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Susac’s Syndrome – Case 1



HomePediatric Ophthalmology and StrabismusUveitis in the Pediatric Age Group
Title: Susac’s syndrome
Author(s): Russell Swan MD, Rachael Jacoby MD
Date: 04/02/2015
Secondary CORE Category: Neuro-ophthalmology / Causes of Decreased Vision / Vascular Disorders
Keywords / Main Subjects: Susac’s syndrome; Branch retinal arterial occlusion
Diagnosis: Susac’s syndrome
Differential: Vasculitis, Churg Strauss, SLE, Sarcoid, Behcets, Eales, Lyme, Syphilis, TB, viral encephalitis, primary CNS lymphoma, MELAS, isolated BRAO
Brief Description: A twenty-seven year old female presents with one week of blurry vision in left eye. She has also had two weeks of dizziness and confusion; she presented to an outside hospital with inability to perform activities of daily living and not oriented to date. She was then transferred for further care at our facility. MRI showed multiple subcortical lesions in multiple vascular territories most noticeably in the corpus collosum.
Our patient was treated with Cellcept and high dose steroids; at her follow up visit she was on Prednisone 40 mg per day and 1000 mg of Cellcept. She will be followed by neurology with serial MRIs and ophthalmology with repeat wide field fluorescein angiogram to monitor for recurrent vascular occlusions.
Further systemic evaluation included:

Labs: normal CBC, CMP, negative cardiolipin antibody, normal CRP, normal A1C, normal lupus anticoagulant, negative Factor V, Protein C/s, antithrombin III, normal homocysteine, ACE, ANCA, SSA/SSB, ANA, RF, ESR;
Lumbar puncture: normal, no oligoclonal bands.

Summary of the Case: Susac’s syndrome is an autoimmune disease first described in 1979 by Dr. John Susac. Most often occurs in females between the ages of 20 to 40 with a 3:1 female to male ratio. Susac’s syndrome typically causes visual field changes, hearing loss and vertigo, headaches with associated vomiting, confusion and cognitive difficulties. The pathophysiology is unknown at this time. Treatment includes steroids, immune modulating treatments such as cyclophosphamide, mycophenaloate, azathioprine and newer biologics, treatment can also include IVIG. The disease process is usually self-limited, lasting between 2 -4 years. The prognosis is varied, as some patients do quite well with limited treatment while others might have a recurrent disease process. Some patients develop long term cognitive deficits, gait disturbances as well as hearing and vision loss.
References:
• Egan, R., Gass, J. et al. Retinal arterial wall plaques in Susac Syndrome. American Journal Ophthalmology 2003, April 135 (4) 483-486
• Susac, J. Susac’s Syndrome. American Journal of Neuroradiology 2004, 25:352-352
Identifier: Moran_CORE_314
Images:

Susac - right

Fundus photos of right eye are normal, left eye shows retinal whitening in the distribution of the superotemporal artery

fa_susec_left_artery_02

Early phase of the fluorescein angiogram in the left eye shows delayed arterial filling in the superotemporal artery

fa_susec_right_artery_02

Follow up wide field FA three months later shows continued delayed arterial filling in the superotemporal artery branch

fa_susac_angiogram_04

Late phase of the follow up angiogram showing vessel wall staining in the inferotemporal vessels.

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