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Transverse myelitis: a diagnostic challenge

Tarig Abkur and Mamoun Saeed
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DOI: https://doi.org/10.7861/clinmed.Let.21.6.1
Clin Med November 2021
Tarig Abkur
Senior specialist registrar in neurology, Southmead Hospital, Bristol, UK
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Mamoun Saeed
Senior specialist registrar in neurology, Chelsea and Westminster Hospital, London, UK
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Editor - We read with interest the article published by Notghi and colleagues.1 The authors described a case of transverse myelitis, with symptoms manifesting 7 days post-administration of first dose of the AstraZeneca COVID-19 vaccination. The patient had a history of inactive pulmonary sarcoidosis. Due to the temporal association with the vaccine, lack of evidence supportive of active sarcoidosis on systemic imaging and the poor response to steroid therapy, the authors postulated that the myelitis is post-vaccination rather than a vaccine triggered relapse of isolated neurosarcoidosis. Due to major implications, distinction is crucial.

Post-vaccination immune-mediated response has been implicated in the pathogenesis of several neurological conditions including Guillain-Barré syndrome, acute demyelinating encephalomyelitis and transverse myelitis.2 Similarly, vaccine administration may trigger a flare-up of pre-existing inflammatory conditions. Recent reports have described a role of vaccination in triggering a relapse of chronic immune-mediated conditions including rheumatological diseases, minimal change disease and microscopic polyangiitis.3–5 Taking this point into account would make the temporal link not useful in discriminating between a purely vaccine-induced disease and a vaccine triggered relapse of inactive disease.

From reading the article, we have also noted that the authors felt that lack of active systemic features of sarcoidosis make neurosarcoidosis unlikely. While patients with neurosarcoidosis often have other systems involvement, several studies have reported isolated neurosarcoidosis.6 We have also noted that some crucial investigations have not been performed in this case, including cerebrospinal fluid (CSF) angiotensin converting enzyme (ACE) and CD4/CD8 ratio. Although insensitive, CSF ACE may be reasonably specific for neurosarcoidosis.7 Additionally, combined elevation of CD4/CD8 ratio and CSF lymphocytosis may provide a specificity of 95% for neurosarcoidosis.8 Measuring CSF interleukins may also play a useful role in this clinical context. Finally, the observed clinical improvement is likely due to steroids starting to take effect rather than plasma exchange that would not be expected to exert a theraputic response by day 2 of the treatment cycle.

We suggest close monitoring of this category of patients for features suggestive of re-emergence of quiescent disease, and we strongly advocate discussing such complex cases at specialised neuro-inflammatory multidisciplinary meetings to guide further management.

  • © Royal College of Physicians 2021. All rights reserved.

References

  1. ↵
    1. Notghi AA
    , Atley J, Silva M. Lessons of the month 1: Longitudinal extensive transverse myelitis following AstraZeneca COVID-19 vaccination. Clin Med 2021;21:e535–8.
    OpenUrlAbstract/FREE Full Text
  2. ↵
    1. Leonhard SE
    , Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol 2019;15:671–83.
    OpenUrlCrossRefPubMed
  3. ↵
    1. Machado PM
    , Lawson-Tovey S, Hyrich K, et al. LB0002 COVID-19 vaccine safety in patients with rheumatic and musculoskeletal disease. Annals of the Rheumatic Diseases 2021;80:199–200.
    OpenUrlAbstract/FREE Full Text
    1. Morlidge C
    , El-Kateb S, Jeevaratnam P, Thompson B. Relapse of minimal change disease following the AstraZeneca COVID-19 vaccine. Kidney Int 2021;100:459.
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  4. ↵
    1. Conticini E
    , d'Alessandro M, Bergantini L, et al. Relapse of microscopic polyangiitis after vaccination against COVID-19: A case report. J Med Virol 2021;93:6439–41.
    OpenUrl
  5. ↵
    1. Allen RK
    , Sellars RE, Sandstrom PA. A prospective study of 32 patients with neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2003;20:118–25.
    OpenUrlPubMed
  6. ↵
    1. Khoury J
    , Wellik KE, Demaerschalk BM, Wingerchuk DM. Cerebrospinal fluid angiotensin-converting enzyme for diagnosis of central nervous system sarcoidosis. Neurologist 2009;15:108–11.
    OpenUrlCrossRefPubMed
  7. ↵
    1. Nordström S
    , Andersson B, Malmeström C. Cerebrospinal fluid CD4+ /CD8+ ratio in diagnosing neurosarcoidosis. Acta Neurol Scand 2020;142:480–5.
    OpenUrl
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Transverse myelitis: a diagnostic challenge
Tarig Abkur, Mamoun Saeed
Clinical Medicine Nov 2021, 21 (6) e682; DOI: 10.7861/clinmed.Let.21.6.1

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Transverse myelitis: a diagnostic challenge
Tarig Abkur, Mamoun Saeed
Clinical Medicine Nov 2021, 21 (6) e682; DOI: 10.7861/clinmed.Let.21.6.1
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