Treatment of Susac syndrome with gamma globulin and corticosteroids,☆☆

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Abstract

Susac syndrome is a rare vasculopathy characterized by visual, hearing, and cognitive dysfunction. Optimal treatment is unknown, but many patients require chemotherapy to control disease activity. We describe two patients with Susac syndrome and their response to intravenous immune globulin (IVIg) and corticosteroids. Both patients improved following acute treatment with IVIg and intravenous methylprednisolone (IVMP), and no further relapses were observed. One patient showed significant improvement in hearing and MRI lesions shortly following acute treatment. Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome.

Introduction

Susac syndrome, or retinocochleocerebral vasculopathy, is a rare, idiopathic disorder manifest by encephalopathy, visual loss, and hearing loss usually affecting women [1]. Despite aggressive treatments, patients are frequently left with significant residual cognitive, visual, hearing, and vestibular deficits. Histopathologic studies show periarteriolar inflammatory changes [2], suggesting a mechanism involving immune-mediated vascular occlusion.

Reported treatments for Susac syndrome include corticosteroids, cyclophosphamide, azathioprine, and plasmapheresis, with variable results (Table 1). Antithrombotic agents and nimodipine have also been used, aiming to maintain blood flow and prevent vasospasm. Optimal treatment is unknown since less than sixty cases of Susac syndrome are reported in the literature, but many patients require aggressive immunosuppression with cyclophosphamide or azathioprine. Petty et al. suggested corticosteroids as initial treatment, but observed that many patients have inadequate clinical response. Use of intravenous immunoglobulin (IVIg) has been reported in two patients with Susac syndrome, but only in combination with cyclophosphamide [3], [4]. To our knowledge, there are no reports of patients treated with a combination of corticosteroids and IVIg, and few reports have addressed the acute effect of any treatment on hearing loss.

Section snippets

Patient 1

A 35-year-old man presented with headaches and cognitive impairment. Examination was notable for decreased attention, visuospatial deficits, severely impaired memory with confabulation, and gait ataxia. Brain MRI demonstrated multiple non-enhancing cerebral T2-weighted hyperintense lesions and pial enhancement (Fig. 1A,B). CSF studies demonstrated lymphocytes 23/μl, protein 225 mg%, normal IgG index, and negative oligoclonal bands. MRI 1 month later demonstrated new brain lesions, but

Discussion

Optimal long-term treatment of Susac syndrome is unknown. Our two patients showed good response to acute treatment with IVIg and high dose corticosteroids. In patient 1, treatment with IVIg and corticosteroids was associated with immediate and significant improvement in hearing and significant improvement in brain lesions. IVIg and corticosteroids were also associated with no further episodes of visual or auditory dysfunction and no new lesions on brain MRI. Prednisone alone was insufficient

Acknowledgment

We thank Dr. John Susac for helpful discussions regarding the patients and the manuscript.

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Supported by a NIH K23 NS 47211 (Dr. Fox) and The E.A. Baker Foundation/The Canadian National Institute for the Blind (Dr. Costello).

☆☆

Authors' note: Both cases reported here were part of a paper describing MRI characteristics of 27 patients with Susac syndrome (JO Susac, et al., Neurology 2003; 61:1783–7). The 2003 report focused on the MRI characteristics at presentation and neither addressed the effects of treatment nor included any figures from the patients described in the current manuscript.

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