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Acute Disseminated Encephalomyelitis (ADEM)

Introduction of Acute Disseminated


Encephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM) is a neurological, immunemediated disorder in which widespread inflammation of the brain and spinal cord
damages tissue known as white matter. White matter is tissue composed of nerve
fibres, many of which are covered by a collection of fats and proteins known as
myelin. Myelin, which collectively may be referred to as the myelin sheath,
protects the nerve fibres, acts as an insulator and increases the speed of
transmission of nerve signals. Damage to the myelin sheath affects the nerves
ability to transmit information and potentially can cause a wide range of
neurological symptoms.

Causes of Acute Disseminated Encephalomyelitis (ADEM)

Theexact cause of ADEMis not known. However, most clinical investigators


agree that the disorder is most likely the result of an abnormal immune system
response to an infection or other trigger. Many researchers suggest that ADEM
may represent an abnormal immune reaction directed against the bodys own
tissues (autoimmune disorder). In autoimmune disorders, the bodys natural
defences (e.g., antibodies, lymphocytes) against substances that are perceived
as foreign (antigens) inappropriately begin to attack healthy tissues, for
unknown reasons

Symptoms of Acute Disseminated Encephalomyelitis (ADEM)


In some cases, a viral infection precedes the development of symptoms by two
days to four weeks. Less often, the disorder may follow a vaccination. However,
a preceding event is not always identified and some cases appear to occur
spontaneously. Typically, ADEM is considered a monophasic disorder, which is a
disorder that has a single occurrence or one phase in a particular individual.
Theprogression and severity of ADEMvaries from one person to another.
Initial symptoms usually develop rapidly and may include various symptoms
common to many different illnesses (nonspecific symptoms) including fever,
headaches, irritability, fatigue, lethargy, a general feeling of ill health (malaise),
unintended weight loss and abdominal complaints including nausea and
vomiting. In some cases, these symptoms may be followed by mental status
changes such as confusion, stupor, delirium and, potentially, coma.
Thesymptoms of ADEMmay also be affected by age of onset. Seizures are
common in children and adults. Long-lasting fevers and headaches are more
common in children than adults. Sensory deficits predominantly affect adults.

Diagnoses of Acute Disseminated Encephalomyelitis (ADEM)

Adiagnosis of ADEMis made based upon identification of characteristic


symptoms, a detailed patient history, a thorough clinical evaluation and a variety
of specialized tests including imaging techniques such asmagnetic resonance
imaging(MRI). An MRI uses a magnetic field and radio waves to produce crosssectional images of particular organs and bodily tissues and can demonstrate
characteristic brain lesions in individuals with ADEM. Additional tests to exclude
other conditions may also be performed. Such tests may include infectious,
immunologic, and metabolic tests.

Treatments of Acute Disseminated Encephalomyelitis (ADEM)

Nostandard therapy for ADEMhas been established. Most therapies that have
been used to treat ADEM have some effect of suppressing the activity of the
immune system (immunosuppressive therapy). Such therapies include
corticosteroids, immunoglobulin (IVIg) therapy, or plasmapheresis.

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