Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
June 2015
Next Case of the Month coming July 7 . . .
Superficial Siderosis Resulting from Paraganglioma
- Superficial siderosis is an uncommon, potentially debilitating disorder resulting from hemosiderin deposition along the surface of the brain and spinal cord secondary to recurrent episodes of subarachnoid hemorrhage.
- Clinical Features: Sensorineural hearing loss, ataxia and pyramidal signs. Some patients can remain asymptomatic.
- Etiologies: Neoplasms (brain and/or spine), vascular malformations, and trauma
- Key Diagnostic Features:
- Susceptibility along the surface of the brain, and sometimes the cord, best appreciated on gradient-echo or SWI sequences. This should prompt a work-up to identify the underlying source of hemorrhage.
- Contrast-enhanced MRI, CT angiogram, should be obtained to assess for underlying mass or vascular malformation. If negative, MRI spine with contrast should be obtained to evaluate for trauma, or any tumor or vascular malformation. If negative, a DSA study can be obtained to evaluate for a vascular malformation, including a dural AV fistula.
- In this particular case, the differential diagnosis considered was an ependymoma or paraganglioma. Pathology revealed paraganglioma.