Susac syndrome
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Etiology
Epidemiology
- rare
Pathology
- encephalopathy
- retinal artery occlusion
- inner ear disease/cochlear disease (hearing loss)
Clinical manifestations
- variable
- headaches (migraine-like) may precede the development of other symptoms
- hearing loss, tinnitus, vertigo
- gait ataxia, dysarthria
- cognitive impairment
- psychiatric symptoms
- skin changes
- subacute clinical progression without remission or relapse[2]
Radiology
- neuroimaging: magnetic resonance imaging (MRI)
- corpus callosum lesions seen[1]
Differential diagnosis
Management
- immunosuppressive agents
- glucocorticoids: prednisone
- mycophenylate mofetil (Cellcept)
- azathioprine (Imuran),
- methotrexate,
- cyclophosphamide
- rituximab
- TNF inhibitor
- intravenous immunoglobulins (IVIG)
- hearing loss
- hearing aid
- profound hearing loss: cochlear implant may be beneficial[1]
More general terms
References
- ↑ 1.0 1.1 1.2 Susac's Syndrome National Organization for Rare Disorder (NORD) https://rarediseases.org/rare-diseases/susacs-syndrome/
- ↑ 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 17, American College of Physicians, Philadelphia 2015
- ↑ 3.0 3.1 Tamez H, Patel SN Retinal Artery Occlusion After a Dog Bite in a 55-Year-Old Man. JAMA Ophthalmol. Published online May 6, 2021 PMID: https://www.ncbi.nlm.nih.gov/pubmed/33956084 https://jamanetwork.com/journals/jamaophthalmology/fullarticle/2779737
AMA Ed Hub. JN Learning https://edhub.ama-assn.org/jn-learning/module/2779737