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Idiopathic infratentorial superficial siderosis of the central nervous system: case report and review of literature
Affiliations- Department of Neurology, ASST San Gerardo Hospital, Monza, Italy
- Unit of Neurology, Neurophysiology, Neurobiology, Department of Medicine, University Campus Bio-Medico, Rome, Italy
- Radiology and Neuroradiology Unit, Diagnostic Imaging Area, Fondazione Policlinico Universitario A. Gemelli, Institute of Radiology, School of Medicine, Catholic University, Rome, Italy
- Milan Center for Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy
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Abstract
The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to an acquired iron-mediated neurodegeneration. First described more than 100 years ago, only recently SS has been divided into two distinct entities, according to the distribution of iron deposition in the CNS: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Here we describe an adult case of iSS, with detailed clinical and radiological features. Moreover, we extensively review the literature of SS, particularly focusing on the pathogenesis, clinical-radiological classification, diagnostic algorithm and treatment options of this rare condition.
Abstract
The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to an acquired iron-mediated neurodegeneration. First described more than 100 years ago, only recently SS has been divided into two distinct entities, according to the distribution of iron deposition in the CNS: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Here we describe an adult case of iSS, with detailed clinical and radiological features. Moreover, we extensively review the literature of SS, particularly focusing on the pathogenesis, clinical-radiological classification, diagnostic algorithm and treatment options of this rare condition.
Keywords
Superficial siderosis, Cerebral amyloid angiopathy, Hemosiderin, Brain Magnetic Resonance Imaging (MRI), Susceptibility weighted imaging
About this articleTitle
Idiopathic infratentorial superficial siderosis of the central nervous system: case report and review of literature
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
102-106
Published online
2017-10-27
Page views
478
Article views/downloads
1163
DOI
10.1016/j.pjnns.2017.10.006
Bibliographic record
Neurol Neurochir Pol 2018;52(1):102-106.
Keywords
Superficial siderosis
Cerebral amyloid angiopathy
Hemosiderin
Brain Magnetic Resonance Imaging (MRI)
Susceptibility weighted imaging
Authors
Andrea Stabile
Vincenzo Di Lazzaro
Cesare Colosimo
Fabrizio Piazza
Carlo Ferrarese
Jacopo C. DiFrancesco
