Malignant multiple sclerosis
- Malignant multiple sclerosis
-
The term malignant multiple sclerosis is often used to describe MS patients who reach significant level of disability in less than 5 years from their first symptoms [1]
The National MS Society Advisory Committee on Clinical Trials of New Agents consensus defined it as: disease with a rapid progressive course, leading to significant disability in multiple neurologic systems or death in a relatively short time after disease onset.[2] Reaching Expanded Disability Status Scale of 6.0 or higher, which is equivalent of needing unilateral support to ambulate [3] (or worse) is generally considered this significant disability level.[4]
Patients with severe forms of more common relapsing remitting or progressive MS subtypes, as well as rare Marburg variant and Balo concentric sclerosis could be considered malignant MS. Patients should be carefully worked up to rule out Neuromyelitis optica (Devic's disease) due to the distinctive pathophysiology and management strategies of this disease.[5]
References
- ^ DeLuca GC, Ramagopalan SV, Herrera BM, et al. (2007). "An extremes of outcome strategy provides evidence that multiple sclerosis severity is determined by alleles at the HLA-DRB1 locus". Proc Natl Acad Sci USA 104 (52): 20896–901. doi:10.1073/pnas.0707731105. PMC 2409238. PMID 18087043. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2409238.
- ^ Lublin FD, Reingold SC (2007). "Defining the clinical course of multiple sclerosis: Results of an international survey". Neurology 46 (4): 907–11. PMID 18087043.
- ^ Kurtzke JF (1983). "Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS)". Neurology 33 (11): 1444–52. PMID 6685237.
- ^ Gholipour T, Healy B, Baruch NF, et al. (2011). "Demographic and clinical characteristics of malignant multiple sclerosis". Neurology 76 (23): 1996–2001. doi:10.1212/WNL.0b013e31821e559d. PMID 21646626.
- ^ Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA (2006). "Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression". Arch. Neurol. 63 (7): 964–968. doi:10.1001/archneur.63.7.964. PMID 16831965. http://archneur.ama-assn.org/cgi/content/full/63/7/964.
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