Acute disseminated encephalomyelitis

The acute disseminated / demyelinating encephalomyelitis - (ADEM or ADE), which is also referred to as perivenous encephalomyelitis or Hurst Encephalitis is a rare, acute inflammatory disease of the central nervous system that often occurs to four weeks after infection. Most children are affected. About the age of 40, the disease is rare. If no history of prior infection known, often remains unclear whether it is not the beginning of a multiple sclerosis ( MS) is, to consist of smooth transitions.

Etiology / origins

Acute disseminated encephalomyelitis is a usually triggered by a preceding viral infection autoimmune disease. The previous infection can not always be determined a history. Very rarely can also paravacciniös arise ( in temporal association with vaccination ) ADEM. The case activated T lymphocytes directed against proteins of the cerebral white matter.

Symptoms

The clinical presentation varies depending on the localization of the lesions, but usually also includes neuropsychological symptoms such as psychomotor retardation or lack of awareness. Headache, neck stiffness, vomiting, severe depression, epileptic seizures, a spinal cord injury, incomplete or unilateral paralysis, speech disturbances, ataxic gait, and especially bilateral optic neuritis with visual disturbances may also occur. The inflammation of the optic nerve is the characteristic symptom.

Course and prognosis

The definitive demarcation to multiple sclerosis is only possible in the clinical course. As part of the ADEM there is no shear- like course, the disease is monophasic. The overall prognosis is favorable. The majority of patients recover completely, there is a rare defect healing. Very severe ( fulminant ) curves can be fatal. The maximum form is the so-called Hurst encephalitis. This leads to death in the course of blood vessels and bleeding in the brain tissue, which then stalls in the row.

Diagnosis

In magnetic resonance imaging (MRI ) typically show bilateral asymmetric lesions with complete ring enhancement. These are compared to the bigger MS and take more contrast agents. In the lumbar puncture to win cerebrospinal fluid, from which one then determines the protein content and antibodies.

Therapy

The treatment of choice is the administration of methylprednisolone for 5 days ( 500-1000 mg / d ), followed by a significant decay of the complaints should already be visible. Will not talk to this therapy, you should give methylprednisolone for another four weeks. Intolerance of methylprednisolone alternative administration of immunoglobulins is possible. In particularly severe cases and in the Hurst encephalitis can resort to cyclophosphamide or remove the autoantibodies by plasmapheresis.

Swell

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

• Inflammatory disease of the central nervous system