Marburg acute multiple sclerosis, also known as Marburg multiple sclerosis or acute fulminantmultiple 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]
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]
^Lublin, F. D.; Reingold, S. C. (1996). "Defining the clinical course of multiple sclerosis: Results of an international survey". Neurology. 46 (4): 907–11.
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^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
[1]
^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
^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
^Capello E, Mancardi GL (November 2004). "Marburg type and Balò's concentric sclerosis: rare and acute variants of multiple sclerosis". Neurol. Sci. 25 (Suppl): S361–3.
doi:
10.1007/s10072-004-0341-1.
PMID15727234.
S2CID12897512.
^Jeffery, Douglas R.; Lefkowitz, David S.; Crittenden, Jeffrey P. (2004). "Treatment of Marburg Variant Multiple Sclerosis with Mitoxantrone". Journal of Neuroimaging. 14 (1): 58–62.
doi:
10.1111/j.1552-6569.2004.tb00217.x.
PMID14748210.
S2CID30709186.
^Kimiskidis, VK; Sakellari, I.; Tsimourtou, V.; Kapina, V.; Papagiannopoulos, S.; Kazis, D.; Vlaikidis, N.; Anagnostopoulos, A.; Fassas, A. (2008). "Autologous stem-cell transplantation in malignant multiple sclerosis: A case with a favorable long-term outcome". Multiple Sclerosis Journal. 14 (2): 278–283.
doi:
10.1177/1352458507082604.
PMID17942513.
S2CID42334384.
^Leussink, V. I.; Lehmann, H. C.; Meyer Zu Hörste, G.; Hartung, H.-P.; Stüve, O.; Kieseier, B. C. (2008). "Rituximab induces clinical stabilization in a patient with fulminant multiple sclerosis not responding to natalizumab". Journal of Neurology. 255 (9): 1436–1438.
doi:
10.1007/s00415-008-0956-x.
PMID18685916.
S2CID38328163.
^Dan L. Longo; et al., eds. (2012). Harrison's principles of internal medicine (18th ed.). New York: McGraw-Hill. p. 3407.
ISBN9780071748896.