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February 21, 2017; 88 (8) Resident and Fellow Section

Teaching NeuroImages: Superficial siderosis due to a dural cervical arteriovenous fistula

Rachid Madkouri, Michael Grelat
First published February 20, 2017, DOI: https://doi.org/10.1212/WNL.0000000000003638
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A 58-year-old man presented to our hospital with cerebellar ataxia, pyramidal signs, dysarthria, bilateral deafness, and cognitive impairment. These symptoms were consistent with superficial siderosis (SS) of the CNS, confirmed by MRI (figure A).1 Cerebral angiography showed a dural arteriovenous fistula perispinal and pontic with venous drainage in the left foramen C1-C2, fed by meningeal branches of the vertebral artery (figure, B). The fistulous point was clipped. SS of CNS is a rare disease resulting from hemosiderin deposition on the surface of the CNS and cranial nerves.2 At diagnosis, the etiology may not be known. Finding etiology is necessary (trauma, vascular malformation).2

Figure
Figure MRI and angiography

(A) Axial T2-weighted gradient echo MRI shows hemosiderin deposition in supratentorial brain (inside of sulcus) and in infratentorial brain (on the surface of the brainstem and the cerebellum). (B) Cerebral angiography: dural arteriovenous fistula in the C1-C2 left foramen, fed by meningeal branches of the vertebral artery.

AUTHOR CONTRIBUTIONS

Rachid Madkouri evaluated the patient and wrote the manuscript. Michael Grelat prepared the images and legends and finalized the manuscript.

STUDY FUNDING

No targeted funding reported.

DISCLOSURE

The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

Footnotes

REFERENCES

  1. 1.↵
    1. Koeppen AH,
    2. Michael SC,
    3. Li D, et al
    . The pathology of superficial siderosis of the central nervous system. Acta Neuropathol 2008;116:371–382.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Kumar N,
    2. Cohen-Gadol AA,
    3. Wright RA,
    4. Miller GM,
    5. Piepgras DG,
    6. Ahlskog JE
    . Superficial siderosis. Neurology 2006;66:1144–1152.
    OpenUrlAbstract/FREE Full Text
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