Idiopathic Superficial siderosis of the central nervous system

Abstract

Background: Regardless of the cause of the superficial sidrosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases. Case presentation: In this article, we report two cases with idiopathic SS. Case 1 presented with bilateral hearing loss, cognitive impairment, sleep disturbances and tremors. Case 2 presented with sensory neural hearing loss, ataxia, and spastic paraparesis. Brain MRI indicated evidence of SS. CT myelogram and SPECT/CT with labeled RBC couldn't help finding the source of occult bleeding. Conclusion: Superficial sisdorsis (SS) is a rare central nervous system disease caused by deposition of hemosiderin in brain and spinal cord which results in progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intradural surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. The condition progresses slowly and, by the time diagnosis is confirmed, the damage is often irreversible. In our cases, brain MRI clarified the definitive diagnosis but we could not find the source of bleeding. SS should be considered in cases with ataxia and hearing loss even if no source of bleeding is found.

Ataxias: Hereditary, Acquired, and Reversible Etiologies

“…Deferiprone is the chelation therapy in addition to surgical repair of bleeding sources. [173][174][175] An ATP1A3-related disorder, the cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndrome should be a top consideration in pediatric patients with a dominant family history of relapsing ataxia and hearing loss. [176][177][178] Cerebellar ataxia can also be a postradiotherapy complication.…”