Which syndrome is indicated by a port-wine stain in the V1 distribution, along with intellectual disability and seizures?

The presence of a port-wine stain in the V1 distribution, coupled with intellectual disability and seizures, is characteristic of Sturge-Weber syndrome. This condition is a neurocutaneous disorder that arises from a vascular malformation due to abnormalities in the development of the mesodermal derivatives, leading to facial capillary malformations, typically seen as port-wine stains, and neurological involvement.

In Sturge-Weber syndrome, the port-wine stain often correlates with underlying leptomeningeal angiomatosis, resulting in the aforementioned neurological symptoms, including seizures and varying degrees of intellectual disability. The neurological implications arise from the presence of abnormal blood vessels in the brain that can create a chronic vascular insult, leading to cortical atrophy, seizures, and developmental delay.

Other syndromes described in the choices do have cutaneous or neurological manifestations but do not specifically correlate with the combination of features provided. For instance, neurofibromatosis type 1 primarily includes neurofibromas and café-au-lait spots, and does not prominently feature the classic port-wine stain. Klippel-Trenaunay syndrome involves capillary malformations primarily affecting the limbs or trunk, and Asherman's syndrome relates to intrauterine adhesions with no direct