Superficial Siderosis

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  • Likely from repeated slow hemorrhage into the subarachnoid space with resultant hemosiderin deposition in subpial layers, the strongest association with this disease is prior trauma. Additionally, tumors, vascular malformations, subdural hematomas, or prior neurosurgery may predispose patients to this disease. Interestingly, a clinical history of prior subarachnoid hemorrhage is often absent.
  • Common clinical findings of this disease include a 90% prevalence of gait ataxia and hearing impairment. Dementia is also common.
  • Diagnosis is most often based on imaging, in which a fluid collection is often seen in the spinal canal. Cerebellar atrophy (particularly vermal) is exceedingly common. Additionally, a thin rim of hypointensity on T2-weighted imaging, most often along the vermis, brainstem and interhemispheric sulci may be observed. There have been case reports of dynamic CT myelography identifying the etiology.
  • There is no definitive cure for this disease. If there is a chronic bleeding source identified, it may be treated to prevent future hemorrhage.

Kumar, N. et al. Superficial Siderosis. Neurology. 2006: (66) 1144-1152.

Offenbacher, H. et al Superficial Siderosis of the Central Nervous System: MRI findings and clinical significance. Neuroradiology 1996; 38 Suppl 1: S51-6.

Kumar, N. et al. Role of Dynamic CT Myelography in Identifying the Etiology of Superifical Siderosis. Neurology 2005; 65: 486-488.

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