Abstract Details

Title Atypical presentation of Neurologic complications of Osler-Weber-Rendu Syndrome: A case series

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 1-004

Objective Understanding the variety of neurological manifestation in patients with Osler-Weber-Rendu syndrome (OWRS) is crucial to determine appropriate management and prevention of complications.

Background

OWRS is an autosomal dominant genetic disorder, characterized by the presence of telangiectasias and arteriovenous malformations (AVMs) in different vascular systems. Management requires a multitargeted approach for both acute and preventive interventions.

Design/Methods We describe a series of five patients with different neurologic manifestations of OWRS evaluated at our institution from January 2017 to September 2018. Ischemic and hemorrhagic strokes were confirmed with imaging studies in all cases. Therapeutic approaches included intravenous tissue plasminogen activator (IV t-PA), cerebral angiogram with embolization and aneurysm clipping.

Results Two patients presented with ischemic strokes, and two with transient ischemic attacks (TIA). In addition, we identified multiple intracranial brain aneurysms. Acute treatment included IV t-PA for ischemic stroke and management of complications, like brain abscesses. Endovascular embolization of pulmonary AVMs and aneurysm clipping were also performed to prevent complications.

Conclusions OWRS most commonly presents as recurrent epistaxis, and gastrointestinal bleeds accompanied with ferropenic anemia. Neurological manifestations can be primary, like brain AVMs, or secondary to embolic events from extracranial sources, such as ischemic strokes and brain abscess. In our series, only two patients had a known OWRS diagnosis. Three of them presented as stroke code activations and the most frequent symptoms were headache (66%), hemiparesis (100%) and dysarthria (33%). Two patients had ischemic strokes, one of which developed hemorrhagic conversion and multiple frontal brain abscesses. Two patients presented with TIA, and one with multiple cerebral aneurysms on surveillance imaging. Pulmonary AVMs were confirmed in four cases and brain aneurysms in one. OWRS is a heterogeneous disorder with multiple neurologic manifestations. Surveillance and early identification are essential in avoiding potentially fatal complications, such as pulmonary AVMs and primary neurological manifestations.

Authors/Disclosures
Faddi G. Saleh Velez, MD (University of oklahoma health Sciences center) PRESENTER	Dr. Saleh Velez has nothing to disclose.
Laura Dresser, MD (MS & Neuromuscular Center of Excellence)	Dr. Dresser has nothing to disclose.
Jorge G. Ortiz-Garcia, MD, FAAN (The University of Oklahoma Health Sciences Center)	Dr. Ortiz-Garcia has nothing to disclose.

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