Superficial siderosis of the central nervous system: case report

Introduction: Superficial Siderosis Central Nervous System (SS) is a rare disease characterized by the deposition of hemosiderin in the brain and spinal cord. Clinically there is progressive ataxia and sensorineural deafness associated with upper motor neuron lesion. The diagnosis is made by examina...

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Published inMedicina (Sao Paulo. 197?) Vol. 48; no. 4; pp. 412 - 415
Main Authors Vilaça, Celmir de Oliveira, Magella, Carla Baars, Afonso, Cristiane, Py, Marco Oliveira, Santos, Alair Sarmet, Novellino, Pietro, Orsini, Marco
Format Journal Article
LanguageEnglish
Portuguese
Published Universidade de São Paulo 11.12.2015
Subjects
Superficial Siderosis. Cerebellar Ataxia. Deafness
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Summary:Introduction: Superficial Siderosis Central Nervous System (SS) is a rare disease characterized by the deposition of hemosiderin in the brain and spinal cord. Clinically there is progressive ataxia and sensorineural deafness associated with upper motor neuron lesion. The diagnosis is made by examination of magnetic resonance imaging (MRI) Brain and Spinal Cord. Objective: Report a case of a patient with characteristic features of the syndrome, associated with the unusual presence of epilepsy. Method: We report the case of a patient with SS followed at the Institute of Neurology Deolindo Couto da UFRJ-RJ. Conclusion: The Superficial Siderosis Central Nervous System is a disease of the CNS that must be considered in any patient with ataxia and progressive deafness, requiring MRI, preferably images Gradient echo T2 for diagnosis.
ISSN:0076-6046
2176-7262
DOI:10.11606/issn.2176-7262.v48i4p412-415