Which syndrome presents with port-wine stains in the trigeminal distribution and leptomeningeal angiomatosis leading to seizures?

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Multiple Choice

Which syndrome presents with port-wine stains in the trigeminal distribution and leptomeningeal angiomatosis leading to seizures?

This item tests recognizing a neurocutaneous syndrome where a facial port-wine stain in the trigeminal distribution signals underlying brain vascular malformations that commonly cause seizures. Sturge-Weber syndrome fits perfectly: a port-wine stain on the face, typically in the V1 (ophthalmic) area of the trigeminal distribution, along with leptomeningeal angiomatosis on the same side. These brain vascular lesions lead to cortical ischemia, calcifications, and seizures, often with progressive neurological changes. Imaging classically shows tram-track calcifications and leptomeningeal enhancement. The other options don’t match this pattern. NF1 features multiple neurocutaneous signs such as café-au-lait macules and neurofibromas, not the characteristic facial port-wine stain with leptomeningeal angiomatosis. Dandy-Walker syndrome is a posterior fossa malformation with an enlarged fourth ventricle and cerebellar vermis hypoplasia. Chiari malformation involves downward displacement of hindbrain structures and does not present with a facial port-wine stain or brain leptomeningeal angiomas.

This item tests recognizing a neurocutaneous syndrome where a facial port-wine stain in the trigeminal distribution signals underlying brain vascular malformations that commonly cause seizures. Sturge-Weber syndrome fits perfectly: a port-wine stain on the face, typically in the V1 (ophthalmic) area of the trigeminal distribution, along with leptomeningeal angiomatosis on the same side. These brain vascular lesions lead to cortical ischemia, calcifications, and seizures, often with progressive neurological changes. Imaging classically shows tram-track calcifications and leptomeningeal enhancement.

The other options don’t match this pattern. NF1 features multiple neurocutaneous signs such as café-au-lait macules and neurofibromas, not the characteristic facial port-wine stain with leptomeningeal angiomatosis. Dandy-Walker syndrome is a posterior fossa malformation with an enlarged fourth ventricle and cerebellar vermis hypoplasia. Chiari malformation involves downward displacement of hindbrain structures and does not present with a facial port-wine stain or brain leptomeningeal angiomas.

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