Superficial siderosis of the CNS.

Abstract

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. 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.