Abstract
Superficial siderosis (SS) of the central nervous system is a rare condition characterized by hemosiderin deposits on the surface of the brain and spinal cord.1,2 In the classical infratentorial type, SS is centered in the superior cerebellum and there is no history of intracranial bleeding.1,2 The clinical triad of infratentorial SS is slowly progressive hearing loss, ataxia, and myelopathy.1,2 About half of patients with infratentorial SS are found to have a spinal dural defect, oftentimes a chronic ventral type 1a spinal CSF leak as is seen in patients with spontaneous intracranial hypotension.1-4 Chronic hemorrhage at the site of the spinal leak is the likely cause of the SS in these patients.5 Identifying the site of leak allows surgical closure of the ventral tear thereby eliminating the cause of the SS. The goal of surgical repair is to stop progression of disease. Improvement of SS on MRI has not been reported following surgery. We now report a patient in whom MRI showed regression of SS 38 months following surgical repair of a spontaneous ventral spinal CSF leak.
- Received January 5, 2021.
- Accepted February 1, 2021.
- © 2021 American Academy of Neurology
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