Limb Shaking Transient Ischemic Attack in Patient with Moyamoya Disease
Jordan Scott1, Gunjanpreet Kaur1, Brian Miremadi1
1St. Louis University School of Medicine
Objective:
N/A
Background:
Limb shaking Transient ischemic attack (LS-TIA) is a rare clinical manifestation characterized by brief, arrhythmic, involuntary jerking of extremities without any associated loss of consciousness. It is most often seen in the setting of severe flow-limiting extracranial carotid stenosis. In rare cases, Moyamoya disease (MMD) can manifest with LS-TIA and/or be an indicator of disease progression.
Design/Methods:
The patient was identified in routine clinical practice. 
Results:
A woman in her late 30s with a known history of MMD presented with subacute onset of involuntary jerking of bilateral arms and legs lasting seconds to minutes. She denied associated loss of consciousness or post-event confusion. Several years prior, she had undergone indirect revascularization with a right-sided encephaloduroarteriosynangiosis (EDAS). Further workup included a Computed tomography angiography which showed findings suggestive of progressive MMD, as well as an Magnetic Resonance Imaging of Brain which was negative for acute pathology. She underwent an Electroencephalogram which was non-diagnostic. Diagnostic catheter cerebral angiography demonstrated bilateral proximal large artery occlusions with associated neovascularization and significant collateral flow from the posterior circulation. The patient was diagnosed with limb‐shaking TIA in the setting of MMD. She was scheduled to undergo direct revascularization with a right superficial temporal artery (STA) to middle cerebral artery (MCA) bypass. 
Conclusions:
 In rare cases, patients with MMD can present LS-TIA due to cerebral hypoperfusion in the setting of severe intracranial arterial steno-occlusive disease. Although there is no curative therapy for patients with this condition, adequate hydration, blood pressure control, and/or revascularization procedures can augment cerebral blood flow and perfusion, while mitigating ischemic manifestations. The prompt recognition of this rare neurological manifestation of limb‐shaking TIA in MMD is essential to avoid misdiagnosis and facilitate timely management.  
10.1212/WNL.0000000000208239