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Journal of Neurology

, Volume 263, Issue 5, pp 981–990 | Cite as

Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients

  • Cécile-Audrey Durel
  • Romain Marignier
  • Delphine Maucort-Boulch
  • Jean Iwaz
  • Emilie Berthoux
  • Marc Ruivard
  • Marc André
  • Guillaume Le Guenno
  • Laurent Pérard
  • Jean-François Dufour
  • Alin Turcu
  • Jean-Christophe Antoine
  • Jean-Philippe Camdessanche
  • Thierry Delboy
  • Pascal Sève
Original Communication

Abstract

Sarcoidosis of the spinal cord is a rare disease. The aims of this study are to describe the features of spinal cord sarcoidosis (SCS) and identify prognostic markers. We analyzed 20 patients over a 20-year period in 8 French hospitals. There were 12 men (60 %), mostly Caucasian (75 %). The median ages at diagnosis of sarcoidosis and myelitis were 34.5 and 37 years, respectively. SCS revealed sarcoidosis in 12 patients (60 %). Eleven patients presented with motor deficit (55 %) and 9 had sphincter dysfunction (45 %). The median initial Edmus Grading Scale (EGS) score was 2.5. The cerebrospinal fluid (CSF) showed elevated protein level (median: 1.00 g/L, interquartile range (IQR) 0.72–1.97), low glucose level (median 2.84 mmol/L, IQR 1.42–3.45), and elevated white cell count (median 22/mm3, IQR 6–45). The cervical and thoracic cords were most often affected (90 %). All patients received steroids and an immunosuppressive drug was added in 10 cases (50 %). After a mean follow-up of 52.1 months (range 8–43), 18 patients had partial response (90 %), 7 displayed functional impairment (35 %), and the median final EGS score was 1. Six patients experienced relapse (30 %). There was an association between the initial and the final EGS scores (p = 0.006). High CSF protein level showed a trend toward an association with relapse (p = 0.076). The spinal cord lesion was often the presenting feature of sarcoidosis. Most patients experienced clinical improvement with corticosteroids and/or immunosuppressive treatment. The long-term functional prognosis was correlated with the initial severity.

Keywords

Sarcoidosis Neurosarcoidosis Spinal cord sarcoidosis Myelopathy Morbidity Sequelae 

Notes

Compliance with ethical standard

Conflicts of interest

No financial or personal relationships could potentially influence the study and its conclusions.

Ethical standard

According to the French law, this retrospective observational study did not require a Local Institutional Review Board approval

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Cécile-Audrey Durel
    • 1
    • 2
  • Romain Marignier
    • 3
  • Delphine Maucort-Boulch
    • 2
    • 4
    • 5
  • Jean Iwaz
    • 2
    • 4
    • 5
  • Emilie Berthoux
    • 6
  • Marc Ruivard
    • 7
  • Marc André
    • 8
  • Guillaume Le Guenno
    • 7
  • Laurent Pérard
    • 9
  • Jean-François Dufour
    • 10
  • Alin Turcu
    • 11
  • Jean-Christophe Antoine
    • 12
  • Jean-Philippe Camdessanche
    • 12
  • Thierry Delboy
    • 13
  • Pascal Sève
    • 1
    • 2
  1. 1.Département de Médicine Interne, Hôpital de la Croix-RousseHospices Civils de LyonLyonFrance
  2. 2.Université de Lyon, Université Lyon 1VilleurbanneFrance
  3. 3.Département de Neurologie, Hôpital Pierre WertheimerHospices Civils de LyonBronFrance
  4. 4.Service de BiostatistiqueHospices Civils de LyonLyonFrance
  5. 5.CNRS UMR 555, Laboratoire de Biométrie et Biologie Evolutive, Equipe Biostatistique SantéVilleurbanneFrance
  6. 6.Département de Médicine InterneCH Saint Luc Saint JosephLyonFrance
  7. 7.Département de Médecine InterneCHU de Clermont-Ferrand, CHU EstaingClermont-FerrandFrance
  8. 8.Service de Médecine InterneCHU Clermont-Ferrand, Hôpital Gabriel MontpiedClermont-FerrandFrance
  9. 9.Département de Médecine Interne, Hôpital Edouard HerriotHospices Civils de LyonLyonFrance
  10. 10.Département de Médecine InterneCentre hospitalier FleyriatBourg-en-BresseFrance
  11. 11.Département de Médecine Interne et Maladies SystémiquesCHU DijonDijonFrance
  12. 12.Département de NeurologieCHU de Saint-Etienne, Hôpital NordSaint Etienne Cedex 022France
  13. 13.Département de Médecine Interne, CH MontluçonMontluçonFrance

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