Updating superficial siderosis of the central nervous system: bleeding of a dorsal osteophyte into the subarachnoid space from a perforating artery

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Superficial siderosis of the central nervous system (SSCNS) is an uncommon and often unrecognized disorder that results from recurrent and persistent bleeding into the subarachnoid space. Currently, there is no effective treatment for SSCNS. The identification and surgical resolution of the cause of bleeding remains the most reliable method of treatment, but the cause of bleeding is often not apparent. The identified sources of recurrent bleeding have typically included neoplasms, vascular malformations, brachial plexus or nerve root injury or avulsion, and previous head and spinal surgery. An association between recurrent bleeding in the CNS and dural abnormalities in the spine has recently been suggested. Dural tears have been identified in relation to a protruding disc or osteophyte. Also in these patients, the exact mechanism of bleeding remains unknown because of a lack of objective surgical data, even in patients who undergo neurosurgical procedures.

The present case concerns a 48-year-old man who presented with longstanding symptoms of mild hearing loss and mild gait ataxia. A diagnosis of SSCNS was made in light of the patient’s history and the findings on physical examination, imaging, and laboratory testing. MRI and CT detected a small calcific osteophyte in the anterior epidural space of T8–9. The patient underwent surgical removal of the bone spur and dural tear repair. During the surgery, the authors detected a perforating artery, which was on the osteophyte, that was bleeding into the subarachnoid space. This case shows a possible mechanism of chronic bleeding from an osteophyte into the subarachnoid space. In the literature currently available, a perforating artery on an osteophyte bleeding into the subarachnoid space has never been described in SSCNS.

ABBREVIATIONS SSCNS = superficial siderosis of the central nervous system.

Article Information

Correspondence Carlo Brembilla: Pope John XXIII Hospital, Bergamo, Italy. carlinobrembo@hotmail.com; carlinobrembo@gmail.com.

INCLUDE WHEN CITING Published online October 12, 2018; DOI: 10.3171/2018.7.SPINE18300.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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Figures

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    Spinal cord and brain T2-weighted MRI scans showing hypointensity in the superficial areas of the CNS (asterisks in gradient-echo sequences), suggestive of hemosiderin deposition, also with a superior pattern of cerebellar atrophy.

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    MRI scans showing a contrast-enhanced spot in front of the T8–9 vertebral body (red arrow), a finding compatible with an osteophyte. Figure is available in color online only.

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    CT scans confirming the presence of a small calcific osteophyte (red arrow) in the anterior epidural space of T8–9. Figure is available in color online only.

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    The perforating artery bleeding on the bone spur is likely the cause of the subarachnoid chronic bleeding. Figure is available in color online only.

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    Two-year postoperative T2-weighted MRI studies of the spinal cord and brain showing a considerable reduction in the CNS hemosiderin signal (asterisks in gradient-echo sequences).

References

1

Angstwurm KSchielke EZimmer CKivelitz DWeber JR: Superficial siderosis of the central nervous system: response to steroid therapy. J Neurol 249:122312252002

2

Bonito VAgostinis CFerraresi SDefanti CA: Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg 80:9319341994

3

Egawa SYoshii TSakaki KInose HKato TKawabata S: Dural closure for the treatment of superficial siderosis. J Neurosurg Spine 18:3883932013

4

Koeppen AHDickson ACChu RCThach RE: The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34:6466531993

5

Kumar N: Beyond superficial siderosis: introducing “duropathies.” Neurology 78:199219992012

6

Kumar N: Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol 31:5142010

7

Kumar NCohen-Gadol AAWright RAMiller GMPiepgras DGAhlskog JE: Superficial siderosis. Neurology 66:114411522006

8

Leussink VIFlachenecker PBrechtelsbauer DBendszus MSliwka UGold R: Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand 107:54612003

9

Levy MLlinas R: Pilot safety trial of deferiprone in 10 subjects with superficial siderosis. Stroke 43:1201242012

10

Takai KKomori TNiimura MTaniguchi M: Superficial siderosis of the central nervous system associated with intraspinal hemorrhage from ventral thoracic epidural veins and a ventral spinal CSF leak: case report. J Neurosurg Spine 26:7517532017

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