open access

Vol 52, No 1 (2018)
Case reports
Submitted: 2017-09-11
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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
DOI: 10.1016/j.pjnns.2017.10.006
·
Neurol Neurochir Pol 2018;52(1):102-106.

open access

Vol 52, No 1 (2018)
Case reports
Submitted: 2017-09-11

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.

Get Citation

Keywords

Superficial siderosis, Cerebral amyloid angiopathy, Hemosiderin, Brain Magnetic Resonance Imaging (MRI), Susceptibility weighted imaging

About this article
Title

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

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 52, No 1 (2018)

Pages

102-106

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

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