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MRI findings in Susac's syndrome
J O Susac1, F R Murtagh, R A Egan
1Neurology and Neurosurgery Associates, Winter Haven, FL 33880, USA. jsusac@neurohaven.com
Neurology
|December 25, 2003
Summary
Susac syndrome (SS) MRI scans reveal distinctive white matter lesions, primarily in the corpus callosum. These findings aid in diagnosing SS, especially when two of the three main symptoms are present.
Area of Science:
- Neurology
- Radiology
- Autoimmune Diseases
Background:
- Susac syndrome (SS) is a rare, presumed autoimmune condition.
- Characterized by a triad of encephalopathy, retinal artery occlusions, and hearing loss.
- Diagnosis can be challenging as not all triad elements may be present.
Purpose of the Study:
- To characterize the spectrum of Magnetic Resonance (MR) imaging abnormalities in Susac syndrome.
- To identify distinctive imaging patterns that support SS diagnosis.
Main Methods:
- Retrospective review of MR images from 27 patients with clinical Susac syndrome.
- Analysis of MR images from 51 patients reported in published literature.
Main Results:
- All 27 patients exhibited multifocal supratentorial white matter lesions, consistently involving the corpus callosum.
- Deep gray matter nuclei (basal ganglia, thalamus) were affected in 70% of patients.
- Parenchymal enhancement occurred in 70%, and leptomeningeal enhancement in 33%.
Conclusions:
- Susac syndrome demonstrates a characteristic MR imaging pattern of supratentorial white matter lesions, always including the corpus callosum.
- Deep gray matter involvement, posterior fossa lesions, and enhancement are common findings.
- Distinctive central callosal lesions differentiate SS from demyelinating diseases, supporting diagnosis with at least two clinical features.