analgesics, oxycodone like many other opioids, leads to hyperpolarization and decreased neuron signaling, causing central nervous system depression.…”

Allergic Reaction Induced Brainstem Stroke in a Patient With Moyamoya Disease: A Case Report

“…The prognosis of moyamoya disease can lead to contradictory results of hemorrhagic or ischemic stroke; however, no methods have been able to predict it yet 3) .…”

“…To date, no pharmacological treatment has been found to reverse the gradual loss in the ICA. Antiplatelet agents are commonly prescribed for ischemic moyamoya disease, although the coexistence of bleeding risk should be considered 3) .…”

Transcranial color‐coded ultrasound for Moyamoya disease—A holistic diagnostic tool?

“…3 Clinicians involved in the care of patients with Moyamoya disease, also need to understand the extent of ischemic and hemorrhagic lesions in the brain, the complex hemodynamics of cerebral circulation in this condition and stage the progression of the disease to tailor interventions accordingly. 4 A summary of the existing imaging techniques available for the evaluation of Moyamoya disease is provided in Table 1. It appears that transcranial color-coded duplex ultrasound (TCCS) provides a simple, inexpensive, real-time, repeatable, bedside measure of many important parameters utilized for both diagnosis and follow up of Moyamoya disease.…”