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Background: Limb-shaking transient ischemic attack (LSTIA) is a rare neurological condition which presents with involuntary jerky movements of the arm or leg, often because of carotid stenosis or occlusion. Due to the rarity of the condition, the epidemiology of LSTIA is poorly understood and the disease is frequently misdiagnosed. There is no standard treatment to date. The purpose of this study is to provide an overview of the epidemiology of LSTIA and its current treatment options. Methods: Embase, MEDLINE, Web of Science, Cochrane Central Register of Controlled Trials for randomized controlled trials and Google Scholar were searched from database inception to 30th of December 2023 for articles containing information on the epidemiology and treatment of LSTIA. An individual patient data meta-analysis (IPD-MA) was performed using data extracted from the included articles. Inclusion criteria were description of both the epidemiology and treatment of LSTIA in patients over the age of eighteen with carotid stenosis/occlusion, confirmed by radiographic imaging. Exclusion criteria were studies focusing on pediatrics, no epidemiological data, internal carotid artery (ICA) stenosis/occlusion not radiologically confirmed, full-text unavailable, full-text not in English or Dutch, and non-original articles. Results: Of the 8855 articles screened, 55 articles containing 251 patients were included. 50 articles harboring 81 patients were included in the IPD-MA and 7 articles harboring 187 patients were included in the cohort analysis. The results of the IPD-MA showed that LSTIA was caused by unilateral ICA stenosis/occlusion in 29 patients (36%) and most often from bilateral ICA stenosis/occlusion in 52 patients (64%). Limb-shaking was unilateral in 66 patients (83%) and was accompanied by weakness in 27 patients (33%). The intervention with the highest success rate was endovascular intervention (carotid stenting or balloon angioplasty), as all 10 patients remained asymptomatic after treatment. The cohort analysis showed that LSTIA can be caused by both unilateral and bilateral carotid stenosis or occlusion. The prevalence within cohorts of TIA patients of LSTIA varied considerably from 3.5% to 29%. Conclusion: A large international clinical registry is warranted to gain a better understanding of the epidemiology of LSTIA. There is insufficient evidence available to suggest a standard treatment.

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