Abstract Details

Title Sturge-Weber Syndrome and Head Trauma: Abnormal Cerebrovascular Hemodynamics

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P12 - Poster Session 12 (12:00 PM-1:00 PM)

Poster/Presentation
Number 4-005

Objective

To describe the clinical characteristics of a patient with Sturge-Weber Syndrome (SWS) who developed neurological deficits following head trauma, to review similar cases available in the literature and to explore the underlying pathophysiology.

Background

SWS is a neurocutaneous disorder characterized by angiomas involving the face, choroid vessels and leptomeninges. Head injury in SWS has been associated with worsening neurological symptoms. It is postulated that in SWS abnormal superficial cortical veins with persistent high flow lead to engorgement of deep cortical veins causing venous hypertension resulting in vascular stasis causing cerebral atrophy, gliosis and gyral calcification. The changes in cerebral hemodynamics in SWS and head trauma are explored.

Design/Methods The clinical presentation and radiographic findings of a patient are described. A descriptive literature review with 6 additional cases was conducted.

Results A 13-year-old boy presented after sustaining a minor head trauma earlier that day and later developed fluctuating mental status, emesis and unsteady gait. Further evaluation revealed aphasia and right-side hemiparesis. A contrast enhanced brain MRI showed abnormal leptomeningeal enhancement of left cerebral hemisphere which led to diagnosis of SWS. He did not have any associated features such as facial angioma, glaucoma or other prior neurological symptoms. He eventually developed refractory focal and bilateral convulsive status epilepticus. Six cases with SWS who developed either new or worsening neurological findings following head trauma are reported.

Conclusions Our patient was diagnosed with a rare subtype of SWS (type III) with exclusive leptomeningeal angiomatosis. Similar to prior published cases, minor head trauma preceded the occurrence of neurological symptoms. Our case and literature review demonstrate head trauma can unmask neurological manifestations of SWS by potentially compromising the already vulnerable cerebrovascular hemodynamics, however the exact mechanism needs to be further elucidated.

Authors/Disclosures
Jeetendra Sah, MD (University of Louisville) PRESENTER	No disclosure on file
Jeetendra Sah, MD (University of Louisville)	No disclosure on file
Yoshimi Hisamoto, MD (University of Rochester Medical Center)	Dr. Hisamoto has nothing to disclose.
	No disclosure on file
Steven G. Pavlakis, MD, F�鶹��ýӳ��	Dr. Pavlakis has nothing to disclose.

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