Abstract
Superficial siderosis of the central nervous system is the result of chronic recurrent hemorrhages (e.g., arteriovenous malformations, tumors, or trauma), which leads to the accumulation of cytotoxic hemosiderin and presents with hearing loss, cerebellar dysfunction, and myelopathy. This article presents a clinical case of an 11-year-old boy in whom the diagnosis of medulloblastoma was established. He underwent surgery, and after a few years, he began to complain of hearing loss. Magnetic resonance imaging revealed the cause of the hearing disturbance. The aim of this article is to review the recent literature related to the etiology, clinical and radiologic features of superficial siderosis, emphasizing the role of magnetic resonance imaging.
Highlights
Superficial siderosis (SS) of the central nervous system (CNS) is an uncommon and often unrecognized disorder in which hemosiderin – a product of the breakdown of blood – is deposited in the leptomeninges, subpial layer, and ependymal surface of the CNS
The first pathologic clinical presentation of SS was a case presented by Hamill in 1908, but only approximately 40 cases appeared in the literature before the widespread use of magnetic resonance imaging (MRI) enabling physicians to make the diagnosis without a biopsy (1, 2)
It is secondary to current or previous CNS tumors (21%), followed by head or back trauma (13%), arteriovenous malformations/aneurysms (9%), postsurgical changes related to neurosurgeries (7%), brachial plexus injury (6%), amyloid angiopathy (3%), and chronic subdural hematomas (6%) (1, 3)
Summary
Superficial siderosis (SS) of the central nervous system (CNS) is an uncommon and often unrecognized disorder in which hemosiderin – a product of the breakdown of blood – is deposited in the leptomeninges, subpial layer, and ependymal surface of the CNS. New MRI scans were performed almost every year, and in 2010, SS was seen as a hypointense rim at the mesencephalic tectum and cerebellar folia (Fig. 1). This finding could readily be seen on previous scans as far back as 2005 (Fig. 2), and since the process seemed to be stable
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