A total of 1213 patients were enrolled, of whom 145(12%) were diagnosed as AMIMCC. Compared with non-AMIMCC, AMIMCC patients manifested as younger age(P=.016), more often female(P=.001), less common for conventional vascular risk factors(P<.05). The constitution of TOAST classification was significantly different between patients with AMIMCC and non-AMIMCC(P=0.000), with higher proportion of stroke of other determined cause(67.6%vs12.4%).Regarding the detailed etiology of AMIMCC, autoimmune or hematologic diseases were the most common(27.6%), followed by peri-procedural infarcts(15.2%), cardioembolism(13.1%), malignant tumor(11.7%), large artery atherosclerosis (10.3%), sudden drop in blood pressure(8.3%). Hypercoagulability and systemic hypo-perfusion were the common underlying mechanisms of AMIMCC. Distinctive lesion distribution patterns were associated with stroke etiologies and mechanisms in AMIMCC patients. Sixty-five percent of the 20 patients diagnosed with SLE/APS and 52.9% of the 17 patients with malignant tumor tended to manifest as supratentorial infarcts. However, 68.4% of cardioembolism presented with both supratentorial and infratentorial infarct lesions. More than half of patients with SOC and SUC had intracranial or extracranial artery stenosis.