Idiopathic infratentorial superficial siderosis of the central nervous system: case report and review of literature.

Andrea Stabile, Vincenzo Di Lazzaro, Cesare Colosimo, Fabrizio Piazza, Carlo Ferrarese, Jacopo C DiFrancesco

Journal: Neurologia i neurochirurgia polska 2019;52(1):102-106

PMID: 29122310

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.

Address: 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.; Department of Neurology, ASST San Gerardo Hospital, Monza, Italy; Milan Center for Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.; Department of Neurology, ASST San Gerardo Hospital, Monza, Italy; Milan Center for Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy. Electronic address: [email protected].

DOI: 10.1016/j.pjnns.2017.10.006

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MeSH Terms: Adult, Central Nervous System, Humans, Magnetic Resonance Imaging, Radiography, Siderosis