Pure Relapsing Short Myelitis

Zélia Poullet (1) , Julie Pique (2) , Adil Maarouf (1, 3, 4) , Clemence Boutiere (1) , Audrey Rico (1, 3, 4) , Sarah Demortiere (1) , Pierre Durozard (1) , Caroline Papeix (5) , Elisabeth Maillart (5) , Nicolas Collongues (6) , Xavier Ayrignac (7) , Helene Zephir (8) , Romain Deschamps (9) , Jonathan Ciron (10) , Jean Pelletier (1, 3, 4) , Romain Marignier (2) , Bertrand Audoin (1, 3, 4)

1 Neurologie, maladies neuro-musculaires [Hôpital de la Timone - APHM]
2 Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL]
3 CRMBM - Centre de résonance magnétique biologique et médicale
4 CEMEREM - Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - APHM]
5 CHU Pitié-Salpêtrière [AP-HP]
6 Service de Neurologie [CHU Strasbourg]
7 CHU Montpellier, Département de Neurologie
8 Université de Lille
9 Fondation Ophtalmologique Adolphe de Rothschild [Paris]
10 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse

Background and Objectives Pure relapsing short myelitis with clinical and paraclinical features suggesting multiple sclerosis (MS) has been described recently. Here, we evaluated the existence of this potential new form of MS by retrospectively searching for similar cases in the databases of the French tertiary MS centers. Methods Patients were included based on the present criteria: at least 2 short (<3 vertebral segments) myelitis episodes; minimum follow-up of 3 years; no MS-like brain lesion during all the followup; tested negative for both anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies in serum; presence of oligoclonal bands in CSF; and comprehensive workup to exclude alternative diagnoses. Results Eighteen patients fulfilled all criteria. The sex ratio (females/males) was 5/1; the median (range) age at first relapse was 35.5 (25-54) years, the disease duration was 80.5 (50-308) months, and the annualized relapse rate was 0.36 (0.1-0.5). The median (range) number of relapses per patient was 2 (2-5), and the median (range) Expanded Disability Status Scale score at last follow-up was 1 (0-7.5). In CSF, the median (range) protein level was 0.34 g/L (0.18-0.77), and the median (range) number of mononuclear cells was 3 (0-28). Spinal cord MRI demonstrated a median (range) number of 2 (1-5) lesions per examination and 3 [1-7] on the last examination. Fifty-five percent of lesions involved the cervical levels. Secondary progressive evolution occurred in 3 of 18 (17%) patients. Discussion Pure spinal MS could be a rare entity in the MS disease spectrum. However, the existence of a distinct entity in the inflammatory CNS disorders cannot be excluded.

Mots clés: All Spinal Cord; All Demyelinating disease (CNS); Multiple sclerosis; MRI; Cerebrospinal Fluid

Domaines: Médecine humaine et pathologie

Zélia Poullet, Julie Pique, Adil Maarouf, Clemence Boutiere, Audrey Rico, et al.. Pure Relapsing Short Myelitis. Neurology Neuroimmunology & Neuroinflammation, 2022, 9 (4), ⟨10.1212/nxi.0000000000001167⟩. ⟨hal-03812521⟩