Malignant multiple sclerosis

Last updated

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]

Contents

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]

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

Related Research Articles

<span class="mw-page-title-main">Acute disseminated encephalomyelitis</span> Autoimmune disease

Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. The cause is often a trigger such as from viral infection or vaccinations.

<span class="mw-page-title-main">Optic neuritis</span> Inflammation of the optic nerve

Optic neuritis describes any condition that causes inflammation of the optic nerve; it may be associated with demyelinating diseases, or infectious or inflammatory processes.

<span class="mw-page-title-main">Multiple sclerosis</span> Disease that damages the myelin sheaths around nerves

Multiple sclerosis (MS) is an autoimmune disease in which the insulating covers of nerve cells in the brain and spinal cord are damaged. Being a demyelinating disease, MS disrupts the ability of parts of the nervous system to transmit signals, resulting in a range of signs and symptoms, including physical, mental, and sometimes psychiatric problems. Symptoms include double vision, vision loss, eye pain, muscle weakness, and loss of sensation or coordination. MS takes several forms, with new symptoms either occurring in isolated attacks or building up over time. In relapsing forms of MS, between attacks, symptoms may disappear completely, although some permanent neurological problems often remain, especially as the disease advances. In progressive forms of MS, bodily function slowly deteriorates once symptoms manifest and will steadily worsen if left untreated.

<span class="mw-page-title-main">Interferon beta-1a</span> Cytokine in the interferon family

Interferon beta-1a is a cytokine in the interferon family used to treat multiple sclerosis (MS). It is produced by mammalian cells, while interferon beta-1b is produced in modified E. coli. Some research indicates that interferon injections may result in an 18–38% reduction in the rate of MS relapses.

<span class="mw-page-title-main">Hematopoietic stem cell transplantation</span> Medical procedure to replace blood or immune stem cells

Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood, in order to replicate inside a patient and produce additional normal blood cells. HSCT may be autologous, syngeneic, or allogeneic.

Neuromyelitis optica spectrum disorders (NMOSD) are a spectrum of autoimmune diseases characterized by acute inflammation of the optic nerve and the spinal cord (myelitis). Episodes of ON and myelitis can be simultaneous or successive. A relapsing disease course is common, especially in untreated patients.

Interferon beta-1b is a cytokine in the interferon family used to treat the relapsing-remitting and secondary-progressive forms of multiple sclerosis (MS). It is approved for use after the first MS event. Closely related is interferon beta 1a, also indicated for MS, with a very similar drug profile.

Experimental autoimmune encephalomyelitis, sometimes experimental allergic encephalomyelitis (EAE), is an animal model of brain inflammation. It is an inflammatory demyelinating disease of the central nervous system (CNS). It is mostly used with rodents and is widely studied as an animal model of the human CNS demyelinating diseases, including multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). EAE is also the prototype for T-cell-mediated autoimmune disease in general.

Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease that affects the central nervous system (CNS). Several therapies for it exist, although there is no known cure.

<span class="mw-page-title-main">Glatiramer acetate</span> Medication

Glatiramer acetate, sold under the brand name Copaxone among others, is an immunomodulator medication used to treat multiple sclerosis. Glatiramer acetate is approved in the United States to reduce the frequency of relapses, but not for reducing the progression of disability. Observational studies, but not randomized controlled trials, suggest that it may reduce progression of disability. While a conclusive diagnosis of multiple sclerosis requires a history of two or more episodes of symptoms and signs, glatiramer acetate is approved to treat a first episode anticipating a diagnosis. It is also used to treat relapsing-remitting multiple sclerosis. It is administered by subcutaneous injection.

Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process. IDDs share characteristics with and are often grouped together under Multiple Sclerosis. They are sometimes considered different diseases from Multiple Sclerosis, but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course.

<span class="mw-page-title-main">Marburg acute multiple sclerosis</span> Medical condition

Marburg acute multiple sclerosis, also known as Marburg multiple sclerosis or acute fulminant multiple sclerosis, is considered one of the multiple sclerosis borderline diseases, which is a collection of diseases classified by some as MS variants and by others as different diseases. Other diseases in this group are neuromyelitis optica (NMO), Balo concentric sclerosis, and Schilder's disease. The graver course is one form of malignant multiple sclerosis, with patients reaching a significant level of disability in less than five years from their first symptoms, often in a matter of months.

<span class="mw-page-title-main">Laquinimod</span> Chemical compound

Laquinimod is an experimental immunomodulator developed by Active Biotech and Teva. It is being investigated as an oral treatment for multiple sclerosis (MS) and Huntington's disease.

Research in multiple sclerosis may find new pathways to interact with the disease, improve function, curtail attacks, or limit the progression of the underlying disease. Many treatments already in clinical trials involve drugs that are used in other diseases or medications that have not been designed specifically for multiple sclerosis. There are also trials involving the combination of drugs that are already in use for multiple sclerosis. Finally, there are also many basic investigations that try to understand the disease better and in the future may help to find new treatments.

A clinically isolated syndrome (CIS) is a clinical situation of an individual's first neurological episode, caused by inflammation or demyelination of nerve tissue. An episode may be monofocal, in which symptoms present at a single site in the central nervous system, or multifocal, in which multiple sites exhibit symptoms. CIS with enough paraclinical evidence can be considered as a clinical stage of multiple sclerosis (MS). It can also be retrospectively diagnosed as a kind of MS when more evidence is available.

<span class="mw-page-title-main">Diagnosis of multiple sclerosis</span>

Current standards for diagnosing multiple sclerosis (MS) are based on the 2018 revision of McDonald criteria. They rely on MRI detection of demyelinating lesions in the CNS, which are distributed in space (DIS) and in time (DIT). It is also a requirement that any possible known disease that produces demyelinating lesions is ruled out before applying McDonald's criteria.

<span class="mw-page-title-main">Pathology of multiple sclerosis</span> Pathologic overview

Multiple sclerosis (MS) can be pathologically defined as the presence of distributed glial scars (scleroses) in the central nervous system that must show dissemination in time (DIT) and in space (DIS) to be considered MS lesions.

MOG antibody disease (MOGAD) or MOG antibody-associated encephalomyelitis (MOG-EM) is an inflammatory demyelinating disease of the central nervous system. Serum anti-myelin oligodendrocyte glycoprotein antibodies are present in up to half of patients with an acquired demyelinating syndrome and have been described in association with a range of phenotypic presentations, including acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, and neuromyelitis optica.

<span class="mw-page-title-main">Shimon Slavin</span> Israeli professor of medicine

Shimon Slavin is an Israeli professor of medicine. He pioneered immunotherapy mediated by allogeneic donor lymphocytes and innovative methods for stem cell transplantation to cure hematological malignancies and solid tumors. He also used hematopoietic stem cells to induce transplantation tolerance to bone marrow and organ allografts.

Anti-AQP4 diseases, are a group of diseases characterized by auto-antibodies against aquaporin 4.

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, 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. 1 2 "SSA - POMS: DI 23022.620 - Malignant Multiple Sclerosis - 09/16/2020". secure.ssa.gov. Retrieved 2021-10-26.
  9. 1 2 "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. 1 2 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, 14(2), 278–283. doi : 10.1177/1352458507082604
This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.