Idiopathic superficial siderosis of the central nervous system

Shakila Meshkat,Parnia Ebrahimi,Aidin Taghiloo,Sajad Shafiee,Abbas Tafakhori,Amir Salimi,Vajiheh Aghamollaii

doi:10.1186/s40673-021-00133-5

Abstract

BackgroundRegardless of the cause of the superficial siderosis (SS) disease, which is bleeding, the source of bleeding cannot be found in some cases.Case presentationIn 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. In both cases, brain MRI indicated evidence of SS. CT myelogram and SPECT with labeled RBC couldn’t help finding the source of occult bleeding.ConclusionSS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intracranial 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.

Highlights

Superficial siderosis (SS) is an extremely rare central nervous system (CNS) condition in which hemosiderin is deposited in the leptomeninges, subpial layer, ependymal surface, and other parts of the CNS and results in progressive neurological dysfunction [1]
SS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits
SS should be considered in cases with ataxia and hearing loss, even if no source of bleeding is found

Summary

Conclusion

SS is a rare central nervous system disease caused by the deposition of hemosiderin in the brain and spinal cord, which results in the progression of neurological deficits. The cause of this hemorrhage is often subarachnoid haemorrhage, intracranial 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. 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

Findings

Introduction

Discussion