Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients
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.
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.
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).
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.
KeywordsMultiple sclerosis Pseudo-tumoral form Balo Schilder Demyelinating disease
Central nervous system
Cerebral spinal fluid
Expanded Disability Status Scale
Fluid Attenuation Inversion Recovery
Magnetic resonance imaging
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.
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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