G Balloy1,2, J Pelletier3, L Suchet3, C Lebrun4, M Cohen4, P Vermersch5, H Zephir5, E Duhin5, O Gout6, R Deschamps6, E Le Page7, G Edan7, P Labauge8, C Carra-Dallieres8, L Rumbach9, E Berger9, P Lejeune10, P Devos11, J-B N'Kendjuo12, M Coustans13, E Auffray-Calvier14, B Daumas-Duport14, L Michel15, F Lefrere15, D A Laplaud15, C Brosset16, P Derkinderen15, J de Seze17, S Wiertlewski15. 1. Neurology Department, University of Nantes Hospital, Nantes, France. gaelle.balloy@chu-nantes.fr. 2. Service de Neurologie, Hopital Laennec, Boulevard Jacques Monod, 44800, Saint Herblain, France. gaelle.balloy@chu-nantes.fr. 3. Neurosciences Unit, Neurology Department, Timone Hospital, Aix Marseille University, APHM, Marseille, France. 4. University of Nice Hospital, Nice, France. 5. University of Lille Hospital, Lille, France. 6. Rothschild Foundation, Paris, France. 7. University of Rennes Hospital, Rennes, France. 8. University of Montpellier Hospital, Montpellier, France. 9. University Besançon Hospital, Besançon, France. 10. La Roche sur Yon Hospital, La Roche-sur-Yon, France. 11. Boulogne-sur-Mer Hospital, Boulogne-sur-Mer, France. 12. Dunkerque Hospital, Dunkerque, France. 13. Cornouaille Hospital, Quimper, France. 14. Radiology Department, University of Nantes Hospital, Nantes, France. 15. Neurology Department, University of Nantes Hospital, Nantes, France. 16. Military Hospital, Marseille, France. 17. University of Strasbourg Hospital, Strasbourg, France.
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.
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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