Abstract Number ‐ 216: Limb‐Shaking Transient Ischemic Attack in the Setting of Moyamoya Disease

Abstract

Introduction Moyamoya disease (MMD) is a cerebrovascular disorder characterized by progressive narrowing of the large intracranial arteries, primarily the internal carotid artery (ICA) and its branches, with secondary development of prominent small‐vessel collaterals. Limb shaking is a rare manifestation of transient ischemic attack (TIA) that is characterized by brief, irregular, involuntary jerking of the arms or legs and momentary disorientation without complete loss of consciousness. It is mainly associated with high grade carotid stenosis. In rare cases, limb‐shaking TIA can occur in the setting of MMD. Methods The patient was identified in routine clinical practice. Results A woman in her 50s presented with a six‐year history of progressive weakness below the neck when standing and initiating movement. These episodes were followed by violent shaking of her arms and legs that lasted several seconds to minutes. She reported transient confusion after the events, but no seizure activity was seen on multiple EEGs, and anti‐epileptic drugs (AEDs) failed to alleviate her symptoms. Further neurological workup included a MR angiogram of the brain that was suggestive of MMD. Catheter angiography confirmed bilateral occlusion of the M1 segments of the middle cerebral arteries (MCAs) and the A1 segment of the left anterior cerebral artery (ACA). There was neovascularization in the same territories with significant collateral flow from the posterior circulation. She was diagnosed with bilateral limb‐shaking TIA in the setting of MMD. She recently underwent two revascularization procedures, a left superficial temporal artery (STA) to middle cerebral artery (MCA) bypass and a right sided encephaloduroarteriosynangiosis (EDAS). Conclusions Limb‐shaking TIA is predominantly associated with severe atherosclerotic extracranial carotid artery stenosis. In rare cases, the limb‐shaking episodes are caused by cerebral hypoperfusion secondary to severe stenosis of the ICAs, and their branches, in the setting of moyamoya disease. Treatment of limb‐shaking TIA to prevent further brain ischemia includes adjustment of fluid intake, careful blood pressure management to avoid episodes of relative brain hypoperfusion, and revascularization procedures. Revascularization is also the most effective method of treating MMD and patients will often experience improvement of their symptoms within one year. Early recognition of limb‐shaking TIA in MMD is critical to avoid unnecessary and potentially harmful treatments (e.g. AEDs), provide patients with accurate explanations of their symptoms, and ensure timely administration of the appropriate treatments.