Case report illustrates the role of MRI in diagnosing superficial siderosis, indicating critical early intervention.
Superficial siderosis (SS) is a rare neurodegenerative condition caused by chronic or recurrent bleeding into the subarachnoid space, leading to the deposition of hemosiderin in the subpial layers of the brain, brainstem, spinal cord, or cranial nerves. SS is categorized into two main forms based on its anatomical distribution: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Among these, iSS is further subdivided into Type 1 (classical iSS) and Type 2 (secondary iSS). Classical iSS refers to cases in which there is no radiologically confirmed single spontaneous or traumatic intracranial hemorrhage and is typically associated with chronic bleeding sources, such as cranial or spinal dural defects. In contrast, secondary iSS is characterized by the presence of a radiologically identifiable single intracranial hemorrhagic event, either spontaneous or traumatic, which is considered the likely cause of the siderosis. Patients typically present with SS in the fourth to sixth decades of life. The prevalence of SS has been increasing in recent years due to the more widespread use of magnetic resonance imaging (MRI). Early imaging and diagnosis are crucial for identifying the bleeding source and enabling timely intervention, which may prevent further neurological deterioration. We report the case of a 69-year-old woman with a history of subdural hematoma and surgical intervention who presented with progressive bilateral hearing loss, imbalance, headache, and hypogeusia. MRI of the brain revealed classical imaging features of SS. Although further evaluation was recommended, the patient declined extensive diagnostic work-up. Empirical treatment with the iron-chelating agent deferiprone was initiated. This case highlights the diagnostic challenges of SS and emphasizes the importance of comprehensive imaging and early intervention.
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Viju et al. (2025) studied this question.
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