General Outline
editDifferences from Multiple Sclerosis
editA very small number of multiple sclerosis cases are considered tumefactive multiple sclerosis (1 in every 1000 multiple sclerosis cases are tumefactive). Multiple Sclerosis is a demyelinating disease, specifically an auto-immune disease where the specific (adaptive) immune system attacks and destroys myelinated axons in the CNS. Tumefactive Multiple Sclerosis is a rare subset of Multiple Sclerosis where demyelination occurs in a specific area of the brain, resulting in an area larger than 2 cm where axons lack myelin.
Clinical Signs and Symptoms
editSymptoms are different for each patient, depending on the location of the demyelination.
Common symptoms include:
- Problems processing information
- Cognitive dysfunction
- Problems with speech and vision
- Spasticity - result of demyelination to the efferent motor pathways.
- Loss of motor control
- Loss of sensation
- Often comorbid with depression
Causes/Pathology
editTumefactive multiple sclerosis is an autoimmune disease that targets oligodendrocytes in the central nervous system, which dramatically affects communication abilities between neuronal connections. Inflammation also occurs, due to a breach in the blood brain barrier, which leads to further damage as a result of inflammation occurring in a closed system.
Epidemiology
editDiagnosis
editMagnetic Resonance Imaging (MRI) - presence of lesions in white matter. Proton MR spectroscopy (H-MRS) detects biochemical changes in the brain, improving pathologic specificity[1] A large brain lesion must be over 2.0 cm in order to be diagnosed as TMS.
Prognosis
editLife expectancy is only 10 years less than that of the average unaffected person. However, quality of life is greatly affected. Couldn't disagree more! I had a tumefactive MS lesion five years ago. It has not turned into full blown MS, I am not on any medication, it has not affected my quality of life and I'm in better shape than before the initial attack. What a joke! My neurologists that I meet with on a yearly basis after my annual MRI feels the chance of recurrance is low and the longer I stay in the remission, the smaller the chance of recurrance.
Management
editReferences
edit- ^ Kaeser, Martha A., Frank Scali, Frank P. Lanzisera, and Norman W. Kettner. "Tumefactive Multiple Sclerosis: An Uncommon Diagnostic Challenge." US National Library of Medicine National Institutes of Health. N.p., Mar. 2011. Web. 8 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3110404/>.
- Kaeser, Martha A., Frank Scali, Frank P. Lanzisera, and Norman W. Kettner. "Tumefactive Multiple Sclerosis: An Uncommon Diagnostic Challenge." US National Library of Medicine National Institutes of Health. N.p., Mar. 2011. Web. 8 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3110404/>.
- Lucchinetti, C. F., R. H. Gavrilova, I. Metz, J. E. Parisi, B. W. Scheithauer, S. Weigand, K. Thomsen, J. Mandrekar, A. Altintas, B. J. Erickson, F. König, C. Giannini, H. Lassmann, L. Linbo, S. J. Pittock, and W. Brück. "Clinical and Radiographic Spectrum of Pathologically Confirmed Tumefactive Multiple Sclerosis." National Center for Biotechnology Information. U.S. National Library of Medicine, 04 June 2008. Web. 13 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2442427/>.
- TA, Hardy, and Chataway J. "Tumefactive Demyelination: An Approach to Diagnosis and Management." National Center for Biotechnology Information. U.S. National Library of Medicine, 19 Jan. 2013. Web. 13 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pubmed/23334629>.
- AP, Dagher, and Smirniotopoulos J. "Tumefactive Demyelinating Lesions." National Center for Biotechnology Information. U.S. National Library of Medicine, Aug. 1996. Web. 13 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pubmed/8880719>.