High Dose Methylprednisolone in Fulminant Progressive Quadriparesis with Optic Neurites: The Marburg Variant

Ahmad Ghayas Ansari *

Department of Medicine, Jawaharlal Nehru Medical College, AMU, Aligarh, India.

Ariba

Department of Medicine, Jawaharlal Nehru Medical College, AMU, Aligarh, India.

Lubna Zafar

Department of Medicine, Jawaharlal Nehru Medical College, AMU, Aligarh, India.

*Author to whom correspondence should be addressed.


Abstract

Marburg described an acute variant of Multiple Sclerosis (MS) which was rare with a fulminant demyelinating progressive course with multiple lesions, predominantly in young adults, leading to severe disability and death within a year of onset. The disease was associated with an Antibody-mediated process, acute fulminant course and typical MRI findings which are diagnostic of the disease. High-dose glucocorticoids, cyclophosphamide and mitoxantrone have shown good response to treatment. The malignant form of MS accounts for less than 4% of the incidence. Here the patient had no history or sign of infectious, metabolic, or vascular pathology and thus the diagnosis of Marburg’s variant was made by exclusion. The Marburg variant is rare and needs a high index of suspicion. In the future, studies of patients with tumefactive demyelinating lesions are necessary for early diagnosis, greater understanding of the disease and for the development of therapeutic strategies.

Keywords: Multiple sclerosis, marburg variant, methylprednisolone


How to Cite

Ansari , A. G., Ariba, & Zafar , L. (2023). High Dose Methylprednisolone in Fulminant Progressive Quadriparesis with Optic Neurites: The Marburg Variant. Asian Journal of Research and Reports in Neurology, 6(1), 116–120. Retrieved from https://journalajorrin.com/index.php/AJORRIN/article/view/86


References

Johnson MD, Lavin P, Whetsell WO. Fulminant monophasic multiple sclerosis, Marburg’s type. J Neurol Neurosurg Psychiatry. 1990;53(10):918–21.

Ludwig A. Marburg’s variant of multiple sclerosis with extensive brain lesions: an autopsy case report. Int J Neurol Neurother [Internet]; 2015. [cited 2023 Aug 19];2(1). Available:https://clinmedjournals.org/articles/ijnn/ijnn-2-027.php?jid=ijnn

Vakrakou AG, Brinia ME, Svolaki I, Argyrakos T, Stefanis L, Kilidireas C. Immunopathology of tumefactive demyelinating lesions-from idiopathic to drug-related cases. Front Neurol. 2022;13:868525.

Walid MS, Sanoufa M. The diagnosis of Marburg disease is course-dependent. GMS Ger Med Sci 8Doc06 ISSN 1612-3174 [Internet]; 2010. [cited 2023 Aug 19]; Available:http://www.egms.de/en/gms/2010-8/000095.shtml

Nozaki K, Abou-Fayssal N. High dose cyclophosphamide treatment in Marburg variant multiple sclerosis. J Neurol Sci. 2010;296(1–2):121–3.

Manuel A, Vasudevan MC. A case of marburg’s variant of multiple sclerosis successfully treated with IVIg and mitoxantrone. Ann Indian Acad Neurol. 2021;24(1):92–4.

Capello E, Mancardi GL. Marburg type and Balos concentric sclerosis: rare and acute variants of multiple sclerosis. Neurol Sci. 2004;25(S4):s361–3.

Aliaga ES, Barkhof F. Chapter 13 - MRI mimics of multiple sclerosis. In: Goodin DS, editor. Multiple Sclerosis and Related Disorders [Internet]. Elsevier. 2014;291–316. (Handbook of Clinical Neurology; vol. 122).

Available:https://www.sciencedirect.com/science/article/pii/B9780444520012000121

Sarbu N, Shih RY, Jones RV, Horkayne-Szakaly I, Oleaga L, Smirniotopoulos JG. White matter diseases with radiologic-pathologic correlation. Radio Graphics. 2016;36(5):1426–47.

Ömerhoca S, Akkaş SY, İçen NK. Multiple sclerosis: diagnosis and differential diagnosis. Noro Psikiyatr Ars. 2018;55(Suppl 1):S1-S9. DOI: 10.29399/npa.23418. PMID: 30692847; PMCID: PMC6278620.

Borisow N, Mori M, Kuwabara S, Scheel M, Paul F. Diagnosis and treatment of NMO spectrum disorder and MOG-encephalomyelitis. Front Neurol. 2018;9:888.