Abstract
Background: Superficial siderosis is a progressively disabling disease caused by recurrent subarachnoid hemorrhage with accumulation of hemosiderin in the surface of the central nervous system. Although a wide variety of conditions may cause superficial siderosis, approximately half of the cases are reported to be associated with a defect in the ventral spinal dura mater, in which case treatment entails surgical repair of the defect. Here, we report a case of superficial siderosis and report on our method to pinpoint the dural defect using a combination of magnetic resonance imaging (MRI) techniques. Methods and Results: A 74-year-old female presented suffering from hearing loss and progressive ataxia over a duration of seven years. A T2-weighted MRI study revealed hypointensity in the superficial areas of the central nervous system, leading to the diagnosis of superficial siderosis, and the presence of a fluid-filled collection in the anterior spinal canal of C7 to T10 suggested that a dural defect was the cause of the repeated hemorrhage. A balanced turbo field echo (BTFE) MRI sequence revealed possible dural defects at T1–T2 and T5–T6, and a dynamic improved motion-sensitized driven-equilibrium steady-state free precession (dynamic iMSDE SSFP) sequence revealed an irregular flow of cerebrospinal fluid through the dura at the T5–T6 level. The dural defect was confirmed and sutured through a minimal T5–T6 laminectomy without neurological consequences, and the patient reported mild improvement in gait one year after surgery. Conclusions: A combination of MRI sequences provided the necessary information to confidently perform minimal surgery to repair the dural defect. We recommend coupling a balanced steady-state free precession (SSFP) sequence to provide high resolution, high contrast images of anatomical structures and a dynamic iMSDE SSFP sequence to confirm cerebrospinal fluid motion through the defect.
Highlights
Superficial siderosis of the central nervous system is an uncommon and often underdiagnosed disorder that is caused by repeated hemorrhages in the subarachnoid space
Patients diagnosed with superficial siderosis need to be screened for the possibility of dural defects with magnetic resonance imaging (MRI)
As to how duropathy in the spinal column could cause hemorrhage into the subarachnoid space, two theories have been proposed: the first cites bleeding from the bridging veins on the surface of the cerebellum that rupture due to brain sagging brought about by intracranial hypotension [4], and the second cites bleeding from the epidural veins around the dural defect [5]
Summary
Superficial siderosis of the central nervous system is an uncommon and often underdiagnosed disorder that is caused by repeated hemorrhages in the subarachnoid space. Since any condition that causes repeated bleeding into the subarachnoid space could lead to superficial siderosis, the listed etiology in the literature is wide-ranging. The literature on spinal duropathy associated with superficial siderosis often describe a ventral dural defect, most often in the thoracic spine, with a longitudinally fluid-filled collection in the anterior aspect of the spinal canal. As to how duropathy in the spinal column could cause hemorrhage into the subarachnoid space, two theories have been proposed: the first cites bleeding from the bridging veins on the surface of the cerebellum that rupture due to brain sagging brought about by intracranial hypotension [4], and the second cites bleeding from the epidural veins around the dural defect [5]. We describe a combination of MRI techniques that allowed us to confidently determine the location of the dural defect and to perform a minimally invasive surgery to repair the lesion
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