3073-3077-Neurological, otolaryngological and ophthalmological implications of Susac syndrome

during the day, numbness of the tongue and mouth, and a buzzing noise in his ears. Audiometry revealed sensorineural hearing loss of about 75 dB in the left ear (Figure 2). After two months, the symptoms relapsed and unstable balance, problems with short-term memory, and disorientation in time and in space were present. Video-electroencephalography (EEG) revealed numerous irregular theta waves generated in the anterior leads. A small number of delta waves were also present and localized in the temporal region. An elevated level of albumin and an IgG level (increased IgG index) without oligoclonal bands were detected in the cerebrospinal fluid (CSF) and an increased IgG concentration in the serum was identified. Magnetic resonance imaging showed numerous macular and ovoid lesions in both cerebral hemispheres (Figure 1), most of which ranged from 5 to 6 mm and were localized periventricularly in the frontal and temporal lobes and in the corpus callosum. They were spreading peripherally and were presented as hyperintense lesions (high-signal changes) in T2-weighted images with intravenous contrast and FLAIR (fluid attenuated inversion recovery) sequences. The biggest change was observed in the left lobar of the corpus callosum and amounted to 11 mm. A similar lesion was localised periventricularly in the frontal lobe and was as much as 10 mm. Moreover, on the left hemisphere of the cerebellum a 4 mm lesion was present. After intravenous contrast, pathologic enhancement was not observed. Because of the non-specific MRI finding, multiple sclerosis was suggested. The neoplasm was excluded. Methylprednisolone and mannitol were implemented intravenously with a positive outcome. The patient was discharged from hospital with the diagnosis of viral encephalitis associated with viral cochlear hearing injury. Abstract. – Susac syndrome is an endotheliopathy affecting the arterioles of the brain, retina, and inner ear. Many cases of Susac syndrome are underdiagnosed, mainly at the early stages of the disease, while prompt diagnosis enables a speedy recovery. Immediate treatment can halt disease progression and even prevent future disability. We report a case of Susac syndrome, describe the difficulties in the diagnosis of this case, and include a detailed history of a 35-year-old man via the presentation of extensive laboratory work-up and imaging studies. Audiometry showed sensorineural hearing loss of about 75 dB in the left ear. Ovoid lesions of the corpus callosum in magnetic resonance (MR) were present as were advanced binocular ophthalmological changes in fluorescent angiography. Methylprednisolone with acetylsalicylic acid and intravenous immunoglobulin (IVIG) were implemented with a positive outcome (clinical and audiometric improvement).