Marburg acute multiple sclerosis explained

Marburg acute multiple sclerosis
Synonyms:Acute multiple sclerosis, Marburg type

Marburg acute multiple sclerosis, also known as Marburg multiple sclerosis or acute fulminant multiple sclerosis, is considered one of the multiple sclerosis borderline diseases, which is a collection of diseases classified by some as MS variants and by others as different diseases. Other diseases in this group are neuromyelitis optica (NMO), Balo concentric sclerosis, and Schilder's disease.[1] The graver course is one form of malignant multiple sclerosis, with patients reaching a significant level of disability in less than five years from their first symptoms, often in a matter of months.[2]

Sometimes Marburg MS is considered a synonym for tumefactive MS,[3] but not for all authors.

Pathogenesis

Marburg MS has been reported to be closer to anti-MOG associated ADEM than to standard MS[4] It has been reported to appear sometimes post-partum[5]

MOG antibody‐associated demyelinating pseudotumor

See main article: anti-MOG associated encephalomyelitis.

Some anti-MOG cases satisfy the MS requirements (lesions disseminated in time and space) and are therefore traditionally considered MS cases. After the discovery of the anti-MOG disease this classification is into revision.[6]

Diagnosis

It took its name from Otto Marburg. It can be diagnosed in vivo with an MRI scan.[7] If Marburg disease occurs in the form of a single large lesion, it can be radiologically indistinguishable from a brain tumor or abscess. It is usually lethal, but it has been found to be responsive to Mitoxantrone[8] and Alemtuzumab,[9] and it has also been responsive to autologous stem cell transplantation.[10] Recent evidence shows that Marburg's presents a heterogeneous response to medication, as does standard MS.[11]

Treatment

Historically, acute MS was a fatal disease, with death occurring within a year of onset, often secondary to extensive brainstem demyelination. Treatments include plasma exchange and/or high-dose glucocorticoids(e.g., 1 g/day of methylprednisolone for 3-5 days). Patients that satisfy criteria for MS will be treated with immunomodulatory therapies, often favoring high efficacy monoclonal antibodies.

Prognosis

Marburg variant of MS is an acute fulminant demyelinating process which in most cases progresses inexorably to death within 1–2 years.[12] However, there are some reports of Marburg MS reaching stability by three years.[13]

See also

Notes and References

  1. 11787357 . Les formes frontières de sclérose en plaques . Revue Neurologique . 157 . 8–9 Pt 2 . 929–34 . Borderline forms of multiple sclerosis . fr . 2001 . Fontaine . B. .
  2. 10.1212/WNL.46.4.907 . 8780061 . Defining the clinical course of multiple sclerosis: Results of an international survey . Neurology . 46 . 4 . 907–11 . 1996 . Lublin . F. D. . Reingold . S. C. . 40213123 .
  3. http://radiopaedia.org/cases/tumefactive-multiple-sclerosis-marburg-s-variant See explanation at
  4. Todd A Hardy, Reddel, Barnett, Palace, Lucchinetti, Weinshenker, Atypical CNS inflammatory demyelinating disease, The lancet neurology, August, 2016, DOI:https://doi.org/10.1016/S1474-4422(16)30043-6, Manuscript Number: THELANCETNEUROLOGY-D-16-00113R1 available at https://ora.ox.ac.uk/objects/uuid:49dc3a62-76a4-4b96-9900-a7cd41d5a61a/download_file?file_format=pdf&safe_filename=Atypical%2Binflammatory%2Bdemyelinating%2Bsyndromes%2Bof%2Bthe%2BCNS.pdf&type_of_work=Journal+article
  5. Eduardo Labat et al., An extremely aggressive case of Marburg's disease treated with high dose cyclophosphamide. A case report, Multiple Sclerosis and Related Disorders, Volume 31, June 2019, Pages 51-53, https://doi.org/10.1016/j.msard.2019.03.014
  6. Yaqing Shu Youming Long Shisi Wang Wanming Hu Jian Zhou Huiming Xu Chen Chen Yangmei Ou Zhengqi Lu Alexander Y. Lau Xinhua Yu Allan G. Kermode Wei Qiu, Brain histopathological study and prognosis in MOG antibody‐associated demyelinating pseudotumor, 08 January 2019, https://doi.org/10.1002/acn3.712
  7. Capello E, Mancardi GL . Marburg type and Balò's concentric sclerosis: rare and acute variants of multiple sclerosis . Neurol. Sci. . 25 . Suppl . S361–3 . November 2004 . 15727234 . 10.1007/s10072-004-0341-1 . 12897512 .
  8. 10.1111/j.1552-6569.2004.tb00217.x . 14748210 . Treatment of Marburg Variant Multiple Sclerosis with Mitoxantrone . Journal of Neuroimaging . 14 . 1 . 58–62 . 2004 . Jeffery . Douglas R. . Lefkowitz . David S. . Crittenden . Jeffrey P. . 30709186 .
  9. Gormley KM, Zajicek JP . Alemtuzumab and craniotomy for severe acute demyelinating illness . 16th Meeting of the European Neurological Society . 2006 . dead . https://web.archive.org/web/20071007114035/http://registration.akm.ch/einsicht.php?XNABSTRACT_ID=23714&XNSPRACHE_ID=2&XNKONGRESS_ID=31&XNMASKEN_ID=900 . 2007-10-07 .
  10. 10.1177/1352458507082604 . 17942513 . Autologous stem-cell transplantation in malignant multiple sclerosis: A case with a favorable long-term outcome . Multiple Sclerosis Journal . 14 . 2 . 278–283 . 2008 . Kimiskidis . VK . Sakellari . I. . Tsimourtou . V. . Kapina . V. . Papagiannopoulos . S. . Kazis . D. . Vlaikidis . N. . Anagnostopoulos . A. . Fassas . A. . 42334384 .
  11. 10.1007/s00415-008-0956-x . 18685916 . Rituximab induces clinical stabilization in a patient with fulminant multiple sclerosis not responding to natalizumab . Journal of Neurology . 255 . 9 . 1436–1438 . 2008 . Leussink . V. I. . Lehmann . H. C. . Meyer Zu Hörste . G. . Hartung . H.-P. . Stüve . O. . Kieseier . B. C. . 38328163 .
  12. Book: Dan L. Longo . etal . Harrison's principles of internal medicine.. 2012. McGraw-Hill. New York. 9780071748896. 3407. 18th.
  13. 10.1007/s10072-010-0287-4 . 20461429 . Long survival and clinical stability in Marburg's variant multiple sclerosis . Neurological Sciences . 31 . 6 . 807–811 . 2010 . Turatti . Marco . Gajofatto . Alberto . Rossi . Francesca . Vedovello . Marcella . Benedetti . Maria Donata . 37354295 .

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