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. 2021 Feb 25;8(1):9.
doi: 10.1186/s40673-021-00133-5.

Idiopathic superficial siderosis of the central nervous system

Shakila Meshkat  1   2 , Parnia Ebrahimi  3 , Abbas Tafakhori  4 , Aidin Taghiloo  5 , Sajad Shafiee  6 , Amir Salimi  7 , Vajiheh Aghamollaii  8
Affiliations

Idiopathic superficial siderosis of the central nervous system

Shakila Meshkat et al. Cerebellum Ataxias. .

Abstract

Background: Regardless of the cause of the superficial siderosis (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. 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.

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. 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.

Keywords: Chronic subarachnoid hemorrhage; Hemosiderin; Sensorineural deafness; Superficial siderosis.

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Conflict of interest statement

There is no competing interest to report.

Figures

Fig. 1
Fig. 1
MRI brain images of the patient 1 showing features characteristic of superficial siderosis. 1. Low rim coating the surface of the brain and brain stem particularity cerebellum with obvious signal void intensity. 2. High signal intense rim in T1 sequences is coated brain stem and cerebellar vermis
Fig. 2
Fig. 2
MRI brain images of the patient 2 showing features characteristic of superficial siderosis. A rim of low intensity coating the surface of the brain and spinal cord with atrophy of cerebellar vermis
See this image and copyright information in PMC

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