acute disseminated encephalomyelitis in children

Introduction

Brief introduction of acute disseminated encephalomyelitis in children Acutely disseminated encephalomyelitis (ADEM) is a cellular immune-mediated autoimmune disease characterized by acute inflammation and demyelination of the central nervous system. The disease usually occurs after acute infection or vaccination, so it is also called encephalomyelitis after infection or post-vaccine encephalomyelitis. There is no history of vaccination or infection before the disease, which can be collectively referred to as acute disseminated encephalomyelitis. basic knowledge Sickness ratio: 0.0002%-0.0008% Susceptible people: children Mode of infection: non-infectious Complications: ataxia, paraplegia, urinary retention, somatosensory disturbance

Cause

The cause of acute disseminated encephalomyelitis in children

Virus infection (45%):

ADEM is associated with a variety of infections, most of which are associated with rash disease, most commonly in measles, followed by rubella, varicella, herpes zoster, herpes simplex, mumps, flu, infectious mononucleosis and mycoplasma infection.

Immunization factor (35%):

Immunization is common in rabies vaccine, whooping cough, diphtheria, measles vaccine and flu vaccination.

Other diseases (20%):

Most patients have non-specific upper respiratory tract infections, gastroenteritis, fever and other prodromal symptoms before the onset, and some have no incentives.

Pathogenesis

The most prominent pathological feature of this disease is the white matter in the central nervous system. The myelin sheath is seen around the blood vessels, especially around the small veins. In the early stage of the disease, numerous demyelinating lesions with a diameter of about 1 mm can be seen, which are widely distributed in the brain and spinal cord. White matter, with white matter around the ventricle, optic nerve and temporal lobe most obvious, axonal relatively intact, perivascular edema and obvious inflammatory cell infiltration, advanced disease, glial cell proliferation around the lesion, scar formation, generally speaking of this disease The pathological changes of the lesions are the same, reflecting that the disease is a clinical single-phase disease.

Prevention

Prevention of acute disseminated encephalomyelitis in children

Do a variety of vaccination work, and actively prevent and treat various infectious diseases, including infectious diseases of the respiratory tract and digestive tract.

Complication

Complications of acute disseminated encephalomyelitis in children Complications, ataxia, paraplegia, urinary retention, somatosensory disturbance

Can cause the nerves of the brain, occur ataxia, can have paraplegia or quadriplegia, sensory disturbances and urinary retention.

Symptom

Acute disseminated encephalomyelitis symptoms in children Common symptoms Convulsion meningeal irritation coma Ataxia edema Rhizomes Awareness disorder Dizziness Sensory convulsions

The disease can occur at any age, common children and young people, acute onset, a history of pre-infection within one month before the disease, after a few days, neurological symptoms appear, the course of the disease is generally monophasic, symptoms and signs can be successive It appears completely within 2 weeks. According to the clinical features, it can be divided into 3 types. The cerebrospinal type is the brain and the spinal cord. The brain type refers to the prominent brain symptoms. The spinal cord type is the spinal cord, and the fever is optional. Diverse performance, mainly brain symptoms, often headache, dizziness, vomiting, convulsions, dysphoria, mental symptoms and meningeal irritation, brain stem symptoms may have cranial nerve involvement, cerebellar damage may have ataxia , nystagmus, etc., the spinal cord affected parts are different, may have paraplegia or quadriplegia, sensory disorder plane and urinary retention.

Examine

Examination of acute disseminated encephalomyelitis in children

In the acute phase of cerebrospinal fluid (CSF), more than half of the patients may have abnormalities, lymphocytes are mild to moderately elevated, protein content is normal or slightly elevated, sugar and chloride content are normal, and IgG index of cerebrospinal fluid is elevated in some children (normal ratio <0.7), oligoclonal (OB) antibody was positive.

EEG has more diffuse slow wave activity changes, brain CT examination shows more normal, brain MRI shows that white matter multiple diffuse in asymmetric long T2 signal, can also invade basal ganglia, thalamus, cerebellum, brain stem and spinal cord.

Diagnosis

Diagnosis and differential diagnosis of acute disseminated encephalomyelitis in children

diagnosis

The diagnosis of this disease is mainly based on typical medical history, disease course, nervous system involvement and brain MRI characteristics, but other cerebrospinal diseases should be excluded. A special type of acute disseminated encephalomyelitis should be noted in diagnosis, called acute hemorrhagic white matter. Encephalitis, some people think that it is a violent mode of acute disseminated encephalomyelitis, a sharp onset, age 2.5 to 60 years old, pathological features are unilateral or bilateral asymmetrical parietal lobe, extensive hemorrhage in the posterior frontal lobe, edema, Necrosis, brainstem and spinal cord can also be affected, obvious myelin loss around the blood vessels, lymphocytic infiltration, pre-existing children often have viral infections, neurological symptoms such as fever, hemiplegia, aphasia, convulsions, etc. Early coma, rapid death within a few days, high mortality, poor prognosis.

Differential diagnosis

Acute disseminated encephalomyelitis should be differentiated from the following major diseases: acute viral encephalitis, multiple sclerosis, disseminated necrotizing leukoencephalopathy, and the like.

Acute viral encephalitis

Acute onset, may have fever, clinical if the brain symptoms are prominent, the two diseases are difficult to identify, such as herpes simplex virus type I caused by encephalitis, often involving the temporal lobe, frontal and insular leaves, brain CT or MRI For bilateral asymmetry, lesions involving gray matter and white matter, the diagnosis of viral encephalitis should be excluded, and the increase in serum and cerebrospinal pathogen-specific IgM antibodies can help diagnose.

2. Multiple sclerosis

It is an autoimmune disease of the central nervous system. It is rare in children, and its clinical manifestations are complex and diverse. The heterogeneity of the lesions and the multifocality of the lesions are the characteristics of this disease. The disease often resolves and recurs early in the disease, according to the damage of the nervous system. Invasion of white matter has coexistence of new and old lesions, lack of space-occupying effect and edema around the lesion.

3. Disseminated necrotizing leukoencephalopathy

For the antifolate preparation methotrexate causes leukoencephalopathy, the most common in acute lymphocytic leukemia, prevention of meningeal leukemia by intrathecal injection or simultaneous radiation therapy can increase its toxicity, according to medical history and imaging is not difficult and acute dissemination Identification of encephalomyelitis.

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