Gass plaques and fluorescein leakage in Susac Syndrome☆,☆☆
Introduction
Susac Syndrome (SS) consists of encephalopathy, branch retinal artery occlusions, and hearing loss and affects women more than men [1], [2]. The complete triad may not be present at initial presentation. Headaches, often migrainous, are usually present including bilateral long tract findings, roaring tinnitus, vertigo, and bizarre personality changes. Magnetic resonance imaging that accompanies the encephalopathic SS patient always shows involvement of the corpus callosum and often strikingly [3]. Any part of the brain may be involved including deep grey matter lesions and leptomeningeal enhancement [3]. This imaging triad has been helpful in establishing the diagnosis at an early stage to allow for early, aggressive treatment.
Two additional and helpful funduscopic findings were recently described in this disorder. The first, the retinal arterial wall plaque (RAWP) now known as GP, originally was described by J. Don Gass in idiopathic branch retinal artery occlusions, now believed to be a form fruste of SS, and in a number of other rare retinal disorders [4], [5], [6]. After our initial description of GP in SS [7], we have found this plaque in a number of SS patients evaluated henceforth.
The second finding is an unusual leakage pattern of arterial wall hyperfluorescence (AWH) on fluorescein angiography (FA), a routine test used for a variety of ophthalmological complaints. Several reports have documented AWH of vessels not necessarily located near the branch retinal artery occlusion (BRAO) that occurs in patients with SS [8], [9]. There is a single report of a young patient who demonstrated AWH five days prior to the onset of a BRAO [10] plus another patient with SS with AWH that was treated with tacrolimus and avoided the onset of BRAO and enjoyed resolution of the AWH [11]. The purpose of this report is to demonstrate these two ophthalmic findings and raise awareness of them to the neurologic practitioner.
Section snippets
Methods
This is a retrospective, multi-center observational study documenting patients with GP and AWH of retinal vessels.
Case 1
A 35 year old woman developed severe headaches, numbness in her fingertips, episodic vision loss, and trouble speaking. Two months after onset she developed permanent partial vision loss in her left eye. Her right fundus appeared normal with the exception of two small yellow refractile GP, one at a retinal arteriolar bifurcation and another more distal to it (Fig. 1A). Her left fundus showed a swollen pallid retina inferior and temporal to the fovea consistent with a BRAO (Fig. 1B). An MRI scan
Discussion
Gass and coworkers were the first to describe multiple yellow GP that may simulate emboli in six of nine patients with idiopathic BRAO and he described them in other disorders that cause focal damage to the arterial wall [4], [5], [6], [12]. Gass attributed GP to atheromatous deposits caused by slow extravasation of blood lipids into the arterial wall at the sites of arterial wall damage. Unlike Fisher platelet–fibrin emboli (gray-white), Hollenhorst cholesterol plaques (orange and refractile),
Acknowledgment
The authors thank Robert Daroff for critical review of the manuscript.
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Susac syndrome with the typical clinical triad: A case report and literature review
2022, Journal of NeuroimmunologyCitation Excerpt :The first clinical presentation of approximately 50% of Susac syndrome patients is visual impairment (Dorr et al., 2013). The characteristic fundus manifestations are BRAO or artery stenosis and small yellowish-white plaques on the artery wall, also known as Gass plaques (Egan et al., 2010). Patients often have irreversible hearing loss on one or both ears, which occurs during or suddenly during the onset of the disease (Gross et al., 2004; Grygiel-Gorniak et al., 2016).
Visual Loss: Retinal Disorders of Neuro-Ophthalmic Interest
2018, Liu, Volpe, and Galetta's Neuro-Ophthalmology: Diagnosis and ManagementCharacteristics of headache in relation to the manifestation of Susac syndrome
2017, Medicina (Lithuania)RETINAL FLUORESCEIN ANGIOGRAPHY in SUSAC SYNDROME: A SHIFTING PATTERN of ARTERIOLAR WALL HYPERFLUORESCENCE - A REPORT of TWO CASES
2023, Retinal Cases and Brief ReportsSUSAC SYNDROME: THE EFFECTIVENESS OF RITUXIMAB MONOTHERAPY
2023, Nauchno-Prakticheskaya Revmatologiya
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This study conformed to the protocol of our Institutional Review Board and did not require approval by our IRB.
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This report is being submitted in conjunction with another report authored by Susac, Rennebohm, Egan, and Daroff titled “Susac Syndrome — Update.”