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Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients

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Abstract

Background

Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo’s concentric sclerosis, Schilder’s disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients.

Methods

Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively. Inclusion criteria were (1) a first clinical event (2) MRI showing one or more large demyelinating lesions (20 mm or more in diameter) with mass-like features. Patients with a previous demyelinating event (i.e. confirmed multiple sclerosis) were excluded.

Results

Mean age at onset was 26 years. The most common initial symptoms (67% of the patients) were hemiparesis or hemiplegia. Aphasia, headache and cognitive disturbances (i.e. atypical symptoms for demyelinating diseases) were observed in 15, 18 and 15% of patients, respectively. The mean largest diameter of the tumefactive lesions was 26.9 mm, with gadolinium enhancement in 66 patients (81%). Twenty-one patients (24%) had a single tumefactive lesion. During follow-up (median time 5.7 years) 4 patients died, 70 patients improved or remained stable and 12 worsened. 86% of patients received initial corticosteroid treatment, and 73% received disease-modifying therapy subsequently. EDSS at the end of the follow-up was 2.4 ± 2.6 (mean ± SD).

Conclusion

This study provides further evidence that the clinical course of MS presenting with large focal tumor-like lesions does not differ from that of classical relapsing-remitting MS, once the noisy first relapsing occurred.

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Abbreviations

CNS:

Central nervous system

CSF:

Cerebral spinal fluid

DMT:

Disease-modifying-treatment

EDSS:

Expanded Disability Status Scale

FLAIR:

Fluid Attenuation Inversion Recovery

MRI:

Magnetic resonance imaging

MS:

Multiple sclerosis

OCB:

Oligoclonal bands

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Acknowledgements

The Société Francophone de la Sclérose en Plaques: J. Pelletier, L. Suchet, C. Lebrun, M. Cohen, P. Vermersch, H. Zephir, E. Duhin, O. Gout, R. Deschamps, E. Le Page, G. Edan, L. Michel, P. Labauge, C. Carra Dallieres, E. Berger, P. Lejeune, P. Devos, MD, M. Coustans, J. de Seze, D.A. Laplaud, S. Wiertlewski.

Author information

Author notes

  1. J. de Seze and S. Wiertlewski contributed equally to the manuscript.

Authors and Affiliations

  1. Neurology Department, University of Nantes Hospital, Nantes, France

    G. Balloy, L. Michel, F. Lefrere, D. A. Laplaud, P. Derkinderen & S. Wiertlewski

  2. Neurosciences Unit, Neurology Department, Timone Hospital, Aix Marseille University, APHM, Marseille, France

    J. Pelletier & L. Suchet

  3. University of Nice Hospital, Nice, France

    C. Lebrun & M. Cohen

  4. University of Lille Hospital, Lille, France

    P. Vermersch, H. Zephir & E. Duhin

  5. Rothschild Foundation, Paris, France

    O. Gout & R. Deschamps

  6. University of Rennes Hospital, Rennes, France

    E. Le Page & G. Edan

  7. University of Montpellier Hospital, Montpellier, France

    P. Labauge & C. Carra-Dallieres

  8. University Besançon Hospital, Besançon, France

    L. Rumbach & E. Berger

  9. La Roche sur Yon Hospital, La Roche-sur-Yon, France

    P. Lejeune

  10. Boulogne-sur-Mer Hospital, Boulogne-sur-Mer, France

    P. Devos

  11. Dunkerque Hospital, Dunkerque, France

    J.-B. N’Kendjuo

  12. Military Hospital, Marseille, France

    C. Brosset

  13. Cornouaille Hospital, Quimper, France

    M. Coustans

  14. University of Strasbourg Hospital, Strasbourg, France

    J. de Seze

  15. Radiology Department, University of Nantes Hospital, Nantes, France

    E. Auffray-Calvier & B. Daumas-Duport

  16. Service de Neurologie, Hopital Laennec, Boulevard Jacques Monod, 44800, Saint Herblain, France

    G. Balloy

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Consortia

On behalf of the Société Francophone de la Sclérose en Plaques

  • J. Pelletier
  • , C. Lebrun
  • , M. Cohen
  • , P. Vermersch
  • , H. Zephir
  • , E. Duhin
  • , O. Gout
  • , R. Deschamps
  • , E. Le Page
  • , G. Edan
  • , L. Michel
  • , P. Labauge
  • , C. Carra Dallieres
  • , E. Berger
  • , P. Lejeune
  • , P. Devos
  • , M. Coustans
  • , J. de Seze
  • , D. A. Laplaud
  •  & S. Wiertlewski

Corresponding author

Correspondence to G. Balloy.

Ethics declarations

Conflicts of interest

The authors declare that they have no competing interests.

Ethical approval

All procedures performed in this study 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.

Additional information

L. Rumbach: Deceased.

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Balloy, G., Pelletier, J., Suchet, L. et al. Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients. J Neurol 265, 2251–2259 (2018). https://doi.org/10.1007/s00415-018-8984-7

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  • DOI: https://doi.org/10.1007/s00415-018-8984-7

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