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SUSAC SYNDROME IN THE ABSENCE OF ENCEPHALOPATHY AND NORMAL MRI
  1. Christopher Owen,
  2. Venkat Garikipati,
  3. Nicolas Weir
  1. Southampton General Hospital

    Abstract

    A 21 year–old young lady was referred to ophthalmology with visual disturbance in the right eye. Two weeks prior she had nausea and vertigo and was thought to have an ear infection. She then suffered from right sided hearing loss, tinnitus, sickness, frontal headaches and a few days later awoke with a right visual defect.

    Examination showed a right inferior visual field loss, branch retinal artery occlusions and visual acuity of 6/36. Neurological review revealed a right sensorineural hearing loss and there was no cognitive impairment.

    Fluoroscein angiogram confirmed branched retinal artery occlusions and cotton wool spots felt unlikely to be due to emboli and more in keeping with a vasculopathy. Investigations including transthoracic echocardiogram, carotid dopplers, thoracic MR angiography, MR brain with venography and autoiimune screen were normal or negative. Audiology confirmed low–frequency hearing loss in the right ear.

    The combination of branch retinal artery occlusions and sensorineural hearing loss was indicative of Susac syndrome. She was treated with with intravenous pulsed steroid followed by oral Prednisolone and Azathioprine.1 Her vision on the right improved to 6/9 with no change in hearing and she remains under follow-up.

    Susac syndrome is an endothelial vasculopathy and consists of the well known triad of branched retinal artery occlusion, sensorineural hearing loss and encephalopathy with typical MRI changes.2 3 All three components need not be present, especially in the early stages of disease. This patient represents a forme fruste of Susac syndrome with absence of obvious encephalopathy and with normal MRI. Prompt diagnosis and treatment in the early stages is needed to prevent further morbidity.4

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