Furthermore, high expression of Lysyl oxidase-like 2 (LOXL2), a matrix-remodeling enzyme that has already been associated with metastasis in hepatocellular carcinoma (HCC), in peritumoral stroma proved to be an independent prognostic factor of worse OS and DFS in patients with iCCA [91]. Here, LOXL2 is linked to infantile convulsions and choreoathetosis.