Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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April 25, 2011
Superficial Siderosis (SS)
- Superficial siderosis results from hemosiderin deposition in the subpial layers of the brain and spinal cord as a consequence of repetitive bleeding in the subarachnoid space.
- The classic clinical presentation of SS includes slowly progressive gait ataxia with cerebellar dysarthria and sensori-neural hearing loss.
- Common etiologies include trauma (eg. cervical nerve root avulsions), neoplasms (eg. oligodendroglioma, ependymoma, astrocytoma), and vascular lesions (eg. arteriovenous malformations, cavernomas, aneurysms, and fragile capillary regrowth after brain surgery). Sometimes, the underlying etiology remains unknown.
- Key Diagnostic Features: MR findings can be subtle. GRE or T2* are especially helpful due to their higher sensitivity for hemosiderin. Rim of susceptibility follows the contours of the brain, cranial nerves, and spinal cord. Role of imaging is to detect the underlying source of bleeding. If this source of bleeding cannot be established using cross-sectional imaging, catheter angiogram should be obtained.
- Treatment: Surgery, if source of bleeding can be established.
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