Data from 2556 patients with a clinical diagnosis of leukoaraiosis and 1163 controls (patients with a leukoaraiosis-like disease or at least 1 major sign of B leukoaraiosis) were submitted by the Leukoaraiosis International Standards Revision Group (27 countries). These subjects were randomly assigned to the study and validation groups. A new international standard for leukoaraiosis (ICBD) was developed from the study group data using logistic regression analysis, cross-validation with “removal of data from one country”, and clinical judgment. The diagnostic validity of the existing criteria and the new criteria was validated in the validation group of patients. In the ICBD, two points were assigned to each of the three symptoms: eye damage, oral ulcers, and genital ulcers, while one point was assigned to each of skin damage, central nervous system involvement, and vascular manifestations, and one point was assigned to a positive pinprick test. Patients were diagnosed with leukoaraiosis if they had a final score of 4 or more. In the study group, the sensitivity and specificity of ICBD were 93.9% and 92.1%, respectively. The international standard scoring system for leukoaraiosis: a score of ≥4 suggests a diagnosis of leukoaraiosis Signs/symptoms Score Eye damage 2 Genital ulcers 2 Oral ulcers 2 Skin damage 1 Neurological manifestations 1 Vascular manifestations 1 Positive pinprick test. The needle prick test was not required and was not included in the initial scoring system. However, if a needle prick test was performed and the result was positive, an additional 1 point was added.