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]
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]
DiagnosisEdit
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]
TreatmentEdit
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]
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]
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]
^Feinstein, Anthony (2007). The clinical neuropsychiatry of multiple sclerosis (2nd ed.). Cambridge: Cambridge University Press. p. 20. ISBN052185234X.
^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
^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.
^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.
^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.
^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
^ abKimiskidis, 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
October 15, 2023
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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 Contents 1 Signs and symptoms 2 Diagnosis 3 Treatment 4 MOG antibody associated demyelinating pseudotumor 5 Autologous stem cell transplantation 6 See also 7 ReferencesSigns and symptoms EditSome 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 EditEarlier 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 EditCurrently 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 EditSome 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 encephalomyelitisAutologous stem cell transplantation EditAnecdotal 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 EditTumefactive multiple sclerosis Marburg acute multiple sclerosis Multiple sclerosis Autologous stem cell transplantationReferences Edit Feinstein Anthony 2007 The clinical neuropsychiatry of multiple sclerosis 2nd ed Cambridge Cambridge University Press p 20 ISBN 052185234X Kimiskidis V Sakellari I Tsimourtou V Kapina V Papagiannopoulos S Kazis D Vlaikidis N Anagnostopoulos A amp Fassas A amp 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 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 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 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 SSA POMS DI 23022 620 Malignant Multiple Sclerosis 09 16 2020 secure ssa gov Retrieved 2021 10 26 a b SSA POMS DI 23022 620 Malignant Multiple Sclerosis 09 16 2020 secure ssa gov Retrieved 2021 10 26 a b SSA POMS DI 23022 620 Malignant Multiple Sclerosis 09 16 2020 secure ssa gov Retrieved 2021 10 26 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 a b Kimiskidis V Sakellari I Tsimourtou V Kapina V Papagiannopoulos S Kazis D Vlaikidis N Anagnostopoulos A amp 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 Retrieved from https en wikipedia org w index php title Malignant multiple sclerosis amp oldid 1170569129, wikipedia, wiki, book, books, library,
