Marburg acute multiple sclerosis

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Marburg acute multiple sclerosis
Other namesAcute multiple sclerosis, Marburg type

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. [1] 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. [2]

Contents

Sometimes Marburg MS is considered a synonym for tumefactive MS, [3] but not for all authors.[ citation needed ]

Pathogenesis

Marburg MS has been reported to be closer to anti-MOG associated ADEM than to standard MS [4] It has been reported to appear sometimes post-partum [5]

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. [6]

Diagnosis

It took its name from Otto Marburg. It can be diagnosed in vivo with an MRI scan. [7] 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 [8] and Alemtuzumab, [9] and it has also been responsive to autologous stem cell transplantation. [10] Recent evidence shows that Marburg's presents a heterogeneous response to medication, as does standard MS. [11]

Treatment

Historically, acute MS was a fatal disease, with death occurring within a year of onset, often secondary to extensive brain stem demyelination. Treatment recommendations, based on anecdotes, include plasma exchange in conjunction with high-dose glucocorticoids(e.g., 1 to 2 g/day of methylprednisolone for 10 days followed by a slow taper). For patients who survive the acute attack, follow-up treatments include immunosuppression (monthly mitoxantrone or cyclophosphamide) either alone or in combination with IFN beta or GA (glatiramer acetate).[ citation needed ]

Prognosis

Marburg variant of MS is an acute fulminant demyelinating process which in most cases progresses inexorably to death within 1–2 years. [12] However, there are some reports of Marburg MS reaching stability by three years. [13]

See also

Related Research Articles

Acute disseminated encephalomyelitis 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. It is often triggered by a viral infection or vaccinations.

Optic neuritis describes any condition that causes inflammation of the optic nerve; it may be associated with demyelinating diseases, or infectious or inflammatory processes. It is also known as optic papillitis, neuroretinitis and retrobulbar neuritis. It is most often associated with multiple sclerosis, and it may lead to complete or partial loss of vision in one or both eyes. Other causes include:

  1. Hereditary optic neuritis
  2. Parainfectious optic neuritis
  3. Infectious optic neuritis (sinus related or associated with cat scratch fever, tuberculosis, lyme disease and cryptococcal meningitis in AIDS patients
  4. Autoimmune causes
  5. Diabetes Mellitus
  6. Low phosphorus levels
  7. Hyperkalaemia
Multiple sclerosis Disease that damages the myelin sheaths around nerves

Multiple sclerosis (MS), also known as encephalomyelitis disseminata, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This damage 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. Specific symptoms can include double vision, blindness in one eye, muscle weakness, and trouble with sensation or coordination. MS takes several forms, with new symptoms either occurring in isolated attacks or building up over time. In the relapsing forms of MS, between attacks, symptoms may disappear completely, although some permanent neurological problems often remain, especially as the disease advances.

Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are 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. In more than 80% of cases, NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the central nervous system. A subset of anti-AQP4-negative cases is associated with antibodies against myelin oligodendrocyte glycoprotein (anti-MOG). Rarely, NMO may occur in the context of other autoimmune diseases or infectious diseases. In some cases, the etiology remains unknown.

Myelin oligodendrocyte glycoprotein

Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein believed to be important in the myelination of nerves in the central nervous system (CNS). In humans this protein is encoded by the MOG gene. It is speculated to serve as a necessary "adhesion molecule" to provide structural integrity to the myelin sheath and is known to develop late on the oligodendrocyte.

Chronic inflammatory demyelinating polyneuropathy Medical condition

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy. CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. It is one of several types of neuropathy.

Pathophysiology of multiple sclerosis

Multiple sclerosis is an inflammatory demyelinating disease of the CNS in which activated immune cells invade the central nervous system and cause inflammation, neurodegeneration, and tissue damage. The underlying cause is currently unknown. Current research in neuropathology, neuroimmunology, neurobiology, and neuroimaging, together with clinical neurology, provide support for the notion that MS is not a single disease but rather a spectrum.

Lesional demyelinations of the central nervous system

Multiple sclerosis and other demyelinating diseases of the central nervous system (CNS) produce lesions and glial scars or scleroses. They present different shapes and histological findings according to the underlying condition that produces them.

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.

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.

Balo concentric sclerosis Medical condition

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 periaxialis concentrica" from the previous definition, and it is currently considered one of the borderline forms of multiple sclerosis.

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 better the disease and in the future may help to find new treatments.

Tumefactive multiple sclerosis Medical condition

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 are "tumor-like" and they mimic tumors clinically, radiologically and sometimes pathologically.

Diffuse myelinoclastic sclerosis, sometimes referred to as Schilder's disease, is a very infrequent neurodegenerative disease that presents clinically as pseudotumoural demyelinating lesions, making its diagnosis difficult. It usually begins in childhood, affecting children between 5 and 14 years old, but cases in adults are also possible.

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.

Multiple sclerosis diagnosis

Current standard for diagnosing multiple sclerosis (MS) is based in the 2018 revision of McDonald criteria. They rely in 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.

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.

There are several ways for pharmaceuticals for treating multiple sclerosis (MS) to reach the market.

Anti-neurofascin demyelinating diseases refers to health conditions engendered by auto-antibodies against neurofascins, which can produce both central and peripheral demyelination. Some cases of combined central and peripheral demyelination (CCPD) could be produced by them.

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

References

  1. ↑ Fontaine, B. (2001). "Les formes frontières de sclérose en plaques" [Borderline forms of multiple sclerosis]. Revue Neurologique (in French). 157 (8–9 Pt 2): 929–34. PMID   11787357.
  2. ↑ Lublin, F. D.; Reingold, S. C. (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.
  3. ↑ See explanation at
  4. ↑ Todd A Hardy, Reddel, Barnett, Palace, Lucchinetti, Weinshenker, Atypical CNS inflammatory demyelinating disease, The lancet neurology, August, 2016, DOI:https://doi.org/10.1016/S1474-4422(16)30043-6, Manuscript Number: THELANCETNEUROLOGY-D-16-00113R1 available at
  5. ↑ Eduardo Labat et al., An extremely aggressive case of Marburg's disease treated with high dose cyclophosphamide. A case report, Multiple Sclerosis and Related Disorders, Volume 31, June 2019, Pages 51-53, https://doi.org/10.1016/j.msard.2019.03.014
  6. ↑ 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
  7. ↑ Capello E, Mancardi GL (November 2004). "Marburg type and Balò's concentric sclerosis: rare and acute variants of multiple sclerosis". Neurol. Sci. 25 (Suppl): S361–3. doi:10.1007/s10072-004-0341-1. PMID   15727234. S2CID   12897512.
  8. ↑ Jeffery, Douglas R.; Lefkowitz, David S.; Crittenden, Jeffrey P. (2004). "Treatment of Marburg Variant Multiple Sclerosis with Mitoxantrone". Journal of Neuroimaging. 14 (1): 58–62. doi:10.1111/j.1552-6569.2004.tb00217.x. PMID   14748210. S2CID   30709186.
  9. ↑ Gormley KM, Zajicek JP (2006). "Alemtuzumab and craniotomy for severe acute demyelinating illness". 16th Meeting of the European Neurological Society. Archived from the original on 2007-10-07.
  10. ↑ Kimiskidis, VK; 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 Journal. 14 (2): 278–283. doi:10.1177/1352458507082604. PMID   17942513. S2CID   42334384.
  11. ↑ Leussink, V. I.; Lehmann, H. C.; Meyer Zu Hörste, G.; Hartung, H.-P.; Stüve, O.; Kieseier, B. C. (2008). "Rituximab induces clinical stabilization in a patient with fulminant multiple sclerosis not responding to natalizumab". Journal of Neurology. 255 (9): 1436–1438. doi:10.1007/s00415-008-0956-x. PMID   18685916. S2CID   38328163.
  12. ↑ Dan L. Longo; et al., eds. (2012). Harrison's principles of internal medicine (18th ed.). New York: McGraw-Hill. p. 3407. ISBN   9780071748896.
  13. ↑ Turatti, Marco; Gajofatto, Alberto; Rossi, Francesca; Vedovello, Marcella; Benedetti, Maria Donata (2010). "Long survival and clinical stability in Marburg's variant multiple sclerosis". Neurological Sciences. 31 (6): 807–811. doi:10.1007/s10072-010-0287-4. PMID   20461429. S2CID   37354295.
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