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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(02): 92-95
DOI: 10.1590/0004-282X20170001
ARTICLES

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

Siderose superficial do sistema nervoso central é uma doença rara e possivelmente subdiagnosticada
Yara Dadalti Fragoso
1   Universidade Metropolitana de Santos, Departamento de Neurologia, Santos SP, Brasil;
,
Tarso Adoni
2   Hospital Sirio-Libanes, Departamento de Neurologia, São Paulo SP, Brasil;
,
Joseph Bruno Bidin Brooks
1   Universidade Metropolitana de Santos, Departamento de Neurologia, Santos SP, Brasil;
,
Sidney Gomes
3   Hospital Beneficência Portuguesa e
Hospital Paulistano, Departamento de Neurologia, São Paulo SP, Brasil;
,
Marcus Vinicius Magno Goncalves
4   Centro Hospitalar Unimed, Departamento de Neurologia, Joinville SC, Brasil;
,
Cassio Lemos Jovem
5   Hospital de Base do Distrito Federal, Departamento de Radiologia, Brasília DF, Brasil;
,
Andre Palma da Cunha Matta
6   Universidade Federal Fluminense, Departamento de Neurologia, Niterói RJ, Brasil;
,
Joao Filipe Oliveira
7   Universidade Regional de Blumenau, Departamento de Neurologia, Blumenau SC, Brasil;
,
Fabio Siquinelli
7   Universidade Regional de Blumenau, Departamento de Neurologia, Blumenau SC, Brasil;
,
Carlos Bernardo Tauil
8   Hospital de Base do Distrito Federal, Departamento de Neurologia, Brasília DF, Brasil;
,
Guilherme Navarro Troiani
1   Universidade Metropolitana de Santos, Departamento de Neurologia, Santos SP, Brasil;
,
Paulo Roberto Wille
9   IdHera, Departamento de Neurorradiologia, Joinville SC, Brasil.
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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.

RESUMO

Siderose superficial (SS) do sistema nervoso central (SNC) é uma doença rara e provavelmente subdiagnosticada, resultante de sangramento crçnico no espaço subaracnóide, levando ao depósito de produtos sanguíneos nas camadas meníngeas subpiais. Ressonância magnética (RM) mostra um padrão curvilíneo característico com hipointensidade nas suas sequências sensíveis a sangue.

Métodos

Série de casos coletados de centros brasileiros.

Resultados

Apresentamos 13 casos de pacientes com história progressiva de disfunção neurológica causada por SS-SNC. Os achados clínicos mais frequentes destes pacientes foram ataxia progressiva da marcha, perda auditiva, hiperreflexia e disfunção cognitiva. O diagnóstico de SS-SNC foi firmado de sete meses a 30 anos após o início da doença.

Conclusão

SS-SNC é uma condição rara que pode permanecer sem diagnóstico por longos períodos. O conhecimento desta entidade é essencial ao clínico.

siderosis - brain - hearing loss - ataxia
siderose - encéfalo - perda auditiva - ataxia


Publication History

Received: 31 May 2016

Accepted: 16 November 2016

Article published online:
05 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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