Idiopathic Superficial Siderosis – a Case Report

Česká verzia

Authors: D. Krajíčková ¹; L. Klzo ²; A. Krajina ²
Authors place of work: LF UK a FN Hradec Králové Neurologická klinika ¹; LF UK a FN Hradec Králové Radiologická klinika ²
Published in the journal: Cesk Slov Neurol N 2013; 76/109(6): 759-762
Category: Case Report

Summary

Superficial siderosis (SS) of the central nervous system (CNS) is a rare, progressive, irreversible and debilitating disorder in which hemosiderin – a blood degradation product – is deposited in the subpial layers of the brain and spinal cord, leading to loss of neurons and myelin and development of neurological deficit. Clinical presentation of SS is characterized by a typical triad of symptoms consisting of adult-onset slowly progressive cerebellar ataxia, signs of myelopathy and sensorineural hearing impairment. MR imaging shows a characteristic T2 hypointensity around the brain stem, cerebellum, and spinal cord. The authors present a case of a woman with progressive hearing impairment from 53 years of age and gradual development of deteriorating paleocerebellar and spinal symptoms. Despite extensive imaging, the source of bleeding has never been identified.

Key words:
siderosis – magnetic resonance – ferritin – haemosiderin – cerebellar ataxia – deafness

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

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