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Improving myelopathy diagnosis now and into the future

  • Laura Cacciaguerra
    Affiliations
    Department of Neurology, Mayo Clinic, Rochester, MN, USA.

    Vita-Salute San Raffaele University, Milan, Italy.

    Neuroimaging Research Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy.
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  • Eoin P. Flanagan
    Correspondence
    Corresponding author at: Mayo Clinic College of Medicine, Rochester, MN, USA.
    Affiliations
    Department of Neurology, Mayo Clinic, Rochester, MN, USA.

    Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
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Published:September 30, 2022DOI:https://doi.org/10.1016/j.jns.2022.120424
      Patients with spinal cord disease are encountered across a wide variety of settings, from emergency departments to neuroimmunology clinics and misdiagnosis of myelopathies is common [
      • Zalewski N.L.
      • Flanagan E.P.
      • Keegan B.M.
      Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses.
      ]. As spinal cord damage develops fast and may quickly become irreversible without disease-specific treatment, myelopathy misdiagnosis is particularly problematic. Thus, the availability of tools that could guide physicians to a correct diagnosis more rapidly represent a major unmet need. In the current issue of Journal of The Neurological Sciences, Murphy and colleagues studied an impressively large cohort of 1193 individuals referred to the Johns Hopkins transverse myelitis clinic with a working diagnosis of “transverse myelitis” [
      • Murphy O.C.
      • Barreras P.
      • Villabona-Rueda A.
      • Mealy M.
      • Pardo C.A.
      Identification of specific causes of myelopathy in a large cohort of patients initially diagnosed with transverse myelitis.
      ]. They analyzed the demographics, clinical features, and final diagnoses of these patients to try identify clues to the etiology with a focus on distinguishing inflammatory from non-inflammatory myelopathies. Despite “transverse myelitis” defining an inflammatory syndrome, the authors found that 35% referred with this diagnosis had a primarily non-inflammatory myelopathy with spinal cord infarction the commonest mimic accounting for 17% of referrals [
      • Murphy O.C.
      • Barreras P.
      • Villabona-Rueda A.
      • Mealy M.
      • Pardo C.A.
      Identification of specific causes of myelopathy in a large cohort of patients initially diagnosed with transverse myelitis.
      ]. With inflammatory myelopathies, symptom onset was usually acute or subacute and the most frequent defined diseases identified were multiple sclerosis, aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorders and infectious/post-infectious myelopathies. Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) accounted for 1% of their cohort [
      • Murphy O.C.
      • Barreras P.
      • Villabona-Rueda A.
      • Mealy M.
      • Pardo C.A.
      Identification of specific causes of myelopathy in a large cohort of patients initially diagnosed with transverse myelitis.
      ], but was likely under-represented given its only recent discovery as illustrated by it accounted for a higher proportion (3.4%) when assessing its frequency in the timeframe for which MOG antibody testing was available [
      • Murphy O.C.
      • Barreras P.
      • Villabona-Rueda A.
      • Mealy M.
      • Pardo C.A.
      Identification of specific causes of myelopathy in a large cohort of patients initially diagnosed with transverse myelitis.
      ]. In prior studies MOGAD accounted for 4.7–6.6% of transverse myelitis cases and thus both MOG and aquaporin-4 antibodies are crucial investigations when evaluating transverse myelitis [
      • Kim K.H.
      • Kim S.H.
      • Hyun J.W.
      • Kim Y.
      • Park H.
      • Kim H.J.
      Seroprevalence of anti-myelin oligodendrocyte glycoprotein antibodies in adults with myelitis.
      ,
      • Sechi E.
      • Shosha E.
      • Williams J.P.
      • et al.
      Aquaporin-4 and MOG autoantibody discovery in idiopathic transverse myelitis epidemiology.
      ].

      Keywords

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