Marburg acute multiple sclerosis, also known as Marburg multiple sclerosis or acute fulminant

multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...

, 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 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 ...

(NMO),

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 periaxial ...

, and Schilder's disease. The graver course is one form of

malignant multiple sclerosis 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., ...

, with patients reaching a significant level of disability in less than five years from their first symptoms, often in a matter of months. Sometimes Marburg MS is considered a synonym for

tumefactive MS Tumefactive multiple sclerosis is a condition in which the central nervous system of a person has multiple demyelinating lesions with atypical characteristics for those of standard multiple sclerosis (MS). It is called tumefactive as the lesions ...

, but not for all authors.

Pathogenesis

Marburg MS has been reported to be closer to anti-MOG associated ADEM than to standard MS It has been reported to appear sometimes post-partum

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.

Diagnosis

It took its name from

Otto Marburg Otto Marburg (May 25, 1874 – June 13, 1948) was an Austrian neurologist known for his contributions to the understanding of multiple sclerosis and for advances in neurooncology. Marburg was born in Römerstadt in Moravia, Austria-Hungary ...

. It can be diagnosed ''in vivo'' with an MRI scan. If Marburg disease occurs in the form of a single large lesion, it can be radiologically indistinguishable from a brain tumor or abscess. It is usually lethal, but it has been found to be responsive to

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 ...

and Alemtuzumab, and it has also been responsive to

autologous stem cell transplantation Autologous stem-cell transplantation (also called autogenous, autogeneic, or autogenic stem-cell transplantation and abbreviated auto-SCT) is autologous transplantation of stem cells—that is, transplantation in which stem cells ( undifferentiat ...

. Recent evidence shows that Marburg's presents a heterogeneous response to medication, as does standard MS.

Treatment

Historically, acute MS was a fatal disease, with death occurring within a year of onset, often secondary to extensive brainstem demyelination. Treatments include plasma exchange and/or high-dose glucocorticoids(e.g., 1 g/day of methylprednisolone for 3-5 days). Patients that satisfy criteria for MS will be treated with immunomodulatory therapies, often favoring high efficacy monoclonal antibodies.

Prognosis

Marburg variant of MS is an acute fulminant demyelinating process which in most cases progresses inexorably to death within 1–2 years. However, there are some reports of Marburg MS reaching stability by three years.

References

External links

{{Diseases of the nervous system Multiple sclerosis Autoimmune diseases