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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.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease and represents the main cause of sudden death in young patients. Cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT) are noninvasive imaging methods with high sensitivity and specificity, useful for the establishment of diagnosis and prognosis of HCM, and for the screening of patients with subclinical phenotypes. The improvement of image analysis by CMR and CCT offers the potential to promote interventions aiming at stopping the natural course of the disease. This study aims to describe the role of RCM and CCT in the diagnosis and prognosis of HCM, and how these methods can be used in the management of these patients. Resumo A cardiomiopatia hipertrófica (CMH) é a doença genética cardiovascular mais comum e representa a principal causa de morte súbita em pacientes jovens. A Ressonância Magnética Cardíaca (RMC) e a Tomografia Computadorizada Cardíaca (TCC) são métodos de imagem não invasivos de alta sensibilidade e especificidade, úteis no diagnóstico e no estabelecimento do prognóstico de CMH, e no rastreamento de indivíduos com fenótipos subclínicos. O aperfeiçoamento da análise da RMC e TCC tem grande potencial para promover intervenções com o objetivo de impedir o curso natural da doença. O presente estudo tem por objetivo descrever o papel da RMC e TCC no diagnóstico e prognóstico da CMH, e como essas ferramentas podem auxiliar no manejo clínico de pacientes portadores dessa doença.