Pure Spinal Multiple Sclerosis

What is it about?

We identified 5 young female Asian patients with the possible new / novel entity within the multiple sclerosis (MS) disease spectrum. These patients presented with relapsing / recurrent short segment partial myelitis with no optic neuritis nor typical MS-like brain lesions.They fulfilled the dissemination in time criteria of the McDonald criteria 2017. They demonstrated dissemination in space within the spinal cords with the multiple lesions at different cord levels in the cervical and thoracic cords. In addition, they demonstrated features suggesting an MS-type pathology while making neuromyelitis optica spectrum disorder and other idiopathic inflammatory demyelinating diseases unlikely, namely (1) peripheral instead of central cord lesion, (2) absence of longitudinal extensive transverse myelitis, optic neuritis, area postrema syndrome and encephalopathy, (3) positive CSF oligoclonal bands, (4) repeatedly negative anti-AQP4 antibody, (5) negative anti-MOG antibody, (6) attacks with moderate severity but good recovery, (&) good response to MS disease modifying therapies, (8) presence of progressive course in some. Anti-MOG related pathology was ruled out as well.

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Why is it important?

Our study described the phenotype of an atypical central demyelinating disorders, i.e. possible new entity within the MS disease spectrum. Such phenotype had never been reported prior to the publication of our study. Hence a proper definition and establishment of such diagnosis with subsequent categorization will be essential in the diagnosis and prompt, appropriate management.