Shakila Meshkat1,2, Parnia Ebrahimi3, Abbas Tafakhori4, Aidin Taghiloo5, Sajad Shafiee6, Amir Salimi7, Vajiheh Aghamollaii8. 1. Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran. 2. Department of Neurology, Roozbeh Psychiatric Hospital, Tehran University of Medical Sciences, Tehran, Iran. 3. Department of Psychiatry, Roozbeh Psychiatric Hospital, Tehran University of Medical Sciences, Tehran, Iran. 4. Iranian Center of Neurological Research (ICNR), Tehran University of Medical Sciences, Tehran, Iran. 5. Department of Radiology, Tehran University of Medical Sciences, Tehran, Iran. 6. Department of Neurosurgery, Mazandaran University of Medical Sciences, Sari, Iran. 7. Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 8. Department of Neurology, Roozbeh Psychiatric Hospital, Tehran University of Medical Sciences, Tehran, Iran. Vajiheh102@gmail.com.
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.
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.
Authors: Nikola D Kresojević; Igor N Petrović; Natasa T Dragaević-Misković; Vladimir S Kostić Journal: Srp Arh Celok Lek Date: 2013 Mar-Apr Impact factor: 0.207