%0 Journal Article
%T Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model
%A Robert M Rennebohm
%A Martin Lubow
%A Jerome Rusin
%A Lisa Martin
%A Deborah M Grzybowski
%A John O Susac
%J Pediatric Rheumatology
%D 2008
%I BioMed Central
%R 10.1186/1546-0096-6-3
%X Susac's syndrome (SS), which consists of the clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss (HL), appears to be due to autoimmune endotheliopathy in the microvasculature of the brain, retina, and inner ear [1,2]. Resultant ischemic injury produces pathognomonic central corpus callosal "holes" (infarcts) on MRI [3], retinal infarcts that are associated with a distinctive multifocal staining on fluorescein retinal angiography [4], and HL that is often accompanied by roaring tinnitus and vertigo.The immunopathogenesis of SS [1,2,5-9] appears to have much in common with that of juvenile dermatomyositis (JDM) [10-13], an autoimmune endotheliopathy that affects the microvasculature of a different triad of tissues (muscle, skin, and gastrointestinal tract). We report a 16 year old female with encephalopathic SS who was successfully treated with a protocol [2,14] based on aggressive treatment of severe dermatomyositis [13,15].Over a period of two weeks in January 2005, a 16 year old Caucasian female developed incapacitating headaches, recurrent vomiting, visual disturbance ("seeing round, dark black spots"), hearing loss, roaring tinnitus, slurred speech, unsteady gait, mild neck stiffness, confusion, short term memory deficit, slow thought processing, emotional lability, personality change, and a tendency to sleep excessively.When seen 2 weeks into the course of her illness, her visual acuity was 20/20 OU, but dilated ophthalmologic examination revealed a subtle "cotton wool" spot in her right eye. Though tired and mildly lethargic, she conversed normally and was able to provide her own history. Her gait was unsteady. She had bilateral long tract findings and difficulty performing rapid alternating movements, particularly on the left. The rest of her physical exam was normal.MRI of the brain showed numerous widespread small hyperintense white matter lesions on T2 and FLAIR in both the supratentorial and infratentorial compartment
%U http://www.ped-rheum.com/content/6/1/3