Acute disseminated encephalomyelitis


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Classification according to ICD-10
G37.8 Other specified central nervous system demyelinating diseases
- Acute demyelinating encephalomyelitis (ADEM)
ICD-10 online (WHO version 2019)

The Acute disseminated / demyelinating encephalomyelitis ( ADEM or ADE ), also known as peri-venous encephalomyelitis or Hurst encephalitis is called, is a rare acute inflammatory disease of the central nervous system that often occurs one to four weeks after infection. Mostly children are affected. The disease is rare above the age of 40. Is a history of any prior infection known often remains unclear if this is not the beginning of a multiple sclerosis is (MS), consist of the smooth transitions.

Etiology / origin

Acute disseminated encephalomyelitis is usually due to a previous viral infection triggered autoimmune disease . The previous infection cannot always be determined from anamnestic. Very rarely, ADEM can also develop paravaccinously (in connection with a vaccination ). The activated T-lymphocytes are directed against proteins of the white matter of the brain.

Symptoms

The clinical symptoms vary greatly depending on the location of the lesions , but usually also include neuropsychological symptoms such as psychomotor slowdown or impaired consciousness . Headache , neck stiffness , vomiting , severe depression, epileptic seizures , a paraplegic syndrome , incomplete or one -sided paralysis , speech disorders , atactic gait disorders and, above all, bilateral inflammation of the optic nerve with visual disturbances can also occur. Inflammation of the optic nerve is the characteristic symptom.

Course and prognosis

The definitive differentiation from multiple sclerosis is only possible in the clinical course. In the context of ADEM, there is no relapsing course, the disease is monophasic. Overall, the prognosis is favorable. The majority of patients make a full recovery; defect healing is less common . Very severe ( fulminant ) courses can be fatal. The maximum form is the so-called Hurst encephalitis . This leads to the death of vessels and bleeding into the brain tissue, which then dies as a result.

diagnosis

In the magnetic resonance imaging (MRI) typically bilaterally asymmetric herd with complete ring enhancement show. Compared to MS, these are larger and absorb more contrast media . With a lumbar puncture , liquor is obtained , from which the protein content and antibodies are then determined.

therapy

The treatment of choice is the administration of methylprednisolone for 5 days (500–1000 mg / d), after which the symptoms should already be clearly subside. If this therapy does not respond, you should give methylprednisolone for another four weeks. If methylprednisolone is intolerant, immunoglobulins can be given as an alternative . In particularly severe cases and with Hurst's encephalitis, you can resort to cyclophosphamide or remove the autoantibodies by means of plasmapheresis .

swell

  • AWMF guideline ADEM
  • Pschyrembel, Clinical Dictionary, 257th edition