Global InformationTumefactive multiple sclerosis information
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| An example of a ring-enhancement around a lesion in gliobastoma. In tumefactive multiple sclerosis, the ring-enhancement is open, not forming a complete ring. |
Tumefactive multiple sclerosis is a condition in which the central nervous system of a person has multiple demyelinating lesions with atypical characteristics for those of standard multiple sclerosis (MS). It is called tumefactive as the lesions are "tumor-like" and they mimic tumors clinically, radiologically and sometimes pathologically.[1]
These atypical lesion characteristics include a large intracranial lesion of size greater than 2.0 cm with a mass effect, edema and an open ring enhancement. A mass effect is the effect of a mass on its surroundings, for example, exerting pressure on the surrounding brain matter. Edema is the build-up of fluid within the brain tissue. Usually, the ring enhancement is directed toward the cortical surface.[2] The tumefactive lesion may mimic a malignant glioma or cerebral abscess causing complications during the diagnosis of tumefactive MS. T2-hypointense rim and incomplete ring enhancement of the lesions on post-gadolinium T1- weighted imaging on brain MRI enable accurate diagnosis of TDL[3]
Normally a tumefactive demyelinating lesion appears together with smaller disseminated lesions separated in time and space, yielding a diagnosis of Multiple Sclerosis. Hence the name "tumefactive multiple sclerosis". When the demyelinating lesion appears alone it has been termed solitary sclerosis.[4][5][6] These cases belong to a multiple sclerosis borderline and there is currently no universal agreement on how they should be considered.
Tumefactive multiple sclerosis is a demyelinating and inflammatory disease. Myelination of the axons are highly important for signalling as this improves the speed of conduction of action potentials from one axon to the next. This is done through the formation of high-resistance, low-conductance myelin sheaths around the axons by specific cells called oligodendrocytes. As such, the demyelination process affects the communication between neurons and this consequently affects the neural pathways they control. Depending on where the demyelination takes place and its severity, patients with tumefactive MS have different clinical symptoms.[7]
- ^ Xia L., Lin S., Wang Z., Li S., Xu L., Wu J., Hao S., Gao C. (2009). "Tumefactive demyelinating lesions: nine cases and a review of the literature". Neurosurg Rev. 32 (2): 171–179. doi:10.1007/s10143-009-0185-5. PMID 19172322. S2CID 1063158.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Kaeser, M. A., Scali, F., Lanzisera, F. P., Bub, G. A., and Kettner, N. W. Tumefactive multiple sclerosis: an uncommon diagnostic challenge. Journal of Chiropractic Medicine 10:29-35 (2011).
- ^ Kilic AK, Kurne AT, Oguz KK, Soylemezoglu F, Karabudak R (2013). "Mass lesions in the brain: tumor or multiple sclerosis? Clinical and imaging characteristics and course from a single reference center" (PDF). Turk Neurosurg. 23 (6): 728–35. doi:10.5137/1019-5149.JTN.7690-12.3 (inactive 31 January 2024). PMID 24310455. Retrieved 19 March 2020.
{{cite journal}}: CS1 maint: DOI inactive as of January 2024 (link) CS1 maint: multiple names: authors list (link) - ^ Schmalstieg WF, Keegan BM, Weinshenker BG (February 2012). "Solitary sclerosis: progressive myelopathy from solitary demyelinating lesion". Neurology. 78 (8): 540–4. doi:10.1212/WNL.0b013e318247cc8c. PMID 22323754. S2CID 52859541.
- ^ Jiménez Arango JA, Uribe Uribe CS, Toro González G (2013). "Lesser-known myelin-related disorders: Focal tumour-like demyelinating lesions". Neurologia. 30 (2): 97–105. doi:10.1016/j.nrl.2013.06.004. PMID 24094691.
- ^ Kalavakunta, Jagadeesh K.; Tokala, Hemasri; Loehrke, Mark (2011-08-01). "Solitary lesion in magnetic resonance imaging: tumor versus multiple sclerosis". The American Journal of the Medical Sciences. 342 (2): 168. doi:10.1097/MAJ.0b013e318200d247. ISSN 1538-2990. PMID 21799469.
- ^ Moore G. R. W., Esiri M. M. (2011). "The pathology of multiple sclerosis and related disorders". Diagnostic Histopathology. 17 (5): 225–231. doi:10.1016/j.mpdhp.2011.02.001.