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]

Signs and symptoms

Some common physical symptoms may include: "weakness in extremities, difficulties with coordination and balance, spasticity, paresthesia, speech impediments, tremors, dizziness, hearing loss, vision impairments, bowel and bladder difficulties"[7]

Diagnosis

Earlier signs include an increase in mobility over a short period of time. Malignant MS happens in patients that already have or have been diagnosed with MS. There is not a specific test to detect malignant MS; it is often confused with acute disseminated encephalomyelitis.

Malignant MS is diagnosed after clinical investigations. Doctors may access the symptoms and to rule out other disorders, a neurological exam is performed. Further diagnostics may include an analysis of the cerebrospinal fluid.[8]

Neurological testing may also be performed, such as "a magnetic resonance imaging (MRI), diffusion-tensor magnetic resonance imaging (DT-MRI), and computerized brain tomography are used to detect central nervous system lesions, myelin loss, white matter abnormalities, and other physical changes in the brain."[8]

Treatment

Currently, there is no cure for malignant MS; however, "immunomodulatory therapy and other physical occupational therapies can help the management of symptoms and help them more easily perform everyday tasks such as handwriting, buttoning, and using eating utensils."[9]

Some mobility aids, such as canes, walkers, and wheelchairs may also be helpful as well for patients struggling with balance and walking.[9]

MOG antibody‐associated demyelinating pseudotumor

Some anti-MOG cases satisfy the MS requirements (lesions disseminated in time and space) and are therefore traditionally considered MS cases. After the discovery of the anti-MOG disease this classification is into revision.[10]

Main article: anti-MOG associated encephalomyelitis

Autologous stem cell transplantation

Anecdotal evidence shows that autologous stem-cell transplantation, intensive immunosuppression combined with autologous stem cell therapy, may be an effective means for treating this life-threatening condition. The process of this therapy entails plasmapheresis or immunosuppression with mitoxantrone, cyclophosphamide, cladribine or bone marrow transplantation. In one study on a 17-year-old patient, researchers tried treating the patient with high-dose chemotherapy plus anti-thymocyte globulin followed by autologous stem cell transplantation. The findings of this study were positive. After being treated with a methylprednisolone (mPDN) i.v, the patient improved significantly. When the patient began to relapse/remit, they were treated again, resulting in improvement. After this, the patient remained stable for roughly 7 months before suffering their third relapse/remit.[11]

This therapy may be a therapy option for patients with malignant MS.[11]

See also

References
  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 (Houndmills, Basingstoke, England), 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.
  7. "SSA - POMS: DI 23022.620 - Malignant Multiple Sclerosis - 09/16/2020". secure.ssa.gov. Retrieved 2021-10-26.
  8. "SSA - POMS: DI 23022.620 - Malignant Multiple Sclerosis - 09/16/2020". secure.ssa.gov. Retrieved 2021-10-26.
  9. "SSA - POMS: DI 23022.620 - Malignant Multiple Sclerosis - 09/16/2020". secure.ssa.gov. Retrieved 2021-10-26.
  10. Yaqing Shu Youming Long Shisi Wang Wanming Hu Jian Zhou Huiming Xu Chen Chen Yangmei Ou Zhengqi Lu Alexander Y. Lau Xinhua Yu Allan G. Kermode Wei Qiu, Brain histopathological study and prognosis in MOG antibody‐associated demyelinating pseudotumor, 08 January 2019, https://doi.org/10.1002/acn3.712
  11. Kimiskidis, V., Sakellari, I., Tsimourtou, V., Kapina, V., Papagiannopoulos, S., Kazis, D., Vlaikidis, N., Anagnostopoulos, A., & Fassas, A. (2008). Autologous stem-cell transplantation in malignant multiple sclerosis: a case with a favorable long-term outcome. Multiple Sclerosis (Houndmills, Basingstoke, England), 14(2), 278–283. doi:10.1177/1352458507082604
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