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Susac syndrome. A differential diagnosis for demyelination
Surjith Vattoth1, Christopher J Compton, Glenn H Roberson
1Department of Radiology, Neuroradiology Section, University of Alabama at Birmingham, Birmingham, AL 35249-6830, USA. drsurjith@yahoo.co.uk
Neurosciences (Riyadh, Saudi Arabia)
|January 8, 2013
Summary
Susac syndrome, an autoimmune microangiopathy, affects the brain, retina, and inner ear. Early recognition of its varied symptoms is crucial to avoid misdiagnosis with conditions like multiple sclerosis.
Area of Science:
- Neurology
- Ophthalmology
- Rheumatology
Background:
- Susac syndrome is a rare autoimmune microangiopathy affecting the brain, retina, and inner ear.
- It is often misdiagnosed as multiple sclerosis or acute disseminated encephalomyelitis due to overlapping symptoms.
Observation:
- A 25-year-old male presented with a year-long progression of encephalopathy, visual disturbances, and hearing loss.
- Characteristic MRI revealed central corpus callosum involvement and brain infarctions.
Findings:
- Fluorescein angiography confirmed branch retinal arterial and arteriolar occlusions, supporting the Susac syndrome diagnosis.
- The complete clinical triad (neurologic, visual, auditory) may not be present initially.
Implications:
- A high index of suspicion is vital for clinicians and radiologists to correctly diagnose Susac syndrome.
- Ophthalmologic follow-up is recommended even with normal initial examinations to detect potential branch retinal artery occlusions.
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