Marburg variant of acute multiple sclerosis

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The Marburg variant , named after its first person who described it, Otto Marburg , is a special form of multiple sclerosis , an inflammatory demyelinating disease of the central nervous system . The Marburg variant is characterized by a particularly aggressive, malicious (malignant) course, which usually leads to death within a few months for those affected.

Symptoms

The first symptoms of the disease can be similar to those of an acute episode of multiple sclerosis. These include sensory disorders of the skin, symptoms of paralysis, coordination disorders, visual disorders, speech and language disorders or unsteady gait. Typical of the Marburg variant is the very rapid progression of the symptoms, which can lead to severe disability within a few days and make admission to a hospital or intensive care unit necessary.

causes

The causes and development of the disease have not yet been adequately clarified. Similar to multiple sclerosis, the immune reaction against the medullary sheaths of central neurons plays a central role.

diagnosis

The differentiation of the Marburg variant from other demyelinating diseases (acute demyelinating encephalomyelitis, progressive multiple sclerosis, diffuse disseminated sclerosis, neuromyelitis optica) is clinically very difficult and in many cases is only possible after death by means of histopathological examination of the inflamed brain regions.

treatment

The treatment is analogous to that of an acute flare-up of multiple sclerosis. Here come glucocorticoids as pulse therapy and plasmapheresis used. Individual healing attempts include the use of cyclophosphamide or monoclonal antibodies (e.g. rituximab ).

swell

  • JC Nunes et al .: The most brilliant course of the Marburg variant of multiple sclerosis-autopsy findings. In: Mult Scler. 21 (4), Apr 2015, pp. 485-487.
  • O. Marburg: The so-called acute multiple sclerosis (encephalomyelitis periaxialis scleroticans). In: Jahrb Psychiat Neurol. 27, 1906, pp. 213-312.