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Recently,a rare syndrome that involves uniformly the brain,inner ear,and retina in previously healthy young women has been described.Brain biopsies and ophthalmologic examinations disclosed small infarcts as a pathoanatomical substrate of the disease.In previous reports,an autoimmune disorder or a coagulopathy have been suggested as possible etiologies.Both patients(aged 22 and 20 years)had brain involvement with neurological and neuropsychological deficits.Multifocal small hyperintensities were shown in magnetic resonance imaging of the brain.Findings of cerebrospinal fluid examination and electroencephalography were pathological in case 1 and of cerebral angiography in case 2.Both patients had a neurosensory hearing loss and multiple retinal branch arteriolar occlusions.Both women were on fenfluramine before onset of the disease.In case 1,attacks recurred during a follow-up of 34 months.At onset of the disease the 5-hydrozyindoleacetic acid and homovanillic acid levels of the cerebrospinal fluid were reduced; 13 months later the 5-hydroxyindoleacetic acid level was still reduced and the homovanillic acid level was low-normal.In case 2,with the longest follow-up of 13 years,the disease was active during only the initial 2 1/4 years.During this period a combination of oral anticoagulant and antiplatelet agents was ineffective.Conclusions:Our findings could not support current etiologic hypotheses.Whether changes in 5- hydroxyindoleacetic acid and homovanillic acid levels in the cerebrospinal fluid and/or fenfluramine intake play a role in the pathogenesis of the disease remains to be elucidated. |
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blindness brain biopsy cerebral infarction cerebrovascular accident cerebrovascular accident,multiple cerebrovascular accident,women cerebrovascular accident,young adult deafness dementia encephalopathy fenfluramine hearing loss microangiopathy,brain microangiopathy,retina microinfarcts MRI MRI,abnormal personality change psychiatric problems in neurologic disorders psychosis retinal branch artery occlusion retinal infarction retinal lesion sensorineural hearing loss Susac's syndrome visual loss
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