Superficial Siderosis of the Central Nervous System: A Report of Two Cases With Spinal Pathology and a Review of the Literature

Cureus, 2024 · DOI: 10.7759/cureus.60486 · Published: May 17, 2024

Neurology Medical Imaging Rehabilitation

Simple Explanation

Superficial siderosis (SS) of the central nervous system is a rare condition where iron deposits in the brain and spinal cord due to chronic bleeding. This report describes two cases of infratentorial superficial siderosis (iSS), where iron deposits specifically affect the brainstem, cerebellum, and spinal cord. One case involved multiple spinal surgeries, while the other involved a spinal cord injury from a gunshot wound; both leading to hemosiderin deposition and neurological symptoms.

Study Duration

Not specified

Participants

2 case reports

Evidence Level

Level 4: Case Reports

Key Findings

1
Infratentorial superficial siderosis can manifest with diverse clinical presentations, including ataxia, hearing loss, cognitive dysfunction, back pain, and spasticity.
2
MRI with iron-sensitive sequences is crucial for the definitive diagnosis of superficial siderosis.
3
The underlying etiology of iSS is often related to spinal dural defects, which can result from trauma, previous surgeries, or other abnormalities.

Research Summary

This report presents two cases of iSS, with a diagnosis confirmed through MRI, exhibiting distinctive clinical presentations, and shedding light on the remarkable heterogeneity of this condition. The first case displayed hemosiderin deposition in the cerebellar vermis and bilateral cerebellar hemispheres, whereas the second case revealed hemosiderin deposits within the thoracic spinal cord. In conclusion, iSS is a rare condition that typically presents with gait ataxia, hearing impairment, and cognitive dysfunction, and MRI imaging with iron-sensitive sequences is essential to confirm the diagnosis.

Practical Implications

Diagnostic Importance of MRI

MRI with iron-sensitive sequences is crucial for early and accurate diagnosis of superficial siderosis, even before the manifestation of clinical symptoms.

Multidisciplinary Management

A multidisciplinary team approach involving rehabilitation medicine, physical therapy, occupational therapy, audiology, and neuropsychology is essential for comprehensive care.

Etiological Consideration

In cases with known spinal dural defects and progressive neurological symptoms, superficial siderosis should be considered, prompting MRI evaluation.

Study Limitations

1
The underlying etiology of SS is not definitively confirmed.
2
Conservative management led to uncertain diagnoses.
3
There is a constraint in the follow-up period, limiting the ability to monitor the progression of the disease.

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