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Update on Susac Syndrome: New Insights in Brain and Retinal Imaging and Treatment Options
- Source :
- Journal of Alzheimer's Disease. 42:S99-S108
- Publication Year :
- 2014
- Publisher :
- IOS Press, 2014.
-
Abstract
- Susac syndrome (SuS) is a rare endotheliopathy of the brain, the retina, and the inner ear. The underlying patho- physiology is likely an autoimmune mediated occlusion of microvessels resulting in variable degrees of central nervous system (CNS) dysfunction, visual disturbances, and hearing loss. The disease manifests either with a monophasic or polycyclic course. Patients suffering from SuS are frequently misdiagnosed as having inflammatory demyelinating CNS disease, particularly mul- tiple sclerosis because of some overlap in the clinical presentation and the paraclinical findings. Since appropriate treatment of SuS is crucial for the prognosis, a timely and sound establishment of the diagnosis is important. Here, we summarize currently available information on the clinical presentation and diagnostic procedures in SuS. In particular, we discuss the added value of advanced techniques of brain and retinal imaging such as ultrahigh field magnetic resonance imaging and optical coherence tomography in SuS with respect to its differential diagnosis and pathophysiology. Since evidence-based treatment standards will not be available in the near future, we share some experiences in terms of treatment options. Finally, we briefly outline future areas of research in SuS.
- Subjects :
- Susac Syndrome
Hearing loss
Central nervous system
Encephalopathy
Disease
Retina
Diagnosis, Differential
Branch retinal artery occlusion
Image Processing, Computer-Assisted
medicine
Humans
Fluorescein Angiography
medicine.diagnostic_test
business.industry
General Neuroscience
Brain
Magnetic resonance imaging
General Medicine
medicine.disease
Magnetic Resonance Imaging
Psychiatry and Mental health
Clinical Psychology
medicine.anatomical_structure
Geriatrics and Gerontology
Differential diagnosis
medicine.symptom
business
Neuroscience
Tomography, Optical Coherence
Subjects
Details
- ISSN :
- 18758908 and 13872877
- Volume :
- 42
- Database :
- OpenAIRE
- Journal :
- Journal of Alzheimer's Disease
- Accession number :
- edsair.doi.dedup.....134f0bc4745f14964e3294cd825db09e