Abstract Details

Title Cortical Venous Thrombosis as the Presenting Symptom of Hugh-Stovin Syndrome

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 088

Objective Here we provide a case of Hugh-Stovin Syndrome in a patient who originally presented with cortical venous thrombosis later found to have multiple pulmonary arterial aneurysms with thrombosis.

Background 46yo Japanese woman with migraines who presented with an acute onset of right-sided weakness. After admission she had a generalized tonic-clonic seizure and was started on levetiracetam. EEG was performed and found to be normal. Brain MRI revealed a posterior medial left frontal lobe diffusion restriction and SWI sequences revealed a cortical vein thrombosis proximal to the area of diffusion restriction. Basic laboratory studies and LP was unremarkable. Patient was noted to be anemic and required 1u pRBCs. Due to the unexplained anemia she underwent an EGD and colonoscopy which did not reveal any signs of active bleeding. Biopsy from the colonoscopy revealed multiple rows of superficial polypoid ulcers. Patient later underwent a CT chest, abdomen and pelvis which revealed multiple pulmonary arterial aneurysms with thrombosis. Autoimmune and thrombophilia workup performed was unrevealing. After discharge she was seen by Neurology and Rheumatology and ultimately given the diagnosis of Hugh-Stovin Syndrome (HSS).

Design/Methods NA

Results NA

Conclusions Hugh-Stovin Syndrome (HSS) is an exceedingly rare disorder with less than 40 published cases in the English medical literature. It typically affects the young adult population with a statistical predilection for males. Although no formal diagnostic criteria have been formulated, the diagnosis of HSS is made when both venous thrombosis and multiple pulmonary and/or bronchial aneurysms are present. Here we present a case of a young Japanese woman presenting with a cortical vein thrombosis, who upon further workup, was found to have multiple pulmonary artery aneurysms with thrombosis. Although rare, HSS should be considered in cases of cerebral venous thrombosis in a young otherwise healthy individual with pulmonary symptomatology such as dyspnea, cough or hemoptysis.

Authors/Disclosures
Austin T. Momii, MD PRESENTER	Dr. Momii has nothing to disclose.
Mohammad N. Kayyali, MD	Dr. Kayyali has nothing to disclose.
Jessica Choi, MD (Cedars-Sinai Medical Center)	Dr. Choi has nothing to disclose.
Matthew Burford, MD (Cedars-Sinai Medical Center)	The institution of Dr. Burford has received research support from Brainstorm Cell Therapeutics. The institution of Dr. Burford has received research support from Pharnext Inc.

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