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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May 7, 2015
Superficial Siderosis Secondary to a Paraganglioma
- Superficial siderosis (SS) is due to hemosiderin deposition in the subpial layers of the brain and spinal cord. The hemosiderin deposition is a consequence of recurrent and persistent bleeding into the subarachnoid space.
- Typical clinical presentation of SS: Slowly progressive gait disorder, ataxia with cerebellar dysarthria, and sensorineural hearing impairment.
- Work-up: MRI of the brain and spinal cord, CT angiogram, MRA, DSA
- Key Diagnostic Features:
- Noncontrast CT head and spine: unremarkable.
- MRI, especially GrE/SWI, demonstrates susceptibility along the surface of the brain and spinal cord.
- The finding of superficial siderosis necessitates evaluation for an underlying cause. Possible causes include a mass or vascular malformation in the brain or spine. A paraganglioma in the spine was the underlying etiology in the currrent case.
- Rx: Depends on the underlying cause. Surgical resection, embolization, or observation (in cases such as amyloid angiopathy).
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