Abstract
Superficial Siderosis (SS) of Central Nervous System is a rare disease characterized by the deposit of hemosiderin in the brain and spinal cord. Clinically, it is characterized by progressive sensorineural ataxia and deafness associated with injury of superior motor neuron. The diagnosis is made by magnetic resonance imaging (MRI) of the encephalon and spinal cord. The objective of the study is to report the case of a patient with characteristic elements of the syndrome, accompanied in a private medical clinic.
Highlights
Superficial Siderosis (SS) of Central Nervous System (CNS) was first reported by Hamill in 1908 as "melanosis of the brain"
With approximately three hundred cases reported in literature, this disorder results in chronic or intermittent bleeding in subarachnoid space that leads to the deposition of blood subproducts in the leptomeninges (Ryu et al, 2016; Vilaça et al, 2015; Offenbacher et al, 1996)
The results showed normal ECG and skull magnetic resonance imaging (MRI) with evidence of right occipital subcortical and cerebellar gliosis (Fig. 1)
Summary
Superficial Siderosis (SS) of Central Nervous System (CNS) was first reported by Hamill in 1908 as "melanosis of the brain". This disease affects approximately one in ten million people between the ages of 14 and 77 and becomes evident on average at age 50. The cause of bleeding is detected in about half of the cases and the most frequent are in highly vascularized spinal tumors, vascular malformations of the CNS, surgical procedures in the posterior fossa, trauma or previous intradural surgery (Sighary, Cohen-Addad & Linder, 2018; Ribeiro et al, 2013; Hiraka et al, 2018; Fragoso et al, 2017; Fearnley, Stevens & Rudge, 1995; Anderson, Sheffield & Hope, 1999)
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