Superficial Siderosis of the CNS

Objective: The objective of this article is to illustrate and describe the cause, physiopathology, natural history, and clinical and imaging presentations of superficial siderosis as a cause of progressive cerebellar ataxia with sensorineural hearing loss and to discuss the therapeutic rationale.

Conclusion: Superficial siderosis resulting in subpial deposition of hemosiderin along the surface of the cerebellum, brain, spinal cord, and cranial and peripheral nerves is a known cause of progressive cerebellar ataxia and sensorineural hearing loss. MRI evaluation of the entire neuraxis plays a key role in establishing the diagnosis and identifying the source of chronic bleeding. Treatment of the source of bleeding can halt the otherwise deteriorating clinical course of disease.