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Multiple cavernous malformations as a cause for superficial siderosis of the central nervous system

  • Clément Richard
  • Laura Simmonet
  • Evelyne Guegan-Massardier
  • Jean-Paul Bouwyn
  • Benjamin HebantEmail author
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Introduction

A 56-year-old man with a past medical history of hypertension and Crohn’s disease was referred to our clinic for slowly progressive gait ataxia with episodes of vertigo and bilateral deafness with progressive worsening over the past 18 months.

He also complained of cognitive impairment for a few years. The patient did not present sudden headache nor stiff neck.

He had no history of neurosurgical procedures, brain tumors, nor cranial or spinal injuries. His familial history was unremarkable.

Clinical examination revealed severe gait impairment in relation with cerebella ataxia, bilateral sensorineural hearing loss and tetrapyramidal syndrome (hyperreflexia and extensor plantar responses).

Gradient echo T2*-weighted axial MRI of the brain showed a rim of hypointensity consistent with the presence of hemosiderin deposits on the surface of brainstem, cerebellum (with particular involvement of the superior cerebellar folia), cerebral hemispheres (Fig.  1a–c) and around the...

Keywords

Superficial siderosis of the central nervous system Cerebral hemorrhage Cavernoma Developmental venous anomaly Deafness 

Notes

Authors contributions

CR: study concept and design. LS: critical revision of the manuscript for important intellectual content. EG-M: critical revision of the manuscript for important intellectual content. J-PB: critical revision of the manuscript for important intellectual content. BH: study concept and design.

Funding information

No funding was reported.

Compliance with ethical standards

Conflict of interest

Clément Richard declares that he has no conflict of interest. Laura Simmonet declares that she has no conflict of interest. Evelyne Guegan-Massardier declares that she has no conflict of interest. Jean-Paul Bouwyn declares that he has no conflict of interest. Benjamin Hebant declares that he has no conflict of interest. The authors report no financial disclosures relevant to the manuscript.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Copyright information

© Belgian Neurological Society 2019

Authors and Affiliations

  • Clément Richard
    • 1
  • Laura Simmonet
    • 1
  • Evelyne Guegan-Massardier
    • 1
  • Jean-Paul Bouwyn
    • 1
  • Benjamin Hebant
    • 1
    Email author
  1. 1.Department of NeurologyRouen University Hospital and University of RouenRouen CedexFrance

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