Marburg acute multiple sclerosis

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.[4]

Diagnosis

It took its name from Otto Marburg. It can be diagnosed in vivo with an MRI scan.[5] 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[6] and Alemtuzumab,[7] and it has also been responsive to autologous stem cell transplantation.[8] Recent evidence shows that Marburg's presents a heterogeneous response to medication, as does standard MS.[9]

Treatment

Historically, acute MS was a fatal disease, with death occurring within a year of onset, often secondary to extensive brain stem demyelination. Treatment recommendations, based on anecdotes, include plasma exchange in conjunction with high-dose glucocorticoids(e.g., 1 to 2 g/day of methylprednisolone for 10 days followed by a slow taper). For patients who survive the acute attack, follow-up treatments include immunosuppression (monthly mitoxantrone or cyclophosphamide) either alone or in combination with IFN beta or GA.[10]

Prognosis

Marburg variant of MS is an acute fulminant demyelinating process which in most cases progresses inexorably to death within 1–2 years.[11] However, there are some reports of Marburg MS reaching stability by three years.[12]

See also

References

  1. ↑ Fontaine B (2001). "Borderline forms of multiple sclerosis". Rev. Neurol. (Paris) (in French). 157 (8–9 Pt 2): 929–34. PMID 11787357.
  2. ↑ Lublin FD, Reingold SC (April 1996). "Defining the clinical course of multiple sclerosis: Results of an international survey". Neurology. 46 (4): 907–11. doi:10.1212/WNL.46.4.907. PMID 8780061.
  3. ↑ See explanation at
  4. ↑ Ayumi Ludwig et al, Marburg’s Variant of Multiple Sclerosis with Extensive Brain Lesions: An Autopsy Case Report. Int J Neurol Neurother 2015, 2:2
  5. ↑ 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. PMID 15727234.
  6. ↑ Jeffery DR, Lefkowitz DS, Crittenden JP (January 2004). "Treatment of Marburg variant multiple sclerosis with mitoxantrone". J Neuroimaging. 14 (1): 58–62. doi:10.1111/j.1552-6569.2004.tb00217.x. PMID 14748210.
  7. ↑ Gormley KM, Zajicek JP (2006). "Alemtuzumab and craniotomy for severe acute demyelinating illness". 16th Meeting of the European Neurological Society. Archived from the original on 2007-10-07.
  8. ↑ Kimiskidis VK, Sakellari I, Tsimourtou V, et al. (2007). "Autologous stem cell transplantation in malignant multiple sclerosis: a case with a favorable long-term outcome". Multiple Sclerosis. 14 (2): 278–83. doi:10.1177/1352458507082604. PMID 17942513.
  9. ↑ Leussink VI, Lehmann HC, Meyer Zu Hörste G, Hartung HP, Stüve O, Kieseier BC (2008). "Rituximab induces clinical stabilization in a patient with fulminant multiple sclerosis not responding to natalizumab: Evidence for disease heterogeneity". Journal of Neurology. 255 (9): 1436–8. doi:10.1007/s00415-008-0956-x. PMID 18685916.
  10. ↑ "Marburg's variant multiple sclerosis - an overview | ScienceDirect Topics". www.sciencedirect.com. Retrieved 2018-05-11.
  11. ↑ Dan L. Longo, et.al, ed. (2012). Harrison's principles of internal medicine (18th ed.). New York: McGraw-Hill. p. 3407. ISBN 9780071748896.
  12. ↑ Turatti, M; Gajofatto, A; Rossi, F; Vedovello, M; Benedetti, MD (Dec 2010). "Long survival and clinical stability in Marburg's variant multiple sclerosis". Neurological Sciences. 31 (6): 807–11. doi:10.1007/s10072-010-0287-4. PMID 20461429.
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