Abstract Details

Title Granulomatous Angiitis manifesting with Multifocal Strokes and Intracranial Hypertension: A Case Report

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) Neuro-ophthalmology/Neuro-otology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 017

Objective To describe uncommon manifestations of granulomatous angiitis – multifocal strokes along with papilledema.

Background Granulomatous angiitis (GA) is characterized by vessel inflammation with deposition of giant and epithelioid cells in the walls of cerebral vessels ranging from small leptomeningeal veins to large cerebral arteries. Headache, hemiparesis, encephalopathy, abnormal cerebrospinal fluid (CSF) protein content, and pleocytosis are suggestive features that warrant brain and leptomeningeal biopsy for confirmation and subsequent cytotoxic therapy to improve outcome. We present a case of granulomatous angiitis in a patient presenting with encephalopathy, bilateral vision loss, intracranial hypertension, and multifocal strokes.

Design/Methods Case report.

Results 19-year-old male with history of renal focal segmental glomerulosclerosis presented to the hospital with 2-weeks of severe headache, vomiting, blurry vision, diplopia, and encephalopathy. On initial exam, patient was obtunded with bilateral grade IV papilledema, bilateral lateral rectus palsy and reduced visual acuity in the left (OS) 20/200; finger counting in the right eye (OD), concerning for intracranial hypertension. Lumbar puncture demonstrated an elevated opening pressure of 55 cm H2O and lymphocytic pleocytosis (WBC 55). All other serologic and CSF studies for infectious, autoimmune, and hypercoagulable etiologies returned negative except for positive VZV IgG antibody in the serum. Brain MRI revealed mild diffuse leptomeningeal enhancement, multifocal bilateral infarcts, transverse-sigmoid sinus junction stenosis, and an empty sella with no embolic source identified. Worsening eye exam despite treatment with high dose acetazolamide prompted an external ventricular drain placement. Dural and cortical biopsies showed findings suggestive of GA. He received 5 days of high dose methylprednisolone and was discharged on acetazolamide and prednisone taper with mental status back to baseline and vision improving to OS 20/70, OD 20/25.

Conclusions Granulomatous angiitis should be suspected in patients with obscure neurological symptoms in the right setting since it can lead to permanent neurological deficits if not promptly identified and treated.

Authors/Disclosures
Courtney Filliben PRESENTER	Ms. Filliben has nothing to disclose.
Prashant Natteru, MBBS (Mayo Clinic Health System)	Dr. Natteru has nothing to disclose.
Oriana Sanchez, MD	Dr. Sanchez has nothing to disclose.
	No disclosure on file
Manasa Gunturu, MD (University of MS Medical Center)	Dr. Gunturu has nothing to disclose.
Shreyas Gangadhara, MD (University of Mississippi Medical Center)	Dr. Gangadhara has nothing to disclose.
Shashank Shekhar, MBBS	The institution of Dr. Shekhar has received research support from Sponsor Department of Health and Human Services. The institution of Dr. Shekhar has received research support from PockitDx.
Christa O'hana S. Nobleza, MD	Dr. Nobleza has nothing to disclose.

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