Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder

ABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imagin...

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Main Authors: Yara Dadalti Fragoso, Tarso Adoni, Joseph Bruno Bidin Brooks, Sidney Gomes, Marcus Vinicius Magno Goncalves, Cassio Lemos Jovem, Andre Palma da Cunha Matta, Joao Filipe Oliveira, Fabio Siquinelli, Carlos Bernardo Tauil, Guilherme Navarro Troiani, Paulo Roberto Wille
Format: Article
Language:English
Published: Academia Brasileira de Neurologia (ABNEURO)
Series:Arquivos de Neuro-Psiquiatria
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000200004&lng=en&tlng=en
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author Yara Dadalti Fragoso
Tarso Adoni
Joseph Bruno Bidin Brooks
Sidney Gomes
Marcus Vinicius Magno Goncalves
Cassio Lemos Jovem
Andre Palma da Cunha Matta
Joao Filipe Oliveira
Fabio Siquinelli
Carlos Bernardo Tauil
Guilherme Navarro Troiani
Paulo Roberto Wille
author_facet Yara Dadalti Fragoso
Tarso Adoni
Joseph Bruno Bidin Brooks
Sidney Gomes
Marcus Vinicius Magno Goncalves
Cassio Lemos Jovem
Andre Palma da Cunha Matta
Joao Filipe Oliveira
Fabio Siquinelli
Carlos Bernardo Tauil
Guilherme Navarro Troiani
Paulo Roberto Wille
author_sort Yara Dadalti Fragoso
collection DOAJ
description ABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imaging (MRI) shows a characteristic curvilinear pattern of hypointensity on its blood-sensitive sequences. Methods Series of cases collected from Brazilian centers. Results We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion SS-CNS is a rare disease that may remain undiagnosed for long periods. Awareness of this condition is essential for the clinician.
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spelling doaj.art-6be6834078fe4f85b8a147c7bc9a9ac22022-12-21T18:33:17ZengAcademia Brasileira de Neurologia (ABNEURO)Arquivos de Neuro-Psiquiatria1678-4227752929510.1590/0004-282x20170001S0004-282X2017000200004Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorderYara Dadalti FragosoTarso AdoniJoseph Bruno Bidin BrooksSidney GomesMarcus Vinicius Magno GoncalvesCassio Lemos JovemAndre Palma da Cunha MattaJoao Filipe OliveiraFabio SiquinelliCarlos Bernardo TauilGuilherme Navarro TroianiPaulo Roberto WilleABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imaging (MRI) shows a characteristic curvilinear pattern of hypointensity on its blood-sensitive sequences. Methods Series of cases collected from Brazilian centers. Results We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion SS-CNS is a rare disease that may remain undiagnosed for long periods. Awareness of this condition is essential for the clinician.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000200004&lng=en&tlng=ensideroseencéfaloperda auditivaataxia
spellingShingle Yara Dadalti Fragoso
Tarso Adoni
Joseph Bruno Bidin Brooks
Sidney Gomes
Marcus Vinicius Magno Goncalves
Cassio Lemos Jovem
Andre Palma da Cunha Matta
Joao Filipe Oliveira
Fabio Siquinelli
Carlos Bernardo Tauil
Guilherme Navarro Troiani
Paulo Roberto Wille
Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
Arquivos de Neuro-Psiquiatria
siderose
encéfalo
perda auditiva
ataxia
title Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
title_full Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
title_fullStr Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
title_full_unstemmed Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
title_short Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
title_sort superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder
topic siderose
encéfalo
perda auditiva
ataxia
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000200004&lng=en&tlng=en
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