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

TL;DR

This paper reports two rare cases of superficial siderosis in the central nervous system, highlighting their spinal pathology and the importance of MRI for diagnosis.

Contribution

The paper adds to the literature by presenting two unique clinical cases with spinal involvement and emphasizing MRI's role in diagnosis.

Findings

Superficial siderosis was confirmed in the spinal cord and cerebellum via MRI in both cases.

Clinical manifestations included myelopathy, hearing loss, and spasticity, with diverse underlying causes.

A multidisciplinary approach is essential for managing this complex condition.

Abstract

Infratentorial superficial siderosis, characterized by hemosiderin deposition in the subpial layers of the brainstem, cerebellum, and spinal cord, is a rare progressive neurologic disorder. We present two cases of infratentorial superficial siderosis. Case 1 involves a 62-year-old female previously diagnosed with tethered cord syndrome and thoracic myelopathy, who, following 11 spinal surgeries, presented with worsening myelopathy, hearing loss, and cognitive impairment. Brain magnetic resonance imaging (MRI) revealed extensive superficial siderosis affecting the cerebellar vermis and bilateral cerebellar hemispheres. Case 2 is a 27-year-old male with a traumatic T4 spinal cord injury from a gunshot wound, complicated by a syrinx, experiencing persistent lower back pain and lower limb spasticity. MRI confirmed superficial siderosis in the spinal cord. This case report explores the clinical manifestations, imaging findings, management strategies, and prognosis of these cases. It also highlights the diverse clinical presentations and underlying etiologies of infratentorial superficial siderosis. It emphasizes the pivotal role of MRI with iron-sensitive sequences for definitive diagnosis. Furthermore, the management underscores the significance of a multidisciplinary team approach in providing comprehensive care for affected individuals.

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