Abstract
Study design:
Case report.
Objectives:
We report on a 52-year-old male patient with tumefactive demyelination of the spinal cord.
Setting:
University Hospital and Jessenius Faculty of Medicine, Comenius University, Martin, Slovakia.
Background:
In contrast to relatively frequent tumefactive fulminant lesions in the brain, cases affecting the spinal cord in isolation have been reported less frequently.
Methods:
Description of the case report.
Results:
Clinical, neuroradiological and necropsy findings are described in a 52-year-old man with tumefactive fulminant demyelination of the spinal cord. Progression of the demyelination process produced paraplegia, mild paresis of the right upper limb, neurogenic bladder and sensitive loss over 2 weeks. MRI scans revealed several ovoid lesions in cervical segments and tumefactive T2-hyperintense signals with oedema and post-contrast enhancement located in thoracic segments Th3 to Th6. Cerebrospinal fluid (CSF) examination displayed lymphomonocytic pleocytosis with normal proteinorhachia, positive CSF oligoclonal IgG bands (OCB) and elevated IgG index (1.55). Serum anti-AQP4-Ab was not tested. Stored frozen CSF samples were later repeatedly examined with negative findings of anti-AQP4-Ab. Treatment with high-dose methylprednisolon and plasma exchange had limited effect. Immunosuppressive medication was interrupted because of an acute urinary infection. The patient died suddenly because of pulmonary embolism as a secondary complication. Histopathology of the spinal cord confirmed active demyelination. We considered that tumefactive demyelination could be a variant of neuromyelitis optica.
Conclusion:
Our case could be anti-AQP4-Ab-negative longitudinally extensive transverse myelitis, a variant of neuromyelitis optica.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
References
Makary MS, Kirch CF . Tumefactive demyelinating disease with isolated spinal cord involvement. Acta Radiologica 2014; 3: 1–3.
Yaghi S, Gokdin M, Sethi H . Tumefactive demyelination of the spinal cord. Acta Neurol Belg 2010; 110: 206–208.
Maezawa H, Takano M, Nagai S, Iida H, Tachibana S . Spinal multiple sclerosis mimicking a spinal cord tumor: a case report. No Shinkei Geka 1995; 23: 1007–1010.
Andersson M, Alvarez-Cermeño J, Bernardi G, Cogato I, Fredman P, Frederiksen J et al. Cerebrospinal fluid in the diagnosis of multiple sclerosis: a consensus report. J Neurol Neurosurg Psychiatry 1994; 57: 897–902.
Rahmlow MR, Kantarci O . Fulminant demyelinating diseases. Neurohospitalist 2013; 3: 81–91.
Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG . Revised diagnostic criteria for neuromyelitis optica. Neurology 2006; 66: 1485–1489.
Trebst C, Jarius S, Berthele A, Friedemann P, Schippling S, Wildemann B et al. Update on the diagnosis and treatment of neuromyelitis optica: Recommendations of the Neuromyelitis Optica Study Group (NEMOS). J Neurol 2014; 61: 1–16.
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; 131: 1759–1775.
Acknowledgements
MUDr Ema Kantorová PhD's work has been supported by Project ESF ITMS kód: 26110230067 and Project ITMS 26110230071 “Virtuálna a simulačná výučba ako nová forma vzdelávania na JLF UK v Martine”. MUDr Štefan Sivák PhD's work has been supported by Project ESF ITMS kód: 26110230067 and Project ITMS 26110230071 “Virtuálna a simulačná výučba ako nová forma vzdelávania na JLF UK v Martine”. MUDr Juraj Marcinek PhD, MUDr Kamil Zeleňák PhD, MUDr Jozef Michalik, Prof MUDr Egon Kurča PhD, Prof MUDr Lukáš Plank CSc and MC Kryštof Kantor declare no potential conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no conflict of interest.
Rights and permissions
About this article
Cite this article
Kantorová, E., Marcinek, J., Zeleňák, K. et al. Tumefactive demyelination of the spinal cord: a case report. Spinal Cord 53, 877–880 (2015). https://doi.org/10.1038/sc.2015.52
Received:
Revised:
Accepted:
Published:
Issue date:
DOI: https://doi.org/10.1038/sc.2015.52
