Superficial siderosis (SS) is a neurodegenerative condition caused by hemosiderin deposition on the surface of the brain, cranial nerves, and spinal cord. Superficial siderosis is an exceptionally rare condition characterized by a triad of hearing loss, ataxia, and myelopathy. The most common etiologies are trauma, previous surgical procedures, dural tears, and tumors of the central nervous system. We have demonstrated the efficacy of deferiprone (Ferriprox), a lipid-soluble iron chelator, for reducing hemosiderin deposition through MR imaging in a series of 10 patients with SS using 30 mg/kg/day of deferiprone. With approval of deferiprone by the US Food and Drug Administration in October 2011, we have launched an observational trial to characterize the potential clinical benefit of hemosiderin chelation (www.clinicaltrials.gov, identifier NCT01284127).
Research by: Rafael H. Llinas, MD
Carotid endarterectomy (CEA) is the currently accepted treatment for patients with significant carotid artery stenosis. However, carotid artery stenting (CAS) has recently emerged as a less invasive alternative procedure for patients considered to be high surgical risk after results from the SAPPHIRE trial (2004, NEJM) suggested that stenting was equivalent to CEA in patients at high risk for complications due to general anesthesia. CAS has not yet been compared to CEA in larger patient populations with asymptomatic significant carotid artery disease. The ACT I trial was designed to address this population and will compare carotid stenting to CEA in patients who are symptom-free and at standard risk for surgery. Enrollment is now underway at over 50 sites throughout North America including Johns Hopkins Bayview Medical Center.