Neurocutaneous syndromes: Sturge-Weber syndrome: practice question

A Dermatology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Dermatology · Neurocutaneous syndromes: Sturge-Weber syndrome

Sample item

A newborn has a unilateral port-wine stain over the forehead and upper eyelid. At 6 months she has focal seizures. What additional complication should be screened for?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

C. Glaucoma and leptomeningeal angioma

Sturge-Weber syndrome presents with a port-wine stain in the V1 distribution, a leptomeningeal angioma causing seizures and calcification, and glaucoma. It results from a somatic GNAQ mutation. Ophthalmology and neuroimaging are required.

Option by option

  • A. Pheochromocytoma

    Pheochromocytoma is linked to NF1 and von Hippel-Lindau disease.

  • B. Optic glioma

    Optic glioma is characteristic of neurofibromatosis type 1.

  • C. Glaucoma and leptomeningeal angioma (correct)

    Correct. Glaucoma and leptomeningeal angioma are the associated problems.

  • D. Renal angiomyolipoma

    Renal angiomyolipoma is associated with tuberous sclerosis.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this Dermatology question

What is the answer to this Dermatology question?

The best answer is C, Glaucoma and leptomeningeal angioma. Sturge-Weber syndrome presents with a port-wine stain in the V1 distribution, a leptomeningeal angioma causing seizures and calcification, and glaucoma.

Why is "Pheochromocytoma" not the answer?

Pheochromocytoma is linked to NF1 and von Hippel-Lindau disease.

Why is "Optic glioma" not the answer?

Optic glioma is characteristic of neurofibromatosis type 1.

Why is "Renal angiomyolipoma" not the answer?

Renal angiomyolipoma is associated with tuberous sclerosis.

What concept does this question test?

It tests Neurocutaneous syndromes: Sturge-Weber syndrome in Dermatology. It results from a somatic GNAQ mutation. Ophthalmology and neuroimaging are required.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Neurocutaneous syndromes: Sturge-Weber syndrome?

Learn each condition by its primary lesion, site and signature sign. Then use the flashcards and the other Dermatology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.