LETTER TO THE EDITOR Aggressive immunosuppression in Susac’s syndrome: 10 years of follow-up E. Capiluppi 1 & L. Romano 1 & S. V. Luccarelli 2 & A. Macerollo 3 & G. Cislaghi 1 Received: 17 March 2018 /Accepted: 20 June 2018 # Springer-Verlag Italia S.r.l., part of Springer Nature 2018 Dear Editor, Susac’s syndrome (SS) is a rare disease affecting small vessels of the brain, inner ear, and retina [1]. The pathophysiology is related to a selective immune mechanism against the endothe- lium [1]. The characteristic clinical triad is acute encephalop- athy, visual field impairment and cochlea-vestibular symp- toms [2]. The common therapeutic mainstay is represented by pharmacological immunosuppression (Supplement 1). Here, we present the long-term outcome of a SS case treated with a specific combination of immunosuppressive drugs. The study has been approved by the local ethics committee and the consent form was obtained from the patient. A 34-year-old Caucasian woman was admitted in August 2008 due to a progressive development of headache, confu- sion, nausea, visual disturbances, weakness, paraesthesia, tin- nitus and cognitive decline. Neurological examination showed mild slowness in voluntary movements and mild truncal ataxia. Cognitive assessment highlighted attention def- icits, dyscalculia, impaired writing and dysexecutive signs. Brain magnetic resonance revealed multiple T2-hyperintense lesions in the supratentorial hemispheric white matter, corpus callosum, basal ganglia and thalami without contrast enhance- ment (Fig. 1a, b). Routine blood tests were normal. Screening for thrombophilic conditions (protein C, protein S, antithrombin III, homocysteine, vitamin B12, and folate) as well as for auto- antibodies (anti-nuclear, anti-ENA, anti-neutrophil cytoplasm, antiphospholipid) was negative. Blood cultures and serological tests for bacterial and viral infections (including HIV, T. pallidum, and B. burgdoferi) were negative as well. EKG, echo- cardiogram, Doppler sonogram of epiaortic arteries, and multi- modal (visual, somatosensory, brainstem auditory) evoked po- tentials were normal. Cerebrospinal fluid examination showed a nonspecific elevation of protein (760 mg/L, normal 150–450) and a mild lymphocytic pleocytosis (10 cells/mmc); oligoclonal IgG bands were absent; serological tests and cultures were neg- ative. Visual field examination demonstrated segmental periph- eral defects in both eyes. A retinal fluorescein angiography evidenced peripheral artery branch occlusions in both eyes (Suppl. Fig. S1). An audiogram revealed low-frequency sensorineural hear- ing loss in the left ear. In the emergency setting, an acute disseminated encepha- lomyelitis was first suspected on the basis of time course and pattern of brain involvement. The patient was treated with high-dose intravenous (IV) methylprednisolone (1 g daily for 5 days) followed by oral prednisone (50 mg/daily). In view of ophthalmological results and brain MRI as well as the selective involvement of brain, retina and ear, the diag- nosis of SS was formulated. Oral salicylate 160 mg, nimodipine 90 mg/day and latanoprost drops were added to the steroids therapy. The neurological features improved dra- matically after steroids. The brain MRI repeated after a week of therapy showed a reduction of the thalamic T2-hyperintensity and a mild dimensional increase of the callosal lesion. Cognitive tests repeated 11 days after the beginning of treatment came back normal. Despite the cognitive and neu- rological improvement, the patient still complained of tinnitus, hearing loss, segmental visual peripheral loss and fluctuating headache. Therefore, IV immunoglobulin (0.4 g/kg/day for 5 days) were started [3]. The brain MRI scan showed a further mild reduction of the cerebral lesions. The retinal fluorescein angiography demonstrated a partial recanalization of the Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10072-018-3481-4) contains supplementary material, which is available to authorized users. * G. Cislaghi giuliana.cislaghi@asst-fbf-sacco.it 1 Neurological Clinic-Department of High Specialties- L. Sacco Hospital, University of Milan, Via GB Grassi 74, 20157 Milan, Italy 2 Eye Clinic-Department of Clinical Sciences BLuigi Sacco^, Sacco Hospital, University of Milan, Milan, Italy 3 Sobell Department of Motor Neuroscience and Movement Disorders, University College of London, London, UK Neurological Sciences https://doi.org/10.1007/s10072-018-3481-4