Objective:

To present a case of thrombolysis in a patient with stroke with Sturge-Weber syndrome, highlighting clinical outcomes despite the lack of established guidelines for such treatment in this population.

Background:

Sturge weber syndrome (SWS) is a very rare disorder and can present with strokes and stroke-like episodes, acquired hemiparesis, cerebral atrophy and calcifications, seizures, and intellectual disorders. However, the role of IV thrombolysis in SWS patients with stroke is not established.

Design/Methods:

N/A

Results:

A 58-year-old female with SWS, focal epilepsy and migraine developed sudden onset right sided weakness, right facial droop and receptive aphasia with some dysarthria. Last known well was two hours ago. Her initial NIHSS score was 7. CT Head was negative. CTA head showed decreased enhancement throughout the cortical branches of the left ACA and MCA, most likely chronic but no large vessel occlusion. Given her disabling symptoms, she received thrombolysis with Tenecteplase (TNK). MRI revealed right punctuate parietal infarct without any hemorrhage which would not explain patient symptoms, but potential explanation is embolic phenomena which was aborted by IV TNK with small residual infarct. Post TNK NIHSS improved to 1 with residual mild disfluency in speech.  EEG revealed the left hemisphere slowing, most likely due left hemisphere atrophy. Echocardiogram revealed an ejection fraction of 45% and a Patent Foramen Ovale (PFO), with aneurysmal interatrial septum. Etiology was most likely embolic, given the patient had PFO with high-risk features and a high ROPE score. Patient was started on aspirin and statin for secondary prevention.

Conclusions:

In Sturge-Weber syndrome (SWS), stroke or stroke-like episodes often resolve over days to months. Low-dose aspirin is recommended often starting in childhood. But there’s no clear guidance on thrombolysis in acute settings given the rarity of the condition. Decisions on thrombolysis should be individualized, weighing symptom severity against hemorrhage risks.