A 2-Month-Old Girl Who May Have Had a Stroke: Osmosis USMLE Study Question

July 26, 2019

Answer: C. Sturge-Weber syndrome

A seizure is not an uncommon occurrence in children and can happen for a variety of reasons including fever, hypoglycemia, and hyponatremia. This child's seizure would be classified as a simple focal seizure. Focal seizures are unilateral and localize to one part of the brain (in this instance, right leg +/- arm). Simple focal seizures are characterized without any impairment of consciousness or awareness. In this case, the infant is normoglycemic, has no history of fever, and has no history of a gastroenteritis, which might cause hyponatremia from fluid loss. Postictal paralysis can last from minutes to hours following a seizure event, but is temporary.

The diagnosis in this infant is made based on the observation that she has a port-wine stain in the V1 trigeminal nerve distribution. Sturge-Weber syndrome is associated with trigeminal nerve distribution port wine stains of the face, seizures, glaucoma, and developmental delay. Cranial hemangiomas on a person's skin can affect underlying and nearby structures as well. Consider the possibility of meningeal, brain, and eye involvement. Head imaging would reveal a cerebral hemangioma with associated calcifications and underlying brain atrophy, usually in the distribution affected by the infant's seizures.

Major Takeaway: Sturge-Weber syndrome is a type of congenital, noninherited phakomatosis characterized by trigeminal nerve distribution port-wine stains of the face, seizures, glaucoma, and developmental delay.

Read more about Sturge-Weber syndrome.


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