Three cases of superficial siderosis of the central nervous system and review of the literature
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Superficial siderosis of the central nervous system results from chronic or intermittent hemorrhage into the subarachnoid space that causes hemosiderin deposition in subpial layers of the brain and the spinal cord leading to neuronal damage. Patients present with progressive and debilitating symptoms that typically include adult-onset slowly progressive cerebellar gait ataxia and sensorineural hearing impairment. Regardless of extensive investigations, the origin of the hemorrhage is often not clear. Because of the good availability of magnetic resonance imaging, asymptomatic cases of superficial siderosis of the central nervous system are increasingly discovered. SS cases are increasingly reported in the literature. We present three new cases. The etiology, pathogenesis, clinical features, and treatment options of SS are reviewed.
KeywordsSiderosis Hemosiderosis Hemosiderin Central nervous system Subarachnoid hemorrhage Sensorineural deafness Ataxia Brachial plexus injury
The authors thank Dr. Markku Päivärinta and Dr. Janne Lähdesmäki for assistance in finding the SS cases. The authors also thank Dr. Timo Takala for help with the laboratory results.
Conflicts of interest
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