%0 Journal Article
%T Susac's Syndrome in a Patient Diagnosed with MS for 20 Years: A Case Report
%A Bijen Nazliel
%A Asli Akyol
%A Hale Zeynep Batur Caglayan
%A Irem Yildirim-Capraz
%A Ceyla Irkec
%J Case Reports in Neurological Medicine
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/214648
%X Susac¡¯s syndrome is an uncommon neurologic disorder of unknown cause. It has been described as a clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusions. Clinically the diagnosis is difficult when the patient presents only a portion of a triad. We present a case with vision loss and sensorineural deafness and who had been diagnosed with MS for 20 years. Susac¡¯s syndrome is presumed to be an autoimmune endotheliopathy. Neurologic symptoms and signs are diffuse and multifocal, acute or subacute in onset, and progress during the active phase of the disease. In some patients the onset was stroke like and in others that of subacute dementia. Headache, often with migrainous features, was a prominent feature initially in more than one half of the patients. A high index of suspicion leading to correct diagnosis and early appropriate therapy may reduce the permanent sequel seen with this disease. Misdiagnosis is common. In patients in whom diagnosis and treatment are delayed permanent morbidity is higher in terms of visual loss, hearing loss, and neurologic debility. In patients in whom rapid diagnosis has led to early administration of immunosuppressive therapy, recovery can be almost complete. 1. Introduction Susac¡¯s syndrome (SS) is an uncommon neurologic disorder of unknown cause. It has been described as a clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusions. Clinically the diagnosis is difficult when the patient presents only a portion of a triad [1]. We present a case with vision loss and sensorineural deafness and who had been diagnosed with MS for 20 years. 2. Case Report A 41-year-old male patient was admitted to our outpatient clinic with a 20-year diagnosis of MS. On ophthalmic examination the visual acuity in the left eye was 20/100 and 20/20 in the right eye. No other neurological deficit or abnormality was identified in the neurological examination. In his past medical history, he had loss of vision in the left eye 20 years earlier, which did not improve at that time. He was diagnosed with MS and had received, once per month, IV 1000£¿mg methylprednisolone treatment for a couple of years. Seven years ago, he had sudden deafness in the left ear. An audiogram performed at that time showed total sensory neural deafness in the left and 20% loss of hearing in the right ear. Brain MRI findings were interpreted as consistent with a demyelinating disease. One year ago, he had sudden loss of vision in his right eye. A fluorescein angiogram performed at that time showed branch retinal artery
%U http://www.hindawi.com/journals/crinm/2014/214648/