Pure Relapsing Short Myelitis

Abstract

Background and ObjectivesPure 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.MethodsPatients 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 follow-up; 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.ResultsEighteen 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.DiscussionPure 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.

Diagnostik der Neuromyelitis-optica-Spektrum-Erkrankung (NMOSD) und der MOG-Antikörper-assoziierten Erkrankung (MOGAD)

“…Diese sind bei MOGAD generell – also auch im Akutstadium – häufiger als bei AQP4-IgG+ NMOSD 49 . Das Fehlen einer langstreckigen Läsion sollte daher keinesfalls als Argument dienen, eine NMOSD oder MOGAD vorschnell diagnostisch zu verwerfen 50 , 51 , 52 . Im Verlauf kann es zu einer ausgedehnten Atrophie in den betroffenen Abschnitten kommen 47 .…”

“…In general, this occurs more frequently with MOGAD than with AQP4-IgG+ NMOSD, including in the acute stage of the disease [49]. For this reason, it would be rash to rule out a diagnosis of NMOSD or MOGAD because of the absence of a long cord lesion [50][51][52]. During the course of the disease, extensive atrophy can develop in the affected cord segments [47].…”