Malignant multiple sclerosis

Malignant multiple sclerosis is used to describe MS patients who reach significant level of disability in a short period of time.[1] Malignant MS cases are not common, less than 5% of patients with MS experience this type of progression.[2]

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.[3] Reaching Expanded Disability Status Scale of 6.0 or higher, which is equivalent of needing unilateral support to ambulate [4] (or worse) is generally considered this significant disability level.[5]

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 to have 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.[6]

  1. ^ Feinstein, Anthony (2007). The clinical neuropsychiatry of multiple sclerosis (2nd ed.). Cambridge: Cambridge University Press. p. 20. ISBN 052185234X.
  2. ^ Kimiskidis, V., Sakellari, I., Tsimourtou, V., Kapina, V., Papagiannopoulos, S., Kazis, D., Vlaikidis, N., Anagnostopoulos, A., & Fassas, A. & Fassas, A. (2008). Autologous stem-cell transplantation in malignant multiple sclerosis: a case with a favorable long-term outcome. Multiple Sclerosis, 14(2), 278–283. doi:10.1177/1352458507082604
  3. ^ Lublin FD, Reingold SC (1996). "Defining the clinical course of multiple sclerosis: Results of an international survey". Neurology. 46 (4): 907–11. doi:10.1212/WNL.46.4.907. PMID 8780061.
  4. ^ Kurtzke JF (1983). "Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS)". Neurology. 33 (11): 1444–52. doi:10.1212/WNL.33.11.1444. PMID 6685237.
  5. ^ 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.
  6. ^ 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.
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