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This report further delineates the clinical and molecular repertoire of the POGZ-related phenotype, adding a novel patient with uncommon clinical and behavioral features and provides the first computer-aided facial study of WHSUS patients. © 2020 Wiley Periodicals, Inc.In 1989, Louis Caplan (1) first used the term branch atheromatous disease (BAD) to indicate an occlusion or stenosis at the origin of a deep penetrating artery of the brain, associated with a microatheroma or a junctional plaque, causing an internal capsule or pontine sm