Acute Disseminated Encephalomyelitis: Symptoms, Treatment, Consequences

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

Acute disseminated encephalomyelitis

The content of the article:

1. Causes and risk factors
2. Forms of the disease
3. Symptoms of acute disseminated encephalomyelitis
4. Features of the course of acute disseminated encephalomyelitis in children
5. Diagnostics
6. Treatment of acute disseminated encephalomyelitis
7. Possible complications and consequences
8. Forecast
9. Prevention
10. Video

Acute disseminated encephalomyelitis (AEM) is an acutely developing disease of the central nervous system, in which, as a result of an autoimmune attack, myelin is destroyed, and, as a result, damage to the white matter of the brain and spinal cord, meninges and the sheath of peripheral nerves.

With WECM, demyelination sites are formed in the brain and spinal cord

Synonym: acute disseminated encephalomyelitis (ADEM).

Causes and risk factors

Symptoms of the disease appear within a short time (from several hours to several days) after an infection, most often a viral one. Less often, the cause of the disease is a past infection caused by bacterial pathogens or mycoplasma, and vaccination. In some cases, the cause cannot be determined.

Sensitization triggers an overactive reaction of the immune system, the cells of which begin to attack not foreign, but the body's own tissues, in this case the protein myelin. In the white matter of the brain, spinal cord, as well as in the nerve sheaths, where myelin is destroyed (this process is called demyelination), inflammatory foci appear, and then areas of necrosis. Presumably, such foci can develop as a result of the direct action of infection (neurotropic viruses), but this has not yet been proven.

Forms of the disease

By origin, the OREM can be viral, bacterial, mycoplasma, post-vaccination, and in the case when the cause is unknown - idiopathic.

Depending on the localization of foci of demyelination, there are:

• acute transverse myelitis;
• opticomyelitis;
• optic neuritis;
• cerebellitis;
• stem encephalitis;
• acute hemorrhagic leukoencephalitis.

Symptoms of acute disseminated encephalomyelitis

The onset of the disease is acute. Against the background of well-being, health worsens, the temperature rises to feverish values, headaches appear, nausea and vomiting may occur. The patient's vision decreases, pains occur in the upper and lower extremities, paresis or paralysis of the extremities, tremor may appear, muscle tone increases or, on the contrary, weakness in the limbs appears, coordination of movements is impaired, speech and fine motor skills suffer. Epileptic-like convulsions and disturbances of consciousness of varying severity may appear. Disorders of cognitive function and the development of psychotic states are possible.

In rabies post-vaccination encephalomyelitis, symptoms appear one to three weeks after rabies vaccination. It starts with a rise in temperature, headaches, and back pain. Then paresis and paralysis occur, it becomes difficult, and in severe cases, urination stops completely.

Neurological symptoms persist for a long time, improvement begins in 2-3 months, but sometimes they persist throughout life, despite the fact that the acute attack does not recur. OREM is characterized by monophasicity, i.e. relapses are not characteristic of it (this distinguishes it from multiple sclerosis and a number of other demyelinating diseases).

Features of the course of acute disseminated encephalomyelitis in children

In 70% of cases, a viral infection is responsible for the onset of the disease in children, namely measles, rubella, chickenpox, herpes types 1 and 6, Epstein-Barr virus, cytomegalovirus, influenza viruses, parainfluenza, mumps. The risk of post-vaccination WECM is extremely low, however, it can be provoked by vaccines against whooping cough, diphtheria, rubella, influenza, poliomyelitis, pneumococcus, measles. Typically, this complication develops after the administration of an insufficiently purified vaccine (containing brain tissue).

The onset of the disease is acute, resembling a respiratory infection: chills, high fever, catarrhal manifestations in the upper respiratory tract. Against the background of fever, vomiting, convulsions, and psychomotor agitation may appear. Typical neurological symptoms usually appear 2-5 days from the onset of the disease. Often the cranial nerves, in particular the optic, are affected, movement disorders, sensory disturbances, muscle spasticity, and pelvic dysfunctions occur.

Despite the turbulent clinical picture, the course of the disease in young patients is favorable. The recovery period lasts about three months; residual neurological disorders persist in only 15% of those who recovered.

Diagnostics

The diagnosis is assumed on the basis of existing complaints and anamnesis (an indication of a recent infectious disease), physical examination, a detailed study of the neurological status (cerebral, meningeal, focal syndromes are investigated). To confirm the diagnosis, laboratory and instrumental studies are performed.

Laboratory methods	Instrumental methods
Clinical blood test (leukocytosis is detected, accelerated ESR)	Contrast-enhanced MRI (large lesions are found in the brain and spinal cord)
General analysis of cerebrospinal fluid (increased protein levels, moderate lymphocytic pleocytosis is possible)	Electroencephalogram (EEG)

WECM must be differentiated from meningitis, encephalitis, multiple sclerosis.

Treatment of acute disseminated encephalomyelitis

Treatment is carried out in a hospital setting. Symptomatic and pathogenetic therapy is carried out.

Symptomatic consists in taking drugs that alleviate the symptoms of the disease (prescribed if necessary):

• antipyretic;
• decongestants;
• anticonvulsants;
• antiemetic;
• sedatives;
• antipsychotics.

Pathogenetic therapy is aimed at stopping the autoimmune process. For this purpose, glucocorticoids, plasmapheresis and / or intravenous immunoglobulin therapy are prescribed. In the case of their insufficient effectiveness, drugs with immunosuppressive action are prescribed, that is, they suppress the excessive activity of the immune system.

Possible complications and consequences

The most formidable complication of the disease can be cerebral edema, which can be fatal. In a hospital setting, this probability is minimal.

Forecast

The lesions in the central nervous system, where the tissue is demyelinated, remain forever. In 25% of cases, some neurological symptoms also persist forever after suffering an AREM, possibly the development of multiple sclerosis. Nevertheless, the neurological deficit in most cases is compensated and a complete recovery occurs. Neurological rehabilitation may be required.

Prevention

There is no specific prophylaxis. General strengthening measures, such as proper nutrition, adequate sleep, a reasonable mode of work and rest, help to protect against infectious diseases and thereby reduce the likelihood of WECM development.

To prevent the development of post-vaccination rabies encephalomyelitis, it is recommended to avoid both overheating and hypothermia during vaccination.

Video

We offer for viewing a video on the topic of the article.

Anna Kozlova Medical journalist About the author

Education: Rostov State Medical University, specialty "General Medicine".

The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!