Stroke with hereditary cerebral small vessel diseases is a rare disease. Its clinical manifestations include early-onset ischemic lacunar or hemorrhagic stroke with high disability. Its typical imaging markers include lacunes, white matter hyperintensities, microbleeds, intracerebral hemorrhages located in deep or lobe of brain, crotical microinfarcts, and enlarged perivascular spaces. As the clinical and neuroimaging signs and symptoms of hereditary cerebral small vessel diseases often overlap with sporadic cerebral small vessel diseases, it is hard to diagnose. This article summarizes the clinical features, importance of obtaining valuable family history, genetic diagnosis, and management of stroke with hereditary cerebral small vessel disease to improve its accuracy diagnosis.
