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Seizure: Delayed Presentation of Sturge-Weber
Varamo, V, DO, O’Connell, K, MD
Kent County Hospital, Emergency Medicine Residency, Warwick RI
Introduction: Sturge-Weber syndrome is a sporadically occurring and uncommon
Neurocutaneous syndrome (on the order of 1:50,000 persons) marked by
leptomeningeal angiomas, facial capillary malformations, glaucoma, seizures, and
mental retardation that is normally diagnosed at birth. Early diagnosis is usually possible
due in part to physical findings. Delayed diagnosis may occur in those persons that do
not have facial stigmata in a subtype of the disorder known as Sturge-Weber Type III.
Case: A 16-year-old female presented to the St. Barnabas Pediatric Emergency
Department(ED) with 3 syncopal episodes today. The last one occurred 30 minutes prior
to arrival, witnessed by a friend. In the ED she was initially somnolent, but would follow
all commands and answer questions succinctly. She did not recall any pre-syncope
symptoms of dizziness, chest pain prior to the events. The observed event lasted 30
seconds, and did not result in injury or loss of bowel/bladder function. In the ED she
complained of a mild frontal headache and nasal congestion for the last 5 days.
Abnormal vitals in the ED included a tachycardia of 113. Physical exam was within
normal limits, except for the aforementioned somnolence. Lab studies were significant
for a hemoglobin of 9.8, hematocrit of 31.0, and a leukocytosis of 14.4. A non-contrast
head CT scan was performed which showed gyriform calcifications in the white matter of
the right temporal/parietal lobe that is consistent with Sturge-Weber syndrome. An MRI
performed reaffirmed the working diagnosis. The patient was admitted to the Pediatric
floor where her hospital stay was complicated by witnessed epileptic seizures on
multiple days, and an EEG that confirmed abnormal electrical activity. Optimal
management of the patient’s seizures was finally achieved using 1000mg of
levetiracetam twice a day and phenytoin 300mg once a day. The patient was discharged
from the hospital after 7 days to follow-up.
Discussion: Sturge-Weber is classified into 3 types. Type I, the most common, has the
aforementioned physical findings and leptomeningeal angiomas resulting in mental
retardation and seizures in the majority of those affected. Type II involves the facial
nevus and glaucoma, but has no leptomeningeal involvement. Type III only has
leptomeningeal association. As of 2005 only 24 cases of Type III Sturge Weber have
been reported. Our patient suffered from the rare Type III and presented late in life for
her diagnosis because she had no known seizures prior to her arrival into the ED. This
case reflects the necessity to maintain a wide differential diagnosis when evaluating
syncope and seizures in the pediatric population in the ED.