head JofIMAB
Journal of IMAB - Annual Proceeding (Scientific Papers)
Publisher: Peytchinski Publishing Ltd.
ISSN: 1312-773X (Online)
Issue: 2023, vol. 29, issue2
Subject Area: Medicine
DOI: 10.5272/jimab.2023292.4943
Published online: 05 June 2023

Case report
J of IMAB. 2023 Apr-Jun;29(2):4943-4946
Magdalina Yanakieva1ORCID logoCorresponding Autoremail, Maya Danovska1ORCID logo, Emilia Ovcharova1ORCID logo, Diana Marinova1ORCID logo, Nicholas Shepherd2ORCID logo,
1) Department of Neurology, Faculty of Medicine, Medical University – Pleven, Bulgaria.
2) Department of Acute Medicine, Northampton General Hospital, UK.

Purpose: To present a clinical case of tumefactive multiple sclerosis (TMS), which is an inflammatory demyelinating disease of the central nervous system considered to be a rare form of multiple sclerosis (MS). It belongs to the group of borderline forms of MS – a collective term used to define a spectrum of demyelination-associated neurological conditions that share similar clinical, neuroimaging and histopathological features but vary widely in severity, clinical course and outcome.
Materials/Methods: We describe the case of a 31-year-old female who was admitted to the Neurology clinic of UMHAT “Dr Georgi Stranski” in Pleven, Bulgaria, with a rapid onset of neurological deficit including right-sided hemiparesis, dysarthria, imbalance, cognitive impairment and urinary incontinence. MRI of the brain showed several tumor-like concentric lesions of demyelination surrounded by moderate brain edema, consistent with the radiological criteria for the demyelinating disease.
Results: High-dosage corticosteroids were applied intravenously for this patient as acute therapy. A progressive improvement in the patient was achieved after the extended pulse corticosteroid therapy in combination with physiotherapy. Glatiramer acetate as a disease-modifying treatment was initiated within three months and had substantial efficacy.
Conclusions: The diagnosis of TMS is always difficult and requires not only complex clinical and neuroimaging investigations but also an extensive follow-up of the patient. It is believed that TMS usually has a progressive course and an unfavorable outcome, but a relapsing-remitting course of TMS, albeit rare, is also possible. Our case report confirms that such benign variants of TMS exist. We believe that highlighting such complex clinical cases will contribute to a better understanding of the mystery of MS.

Keywords: demyelinating disease, multiple sclerosis, tumefactive MS, magnetic resonance imaging,

pdf - Download FULL TEXT /PDF 1147 KB/
Please cite this article as: Yanakieva M, Danovska M, Ovcharova E, Marinova D, Shepherd N. Tumefactive multiple sclerosis: a diagnostic enigma. A case report. J of IMAB. 2023 Apr-Jun;29(2):4943-4946. DOI: 10.5272/jimab.2023292.4943

Corresponding AutorCorrespondence to: Magdalina Yanakieva, Department of Neurology, Faculty of Medicine, Medical University – Pleven; 1, Kliment Ohridski Str., 5800 Pleven, Bulgaria; E-mail: m.yanakieva94@gmail.com

1. Štourač P, Kolčava J, Keřkovský M, Kopřivová T, Křen L, Bednařík J. Progressive Tumefactive Demyelination as the Only Result of Extensive Diagnostic Work-Up: A Case Report. Front Neurol. 2021 Jul 8;12:701663. [PubMed]
2. Kaeser MA, Scali F, Lanzisera FP, Bub GA, Kettner NW. Tumefactive multiple sclerosis: an uncommon diagnostic challenge. J Chiropr Med. 2011 Mar;10(1):29-35. [PubMed]
3. Ayrignac X, Carra-Dalliиre C, Labauge P. Atypical inflammatory demyelinating lesions and atypical multiple sclerosis. Rev Neurol (Paris). 2018 Jun;174(6):408-418. [PubMed]
4. Mando R, Muallem E, Meka SG, Berghea R. A Blind Spot in the Diagnostic Field: The Challenging Diagnosis of Tumefactive Multiple Sclerosis. Case Rep Neurol Med. 2018 Jun 27;2018:6841291. [PubMed]
5. Lucchinetti CF, Gavrilova RH, Metz I, Parisi JE, Scheithauer BW, Weigand S, et al. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain. 2008 Jul;131(Pt 7):1759-75. [PubMed]
6. Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983 Nov;33(11):1444-52 [PubMed]
7. Dolic K, Bilic I, Buca A, Radovic D, Titlic M. Differentiation of Tumefactive Demyelinating Lesions from Metastatic Brain Disease with FDG PET-CT: A Case Report. J Mult Scler. 2014; 1(1):108. [Crossref]
8. Vakrakou AG, Tzanetakos D, Argyrakos T, Koutsis G, Evangelopoulos ME, Andreadou E, et al. Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature. Front Neurol. 2020 Jun 30;11:536. [PubMed]
9. Wadhwa A, Chatterjee A, Chaudhry N, Laskar S, Chandan S. A Case of Relapsing-Remitting Tumefactive Demyelination. Ann Indian Acad Neurol. 2019 Jan-Mar;22(1):124-126. [PubMed]
10. Hardy TA, Chataway J. Tumefactive demyelination: an approach to diagnosis and management. J Neurol Neurosurg Psychiatry. 2013 Sep;84(9):1047-53. [PubMed]
11. Zaheer K, Ajmeri AN, Singh M, Suliman MS, Teka S. Tumefactive Multiple Sclerosis, A Rare Variant Presenting as Multiple Ring-enhancing Lesions in an Immunocompetent Patient: A Case Report. Cureus. 2018 Dec 17;10(12):e3738. [PubMed]
12. Brod SA, Lindsey JW, Nelson F. Tumefactive demyelination: Clinical outcomes, lesion evolution and treatments. Mult Scler J Exp Transl Clin. 2019 Jun 18;5(2):2055217319855755. [PubMed]

Received: 21 December 2022
Published online: 05 June 2023

back to Online Journal