Atypical Multiple Sclerosis and Idiopathic Inflammatory Demyelinating Diseases in a series of 74 consecutive cases
ECTRIMS Online Library. Labauge P. Oct 25, 2017; 199323
Pierre Labauge
Pierre Labauge
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Abstract: EP1302

Type: ePoster

Abstract Category: Clinical aspects of MS - 2 MS Variants

Objectives: To classify patients with atypical MS findings and Idiopathic Inflammatory Demyelinating Diseases (IIDD).
Background: More and more cases have been recognized with atypical clinical or MR findings, suggesting atypical MS diagnosis or (IIDD).
Material and methods: Since 2007, we conducted a nationwide study to collect all the patients with atypical MS / IIDD. Patients have been categorized into 6 groups as previously described: cavitary MS (defined by large area of hypointensities within high intensities on Flair sequences), solitary sclerosis ( isolated lesion involving the upper cervical spinal cord / medulla junction), ADEM like lesions (defined by a first clinical episode with MRI showing extensive enhanced lesions by gadolinium injection), tumoral demyelinating lesion (TDL) (isolated lesion > 20 mm diameter of the white matter). Two other IIDD types are identified: “MS with normal brain MRI” type (defined by a persistent normal brain MRI despite clinical temporal and spatial dissemination) and “pseudo-leukodystrophy MS” (defined by symmetrical demyelinating lesions on the initial brain MRI).
Results: 74 patients met the inclusion criteria. Cavitary MS consist of 11 cases (F: 7 / M: 4, mean age of onset: 41.0, range: 30-52), characterized by a progressive course in 10/11 with a mean EDSS score of 6.4 (range 2-8). Solitary sclerosis included 4 cases (1F / 3M), mean age 32.3 (28-39), TDL group consisted of 25 cases (19F / 6M), mean age: 34.3 (18-65). During a mean follow of 70.1 (6-181) months, 36 % fulfilled McDonald 2010 MS criteria. 76% did not present any clinical relapse. Normal brain MR group consisted of 12 cases (F 9/M 3; mean age: 29.3yo, range: 12-46). All of them presented spatial and temporal dissemination, but none fulfilled Wingerchuk 2015 criteria for seronegative NMOSD. Initial symptoms included optic neuritis in 5, myelitis in 5 and brainstem related symptoms in 2. Pseudoleukodystrophy MS consisted of 10 cases (4F/6M, mean age: 35.0 yo, range: 16-47), with a progressive evolution in 7/10 of these cases and a mean EDSS score of 4.7 (range 0-9). ADEM group consists of 12 cases with multiple enhanced lesions. Careful analysis of the MRI led to objective a few lesions without gadolinium enhancement in 11/12 . 9/12 presented occurrence of further relapses.
Conclusions: Spectrum of atypical MS and IIDD is enlarged in this series. These results lead to extend the previous classification of IIDD / atypical MS.
Pierre Labauge: nothing to disclose
Pekes Codjia: nothing to disclose
Xavier Ayrignac: nothing to disclose
Mikael Cohen: nothing to disclose
Clarisse Carra Dalliere: nothing to disclose
Mahmoud Charif: nothing to disclose
Anais Lippi: nothing to disclose
Nicolas Collongues: has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva;
Lucas Corti: nothing to disclose
Francoise Durand Dubief has received funding for travel and honoraria Bayer-Schering, Biogen, Genzyme, Novartis, Merck, Roche, Sanofi Aventis and Teva Pharma.
Jerome de Seze: nothing to disclose
Christine Lebrun: nothing to disclose

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