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Journal of Neurology

, Volume 259, Issue 10, pp 2240–2242 | Cite as

Erdheim–Chester disease presenting with an intramedullary spinal cord lesion

  • Charalampos TzoulisEmail author
  • Ivar Otto Gjerde
  • Eirik Søfteland
  • Gesche Neckelmann
  • Eivind Strøm
  • Olav Karsten Vintermyr
  • Lisbeth Sviland
  • Martin Biermann
Letter to the Editors

Dear Sirs,

Erdheim–Chester disease (ECD) is a rare form of non-Langerhans histiocytosis characterized by multi-systemic xanthogranulomatous infiltration. The disease commonly affects the skeleton, but may also involve the skin, viscera including the cardiovascular system and lungs, as well as the central nervous system [1, 2, 3, 4]. Diagnosis is based on histopathology typically showing infiltration by histiocytes expressing CD68, but not CD1a [3]. In the nervous system, ECD commonly affects the neurohypophysis resulting in diabetes insipidus. Extrahypophyseal nervous system involvement is seen in about a third of ECD patients and includes intra-axial infiltrative lesions, meningioma-like masses, and periarterial infiltration [3, 5, 6]. Spinal cord involvement may occur due to extramedullary masses [3, 7, 8] but intra-axial cord lesions are rare and only a single patient has been described [9]. We report a case of ECD presenting with progressive spastic paraparesis caused by an...

Keywords

Diabetes Insipidus Multinucleated Giant Cell Spastic Paraparesis Cerebellar White Matter Intramedullary Spinal Cord 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Conflicts of interest

The authors declare that they have no competing interests.

Ethical standards

This work has been deemed quality control by our local ethical committee.

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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Charalampos Tzoulis
    • 1
    • 2
    Email author
  • Ivar Otto Gjerde
    • 1
  • Eirik Søfteland
    • 3
    • 2
  • Gesche Neckelmann
    • 4
  • Eivind Strøm
    • 4
  • Olav Karsten Vintermyr
    • 5
  • Lisbeth Sviland
    • 5
  • Martin Biermann
    • 6
  1. 1.Department of NeurologyHaukeland University HospitalBergenNorway
  2. 2.Department of Clinical MedicineUniversity of BergenBergenNorway
  3. 3.Department of MedicineHaukeland University HospitalBergenNorway
  4. 4.Department of RadiologyHaukeland University HospitalBergenNorway
  5. 5.Department of PathologyHaukeland University HospitalBergenNorway
  6. 6.Department of Nuclear MedicineHaukeland University HospitalBergenNorway

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