In what syndrome is the presence of a "starry sky" appearance in the brain tissue observed?

The "starry sky" appearance in brain tissue is characteristic of Sturge-Weber syndrome. This condition is a neurocutaneous syndrome caused by a congenital vascular malformation, specifically a leptomeningeal angiomatosis that can lead to abnormalities in the brain. In Sturge-Weber syndrome, the presence of abnormal blood vessel formation and associated gray matter changes results in a distinctive histopathological finding, where sections of brain tissue may demonstrate a starry sky pattern due to the accumulation of activated microglia and hemosiderin-laden macrophages around the blood vessels. This appearance can be observed in neuroimaging studies and histological examinations, contributing to the diagnostic criteria for the syndrome.

The other syndromes listed do not feature this specific histopathological finding. For instance, lissencephaly refers to a smooth brain surface without the usual folds or grooves, which is quite distinct from the vascular findings seen in Sturge-Weber. Neurofibromatosis can involve various neurogenic tumors and skin findings, but does not show the starry sky appearance. Rett syndrome primarily affects neurodevelopment and leads to regression in development, but does not have this specific pattern associated with it. Thus, Sturge-Weber syndrome is uniquely identified by