1. Idiopathic superficial siderosis of the central nervous system
- Author
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Sajad Shafiee, Aidin Taghiloo, Abbas Tafakhori, Amir Salimi, Parnia Ebrahimi, Vajiheh Aghamollaii, and Shakila Meshkat
- Subjects
medicine.medical_specialty ,Ataxia ,Neurology ,Hearing loss ,Chronic subarachnoid hemorrhage ,Superficial siderosis ,Case Report ,Hemosiderin ,lcsh:RC346-429 ,Central nervous system disease ,03 medical and health sciences ,0302 clinical medicine ,medicine ,030212 general & internal medicine ,lcsh:Neurology. Diseases of the nervous system ,business.industry ,Arteriovenous malformation ,Sensorineural deafness ,medicine.disease ,Neurology (clinical) ,Neurosurgery ,Radiology ,medicine.symptom ,business ,030217 neurology & neurosurgery - 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.
- Published
- 2021