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Marburg variant of multiple sclerosis

Dr Rohit Sharma and Dr Nasir Siddiqui et al.

The Marburg variant of multiple sclerosis, also known as acute, fulminant, or malignant multiple sclerosis, is characterized by extensive and fulminant acute demyelination, often resulting in death within one year after the onset of clinical signs.

Please, refer on multiple sclerosis (MS) for a broad discussion on this entity.

On this page:

Article:

Epidemiology
Clinical presentation
Pathology
Radiographic features
Treatment and prognosis
History and etymology
Differential diagnosis
Related articles
References

Images:

Cases and figures

Epidemiology

Marburg variant is typically seen in younger patients ^1,4.

Clinical presentation

Symptoms may be preceded by fevers and are typically monophasic.

Pathology

Areas of demyelination demonstrate marked macrophage infiltration, marked associated inflammation, and extensive tissue destruction, necrosis and axonal damage and necrosis ^2,4.

Unlike other multiple sclerosis variants, the peripheral nervous system may also be involved ².

Radiographic features

As is the case with other demyelinating diseases, MRI is the modality of choice for assessing patients with suspected Marburg type multiple sclerosis.

MRI

Extensive confluent areas tumefactive demyelination are seen with mass effect and defined rings and incomplete ring enhancement ^1,4.

Only rarely are numerous smaller lesions seen disseminated throughout the brain ².

Treatment and prognosis

Marburg type MS typically is rapidly progressive, fulminant and often results in death from involvement of the brain stem or due to marked mass effect and herniation ^2,4.

Only rarely do affected areas remyelinate ².

History and etymology

It was first described in 1906 by the Austrian neurologist Otto Marburg (1874-1948)³.

Differential diagnosis

Possible imaging differential considerations include:

Balo concentric sclerosis (BCS)
acute disseminated encephalomyelitis (ADEM)
- similar, although Marburg is usually more severe

Quiz questions

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References

White matter disorders

white matter
normal myelination
terminology
white matter disorders
- demyelination
  - anti-MOG associated encephalomyelitis
  - Guillain-Barre Syndrome (GBS)
    - acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
    - axonal subtypes
      - acute motor axonal neuropathy (AMAN) (Chinese paralytic syndrome)
      - acute motor-sensory axonal neuropathy (AMSAN)
    - regional GBS syndromes
      - Miller Fisher variant
  - chronic inflammatory demyelinating polyneuropathy (CIDP)
  - transverse myelitis
  - tumefactive demyelinating lesions
  - acute disseminated encephalomyelitis (ADEM)
    - Callen MS-ADEM criteria
  - acute hemorrhagic encephalomyelitis (AHEM)
  - neuromyelitis optica (NMO) (Devic disease)
    - signs
      - marbled pattern of the corpus callosum
      - longitudinally extensive spinal cord lesion (LESCL)
  - multiple sclerosis (MS)
    - McDonald diagnostic criteria for MS (current 2017 revision)
      - previous 2016 MAGNIMS consensus
    - signs
    - variants
      - tumefactive multiple sclerosis
      - acute malignant Marburg type
      - Schilder type (diffuse cerebral sclerosis)
      - Balo concentric sclerosis (BCS)
      - opticospinal multiple sclerosis (OSMS)
  - radiologically isolated syndrome (RIS)
  - clinically isolated syndrome (CIS)
- astrocytopathies
- leuko-axonopathies
  - early-onset neuronal degenerative disorders
    - GM1 & GM2 gangliosidoses (e.g. Tay Sachs disease)
  - giant axonal neuropathy
  - hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC)
  - hypomyelination with congenital cataract
  - leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation
  - hypomyelination with brainstem and spinal cord involvement and leg spasticity
  - Pol III-related leukodystrophies
- leuko-vasculopathies
- microgliopathies
  - CSF1R-related disorders
    - adult-onset leukoencephalopathy with axonal spheroids and pigmented glia
    - pigmentary ortochromatic leukodystrophy
  - Nasu Hakola disease
- myelin disorders
  - hypomyelination
  - demyelination
    - metachromatic leukodystrophy
  - myelin vacuolisation
    - Canavan disease
    - globoid cell leukodystrophy (Krabbe disease)
    - mitochondrial disorders
    - multiple sulfatase deficiency
    - phenylketonuria
    - x-linked adrenoleukodystrophy
- other
  - adult-onset autosomal dominant leukodystrophy
  - cerebrotendinous xanthomathosis
  - cystic leukoencephalopathy without megalencephaly
  - L-2-hydroxyglutaric aciduria
  - lysosomal storage diseases
    - free sialic acid storage disorders (e.g. Salla disease)
    - Niemann-Pick disease
  - peroxisomal disorders
    - Zellweger syndrome
  - Sjögren-Larsson syndrome

Article information

rID: 12276

System: Central Nervous System

Tag: cases

Synonyms or Alternate Spellings:

Marburg multiple sclerosis
Multiple sclerosis Marburg variant
Acute malignant marburg type (MS variant)
Acute malignant marburg type (multiple sclerosis variant)
Marburg variant
Marburg's variant multiple sclerosis

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