Fluorescene Angiogram Conference Russell Swan

History CC: Blurry vision OS for ~1 week HPI: 27 year old female with ~ 1 week of vision changes in the left eye. Poor historian 2/2 difficulties with cognition – Per mom, mid December 2 weeks of recurrent dizziness and increased confusion. Seen OSH with normal head CT and treated for migraines – : developed nausea, vomiting, HA, and worsening dizziness; – : seen again at OSH ER; not oriented to date, not able to perform ADL’s or take care of children – : Admitted OSH: oriented to person only transferred to U of U for further care and eval

History Cont. PMH: – PFO – Hypothyroid – Migraines – Depression – HLD – GERD – Gestational DM and HTN during 2 nd pregnancy

History Cont. PSH: – Right ankle surgery x2 – Cyst removal from chin FH: – Brother with epilepsy 2/2 hypoxic brain injury as a child – No eye problems SH: – Divorced, 2 children

History Cont. Meds: – Lipitor – Klonipin – Prilosec – Metformin – Topamax – Levothyroxine – Imitrex prn – Mirena IUD – Vortioxetine

What does she have? Differential Ddx? Other testing? Recommendations to primary team?

Differential: Vasculitis, Churg Strauss, SLE, Sarcoid, Behcets, Eales, Lyme, Syphillis, TB, viral encehpalitis, primary CNS lymphoma, MELAS, isolated BRAO

MRI: multiple subcortical lesions in multiple vascular territories (most noticable in corpus collosum), leptomeningeal enhancement Doppler US of legs: no DVT TEE: no vegetation, right to left PFO LDL: 102 CTA: normal without stenosis Cerebral angiogram: no stenosis or vasculitis Labs: nl CBC, CMP, negative cardiolipi ab, normal CRP, A1C, lupus anticoagulant, Factor V, Protein C/S, antithrombin III, homocysteine, ACE, ANCA, SSA/SSB, ANA, Rf, ESR LP: unremarkable

Susac Syndrome Autoimmune disease first described in 1979 by Dr John Susac Epidemiology: Most often affects females between the ages of 20 to 40; 3:1 F:M; ages 8-58 Classic Symptoms: – Brain HA with associated vomiting Confusion, cognitive difficulties Change in personality and psychiatric problems – Eye: Visual field changes – Ear: Hearing loss Vertigo Tinnitus

Clinical Findings Examination: Gass plaques: yellow-white retinal arteriolar wall plaques occurring at the midarteriolar segments (not at bifurcations) MRI Findings: classic is multifocal white matter changes including in the corpus collosum, enhance during the acute stage (70%), letomeningeal enhancement (30%) Cerebral angiogram: usually normal (involved precapillary arterioles are <100um) FA: BRAO and multifocal fluorescence of branch arterioles

Treatment: – Steroids – IMT: cyclophsophamide, mcophenolate, azathioprine, newer biologics – IVIG

Pathophysiology: Unknown to date Prognosis: – Difficult to predict at onset as some doe well with limited treatment while others have a recurrent disease process – Usually self-limited lasting 2-4 years – Unfortunately some patients end up suffering long term congitive deficits, gait disturbances, and hearing loss

3 month follow-up apt Vision stable On Cellcept 1000mg and Prednisone taper, repeat MRI in 4 months

References Egan, R., Gass, J. et al. Retinal arterial wall plaques in Susac Syndrome. American Journal Ophthalmology 2003, April 135 (4) Susac, J. Susac’s Syndrome. American Journal of Neuroradiology 2004, 25: