Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center
  • Home
  • Articles
  • Issues
  • COVID-19 Article Hub
  • Infographics & Video Summaries

User menu

  • My Alerts
  • Log in

Search

  • Advanced search
Neurology Neuroimmunology & Neuroinflammation
Home
A peer-reviewed clinical and translational neurology open access journal
  • My Alerts
  • Log in
Site Logo
  • Home
  • Articles
  • Issues
  • COVID-19 Article Hub
  • Infographics & Video Summaries

Share

July 2022; 9 (4) Research ArticleOpen Access

Pure Relapsing Short Myelitis

Part of the Multiple Sclerosis Spectrum or New Entity?

Zélia Poullet, Julie Pique, Adil Maarouf, Clemence Boutiere, Audrey Rico, Sarah Demortiere, View ORCID ProfilePierre Durozard, Caroline Papeix, View ORCID ProfileElisabeth Maillart, View ORCID ProfileNicolas Collongues, View ORCID ProfileXavier Ayrignac, Helene Zephir, View ORCID ProfileRomain Deschamps, View ORCID ProfileJonathan Ciron, Jean Pelletier, Romain Marignier, View ORCID ProfileBertrand Audoin
First published April 26, 2022, DOI: https://doi.org/10.1212/NXI.0000000000001167
Zélia Poullet
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: zelia.poullet@ap-hm.fr
Julie Pique
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: julie.pique@chu-lyon.fr
Adil Maarouf
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: adil.maarouf@ap-hm.fr
Clemence Boutiere
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: clemence.boutiere@ap-hm.fr
Audrey Rico
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: audrey.rico@ap-hm.fr
Sarah Demortiere
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: sarah.demortiere@ap-hm.fr
Pierre Durozard
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Pierre Durozard
  • For correspondence: pierre.durozard@ap-hm.fr
Caroline Papeix
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: caroline.papeix@aphp.fr
Elisabeth Maillart
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Elisabeth Maillart
  • For correspondence: elisabeth.maillart@aphp.fr
Nicolas Collongues
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Nicolas Collongues
  • For correspondence: nicolas.collongues@chru-strasbourg.fr
Xavier Ayrignac
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Xavier Ayrignac
  • For correspondence: xavier.ayrignac@yahoo.fr
Helene Zephir
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: helene.zephir@chru-lille.fr
Romain Deschamps
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Romain Deschamps
  • For correspondence: rdeschamps@for.paris
Jonathan Ciron
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Jonathan Ciron
  • For correspondence: ciron.j@chu-toulouse.fr
Jean Pelletier
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: jean.pelletier@ap-hm.fr
Romain Marignier
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: romain.marignier@chu-lyon.fr
Bertrand Audoin
From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Bertrand Audoin
Full PDF
Citation
Pure Relapsing Short Myelitis
Part of the Multiple Sclerosis Spectrum or New Entity?
Zélia Poullet, Julie Pique, Adil Maarouf, Clemence Boutiere, Audrey Rico, Sarah Demortiere, Pierre Durozard, Caroline Papeix, Elisabeth Maillart, Nicolas Collongues, Xavier Ayrignac, Helene Zephir, Romain Deschamps, Jonathan Ciron, Jean Pelletier, Romain Marignier, Bertrand Audoin
Neurol Neuroimmunol Neuroinflamm Jul 2022, 9 (4) e1167; DOI: 10.1212/NXI.0000000000001167

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
701

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

Abstract

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 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.

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.

Glossary

anti-AQP4=
anti–aquaporin 4;
anti-MOG=
anti–myelin oligodendrocyte glycoprotein;
ARR=
annualized relapse rate;
DMT=
disease-modifying therapy;
EDSS=
Expanded Disability Status Scale;
MS=
multiple sclerosis;
NOMADMUS=
Neuromyelitis Optica Study Group in France;
OCB=
oligoclonal band

Cases of pure relapsing short myelitis with clinical and paraclinical features suggesting multiple sclerosis (MS) have been reported recently.1 Better description of this potential new form of MS is of particular importance because nowadays these patients do not fulfill international diagnostic criteria of MS2 and could be consequently excluded from effective therapeutic strategies. Here, we evaluated the existence of this potential new form of MS by retrospectively searching for similar cases in the databases of the French centers involved in neuromyelitis optica and associated neurologic disorders (NOMADMUS) network.

Methods

Protocol and Participants

To be included, patients had to fulfill the following criteria: age >18 years at inclusion; evidence of at least 2 short (<3 vertebral segments) myelitis episodes; minimum follow-up of 3 years; no typical MS-like brain lesion during all the follow-up; no clinical history or visual evoked potential or eye examination suggesting prior optic neuritis; no history of clinical episode suggesting brain lesion; tested negative for both anti–myelin oligodendrocyte glycoprotein (anti-MOG) and anti–aquaporin 4 (anti-AQP4) antibodies in serum; presence of oligoclonal bands (OCBs) in CSF; and comprehensive workup to exclude alternative diagnoses of myelitis, namely, infections, vascular diseases, and subacute combined degeneration of spinal cord and autoimmune diseases.3,-,5 Particularly, other inflammatory causes of myelitis, including sarcoidosis, Behcet disease, paraneoplastic disorders, and connective tissue diseases, were excluded by using biological and imaging explorations. Anonymized centralized (Marseille) reinterpretation of brain MRIs by expert neurologists (consensus required among B.A., J.P., A.M., A.R., and C.B.) was performed to exclude all patients with any typical MS-like brain lesion.6

Standard Protocol Approvals, Registrations, and Patient Consent

The authors obtained ethical approval of national ethical authority (NOMADMUS cohort, CNIL decision DR-2014-558) to conduct the present study. Each participant gave free and informed written consent for anonymized use of clinical, MRI, and biological data for research purposes.

Data Availability

All data analyzed during this study will be shared anonymized by reasonable request of a qualified investigator to the corresponding author.

Results

Among 62 patients first screened in the French tertiary MS centers, 18 fulfilled all inclusion criteria (Figure 1).

Figure 1
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 1 Flowchart of the Patients Included

Clinical Features

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 (ARR) 0.36 (0.1–0.5) (Table 1). The median (range) number of relapses per patient was 2 (2–5), and the median (range) Expanded Disability Status Scale (EDSS) score during relapse and at last follow-up was 2.5 (0.5–5.5) and 1 (0–7.5), respectively. Among the 50 relapse cases, 24 (48%) showed pure sensitive signs (paresthesia, numbness, or proprioceptive ataxia); 11 (22%) sensitive and motor signs (arm or lower limb weakness); 7 (14%) sensitive, motor, and sphincter signs; 4 (8%) pure motor signs; 2 (4%) motor and sphincter signs; 1 (2%) sensitive and sphincter signs; and 1 (2%) pure sphincter signs. Of the 50 relapse cases, 30 (60%) involved the 4 limbs and 20 (40%) the lower limbs only.

View this table:
  • View inline
  • View popup
  • Download powerpoint
Table 1

Clinical and MRI Evolution of Patients

Laboratory Findings

No patient presented atypical CSF findings for MS. In CSF, the median (range) protein level was 0.34 (0.18–0.77) g/L, and the median (range) number of mononuclear cells was 3 (0–28). Nine of 18 patients were tested at least twice for anti-MOG and anti-AQP4 antibodies, 5 of 9 were tested twice, 3 of 9 three times, and 1 of 9 four times.

Brain and Spinal Cord MRI

No typical MS-like brain lesion was detected in any patient despite repeat examination (median [range] number of brain MRI examinations per patient 4 [2–9]) (Figure 2). Importantly, 3D fluid-attenuated inversion recovery imaging was available for 15 of 18 patients. A median of 5 (2–6) spinal cord MRI examinations were performed per patient. All spinal cord MRI examinations included sagittal T2/STIR sequences. At least 1 series of axial sequences was available for 8 of 18 patients. Spinal cord MRI demonstrated a median (range) number of 2 (1–5) lesions per examination and a median (range) number of 3 (1–7) lesions on the last examination. The median (range) sagittal extension of the spinal cord lesions was 1 (0.5–2) vertebral segments. Among all spinal cord lesions (n = 67) depicted, 37 (55%) and 30 (45%) involved the cervical and thoracolumbar levels, respectively. Gadolinium injection was performed in 45 examinations, and 21 of 46 (45%) lesions showed gadolinium enhancement. Overall, 18 lesions in 9 patients were explored on the axial plane, and 12 (67%) showed partial myelitis (Figure 3).

Figure 2
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 2 Last Brain and Spinal Cord MRI of the Patients

The patient's number is displayed in each image.

Figure 3
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 3 Axial View of Spinal Cord Lesion Performed in 8 of 18 Patients

Treatment and Progress

In all, 30 of 50 (60%) relapse cases were treated with high-dose IV corticosteroids. The median (range) EDSS score during relapse and after recovery was 2.5 (0.5–5.5) and 1 (0–4.5), respectively.

View this table:
  • View inline
  • View popup
  • Download powerpoint
Table 2

Characteristics and Evolution of Treated Patients (n = 12)

Disease-modifying therapy (DMT) was used in 12 of 18 (67%) patients (Table 2). In these patients, the median (range) follow-up before and after DMT onset was 55.5 (3–191) and 40 (4–248) months, respectively. The median (range) ARR before and after DMT onset was 0.5 (0.1–1) and 0 (0–0.5), respectively, after excluding patients with a follow-up <6 months before or after DMT onset. Seven of 11 (63%) patients were free from relapse after DMT onset. The median EDSS (range) score at DMT onset and at last follow-up was 2 (0–6) and 2.5 (0–7.5), respectively.

Secondary progressive evolution occurred in 3 of 18 (17%) patients. The median (range) follow-up of these patients was 242 (236–308) vs 64 (36–296) months for other patients. The median (range) EDSS score of these patients was 7 (6–7.5) vs 1.5 (0–4.5) for other patients.

Discussion

The present study including the French centers involved in the NOMADMUS network reports 18 cases of pure relapsing short myelitis. According to the retrospective design of the study, the number of cases is probably underestimated, which prevents any conclusion about the prevalence of pure relapsing short myelitis. In addition, some cases were probably not reported because they never experienced a second relapse in that treatment onset occurred just after the first myelitis. However, this therapeutic attitude is highly unusual in France, which limits the number of potential underreported cases.

Because all patients included in the present study had to have OCBs in the CSF, we paid careful attention to searching and excluding all known causes of inflammatory myelitis. Moreover, we also excluded vitamin B12 deficiency in terms of its frequency. However, if a search for other rare metabolic causes of myelopathy such as copper deficiency was not available in the medical chart, patients were not excluded because radiologic and CSF findings did not suggest metabolic disorders.

Several features of these cases argue for the existence of a pure spinal form of MS. First, the characteristics of myelitis highly suggest relapsing MS: no clinical presentation suggested transverse myelitis, clinical presentations mostly suggested an involvement of the posterior part of the spinal cord, spinal cord MRI demonstrating most partial and posterior myelitis in the axial plane. Second, all patients showed typical CSF findings for MS. Third, in all patients receiving MS DMTs, disease activity decreased. Fourth, 17% of patients showed secondary progression—an evolution highly suggestive of MS—several years after disease onset. Finally, we were not able to provide a better explanation than MS in all patients despite extensive explorations. In that way, we recommend preferentially using DMTs with demonstrated efficacy in MS to treat pure relapsing short myelitis.

Nevertheless, several features may argue for the existence of a possible distinct inflammatory entity. First, the sex ratio was more imbalanced in favor of females as compared with MS. Second, it is unexpected in a pathologic perspective that brain involvement could be totally absent after several years of evolution with MS.

Whatever the nosological classification, the existence of patients with pure relapsing short myelitis argues for systematically adding spinal cord MRI to brain MRI for the imaging surveillance of patients followed after an isolated myelitis episode with OCBs in the CSF. For patients with recurrent myelitis, we recommend performing imaging at least annually as recommended for MS but systematically adding spinal cord imaging to brain imaging. According to the relative low disease activity evidenced in the patients reported here, we do not recommend exceeding annual imaging in the absence of relapse or therapeutic considerations.

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. Future studies are needed to disentangle these 2 interpretations.

Study Funding

No targeted funding reported.

Disclosure

Dr. Poullet reports no disclosures relevant to the manuscript. Go to Neurology.org/NN for full disclosures.

Appendix Authors

Table

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • The Article Processing Charge was funded by the authors.

  • Submitted and externally peer reviewed. The handling editor was Friedemann Paul, MD.

  • Received December 6, 2021.
  • Accepted in final form March 7, 2022.
  • Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

References

  1. 1.↵
    1. Schee JP,
    2. Viswanathan S
    . Pure spinal multiple sclerosis: a possible novel entity within the multiple sclerosis disease spectrum. Mult Scler. 2019;25(8):1189-1195.
    OpenUrl
  2. 2.↵
    1. Thompson AJ,
    2. Banwell BL,
    3. Barkhof F, et al.
    Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 2018;17(2):162-173.
    OpenUrlCrossRefPubMed
  3. 3.↵
    1. Charil A,
    2. Yousry TA,
    3. Rovaris M, et al.
    MRI and the diagnosis of multiple sclerosis: expanding the concept of “no better explanation”. Lancet Neurol. 2006;5(10):841-852.
    OpenUrlCrossRefPubMed
  4. 4.↵
    1. Hardy TA,
    2. Reddel SW,
    3. Barnett MH,
    4. Palace J,
    5. Lucchinetti CF,
    6. Weinshenker BG
    . Atypical inflammatory demyelinating syndromes of the CNS. Lancet Neurol. 2016;15(9):967-981.
    OpenUrl
  5. 5.↵
    1. Calabrese M,
    2. Gasperini C,
    3. Tortorella C, et al.
    Better explanations” in multiple sclerosis diagnostic workup. Neurology. 2019;92(22):e2527-e2537.
    OpenUrl
  6. 6.↵
    1. Wattjes MP,
    2. Ciccarelli O,
    3. Reich DS, et al.
    MAGNIFY-CMSC-NAIMS consensus recommendations on the use of MRI in patients with multiple sclerosis. Lancet Neurol. 2021;20(8):653-670.
    OpenUrlCrossRefPubMed

Letters: Rapid online correspondence

  • Reader Response: Pure Relapsing Short Myelitis: Part of the Multiple Sclerosis Spectrum or New Entity?
    • Juichi Fujimori, Neurologist, Tohoku Medical and Pharmaceutical University
    • Ichiro Nakashima, Neurologist, Division of Neurology, Tohoku Medical and Pharmaceutical University
    Submitted May 04, 2022
Comment

REQUIREMENTS

If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org

Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.

If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.

Submission specifications:

  • Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
  • Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
  • Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • Abstract
    • Glossary
    • Methods
    • Results
    • Discussion
    • Study Funding
    • Disclosure
    • Appendix Authors
    • Footnotes
    • References
  • Figures & Data
  • Info & Disclosures
Advertisement

Preferences and User Experiences of Wearable Devices in Epilepsy A Systematic Review and Mixed-Methods Synthesis

Dr. Daniel Friedman and Dr. Sharon Chiang

► Watch

Related Articles

  • No related articles found.

Topics Discussed

  • All Spinal Cord
  • All Demyelinating disease (CNS)
  • Multiple sclerosis
  • MRI
  • Cerebrospinal Fluid

Alert Me

  • Alert me when eletters are published
Neurology - Neuroimmunology Neuroinflammation: 10 (2)

Articles

  • Articles
  • Issues
  • Popular Articles

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Education
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology: Neuroimmunology & Neuroinflammation
Online ISSN: 2332-7812

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise