Malignant multiple sclerosis is used to describe MS patients who reach significant level of disability in a short period of time. Malignant MS cases are not common, less than 5% of patients with MS experience this type of progression.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. 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. Reaching Expanded Disability Status Scale of 6.0 or higher, which is equivalent of needing unilateral support to ambulate (or worse) is generally considered this significant disability level. Patients with severe forms of more common

relapsing remitting In internal medicine, relapse or recidivism is a recurrence of a past (typically medical) condition. For example, multiple sclerosis and malaria often exhibit peaks of activity and sometimes very long periods of dormancy, followed by relapse or r ...

or progressive MS subtypes, as well as rare

Marburg variant 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

Balo concentric sclerosis Baló's concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact. It was described by József Mátyás Baló who initially named it "leuko-encephalitis periaxia ...

, could be considered to have malignant MS. Patients should be carefully worked up to rule out

Neuromyelitis optica Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). Episodes of ON and myelitis ...

(Devic's disease) due to the distinctive pathophysiology and management strategies of this disease.

Signs and symptoms

Some common physical symptoms may include: "weakness in extremities, difficulties with coordination and balance, spasticity,

paresthesia Paresthesia is an abnormal sensation of the skin (tingling, pricking, chilling, burning, numbness) with no apparent physical cause. Paresthesia may be transient or chronic, and may have any of dozens of possible underlying causes. Paresthesias ar ...

, speech impediments, tremors, dizziness, hearing loss, vision impairments, bowel and bladder difficulties"

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 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 ...

. 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 Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates. CSF is produced by specialised ependymal cells in the choroid plexus of the ventricles of the bra ...

. 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 Myelin is a lipid-rich material that surrounds nerve cell axons (the nervous system's "wires") to insulate them and increase the rate at which electrical impulses (called action potentials) are passed along the axon. The myelinated axon can be l ...

loss, white matter abnormalities, and other physical changes in the brain."

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

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.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

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 Plasmapheresis (from the Greek πλάσμα, ''plasma'', something molded, and ἀφαίρεσις ''aphairesis'', taking away) is the removal, treatment, and return or exchange of blood plasma or components thereof from and to the blood circulati ...

immunosuppression Immunosuppression is a reduction of the activation or efficacy of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse react ...

with

mitoxantrone Mitoxantrone (INN, BAN, USAN; also known as Mitozantrone in Australia; trade name Novantrone) is an anthracenedione antineoplastic agent. Uses Mitoxantrone is used to treat certain types of cancer, mostly acute myeloid leukemia. It improves the ...

cyclophosphamide Cyclophosphamide (CP), also known as cytophosphane among other names, is a medication used as chemotherapy and to immunosuppressant, suppress the immune system. As chemotherapy it is used to treat lymphoma, multiple myeloma, leukemia, ovarian can ...

cladribine Cladribine, sold under the brand name Leustatin, among others, is a medication used to treat hairy cell leukemia (leukemic reticuloendotheliosis) and B-cell chronic lymphocytic leukemia. Cladribine, sold under the brand name Mavenclad, is indica ...

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 Methylprednisolone (Depo-Medrol, Medrol, Solu-Medrol) is a synthetic glucocorticoid, primarily prescribed for its anti-inflammatory and immunosuppressive effects. It is either used at low doses for chronic illnesses or used concomitantly at high ...

(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.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 This therapy may be a therapy option for patients with malignant MS.

References