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Journal of Neurology

, Volume 265, Issue 10, pp 2251–2259 | Cite as

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

  • G. BalloyEmail author
  • J. Pelletier
  • L. Suchet
  • C. Lebrun
  • M. Cohen
  • P. Vermersch
  • H. Zephir
  • E. Duhin
  • O. Gout
  • R. Deschamps
  • E. Le Page
  • G. Edan
  • P. Labauge
  • C. Carra-Dallieres
  • L. Rumbach
  • E. Berger
  • P. Lejeune
  • P. Devos
  • J.-B. N’Kendjuo
  • M. Coustans
  • E. Auffray-Calvier
  • B. Daumas-Duport
  • L. Michel
  • F. Lefrere
  • D. A. Laplaud
  • C. Brosset
  • P. Derkinderen
  • J. de Seze
  • S. Wiertlewski
  • On behalf of the Société Francophone de la Sclérose en Plaques
Original Communication

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.

Keywords

Multiple sclerosis Pseudo-tumoral form Balo Schilder Demyelinating disease 

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

Notes

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.

Compliance with ethical standards

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.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • G. Balloy
    • 1
    • 16
    Email author
  • J. Pelletier
    • 2
  • L. Suchet
    • 2
  • C. Lebrun
    • 3
  • M. Cohen
    • 3
  • P. Vermersch
    • 4
  • H. Zephir
    • 4
  • E. Duhin
    • 4
  • O. Gout
    • 5
  • R. Deschamps
    • 5
  • E. Le Page
    • 6
  • G. Edan
    • 6
  • P. Labauge
    • 7
  • C. Carra-Dallieres
    • 7
  • L. Rumbach
    • 8
  • E. Berger
    • 8
  • P. Lejeune
    • 9
  • P. Devos
    • 10
  • J.-B. N’Kendjuo
    • 11
  • M. Coustans
    • 13
  • E. Auffray-Calvier
    • 15
  • B. Daumas-Duport
    • 15
  • L. Michel
    • 1
  • F. Lefrere
    • 1
  • D. A. Laplaud
    • 1
  • C. Brosset
    • 12
  • P. Derkinderen
    • 1
  • J. de Seze
    • 14
  • S. Wiertlewski
    • 1
  • On behalf of the Société Francophone de la Sclérose en Plaques
  1. 1.Neurology DepartmentUniversity of Nantes HospitalNantesFrance
  2. 2.Neurosciences Unit, Neurology Department, Timone HospitalAix Marseille University, APHMMarseilleFrance
  3. 3.University of Nice HospitalNiceFrance
  4. 4.University of Lille HospitalLilleFrance
  5. 5.Rothschild FoundationParisFrance
  6. 6.University of Rennes HospitalRennesFrance
  7. 7.University of Montpellier HospitalMontpellierFrance
  8. 8.University Besançon HospitalBesançonFrance
  9. 9.La Roche sur Yon HospitalLa Roche-sur-YonFrance
  10. 10.Boulogne-sur-Mer HospitalBoulogne-sur-MerFrance
  11. 11.Dunkerque HospitalDunkerqueFrance
  12. 12.Military HospitalMarseilleFrance
  13. 13.Cornouaille HospitalQuimperFrance
  14. 14.University of Strasbourg HospitalStrasbourgFrance
  15. 15.Radiology DepartmentUniversity of Nantes HospitalNantesFrance
  16. 16.Service de NeurologieHopital LaennecSaint HerblainFrance

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