Acute disseminated encephalomyelitis (ADEM): Symptoms, causes, diagnosis and treatment - PowerPoint PPT Presentation

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Acute disseminated encephalomyelitis (ADEM): Symptoms, causes, diagnosis and treatment

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Acute disseminated encephalomyelitis (ADEM) is a neurological, immune-mediated disorder in which widespread inflammation of the brain and spinal cord damages tissue known as white matter. – PowerPoint PPT presentation

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Title: Acute disseminated encephalomyelitis (ADEM): Symptoms, causes, diagnosis and treatment


1
Acute Disseminated Encephalomyelitis (ADEM)
2
Acute Disseminated Encephalomyelitis (ADEM)
  • Acute disseminated encephalomyelitis (ADEM) is a
    neurological, immune-mediated 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.

3
Causes of Acute disseminated encephalomyelitis
(ADEM)
  • The exact cause of ADEM is 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.

4
Symptoms
  • 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.  The progression and severity of
    ADEM varies 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.  The symptoms of ADEM may
    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.

5
Diagnosis of Acute disseminated encephalomyelitis
(ADEM) 
  • A diagnosis of ADEM is 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 as magnetic
    resonance imaging (MRI). An MRI uses a magnetic
    field and radio waves to produce cross-sectional
    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.

6
Treatments
  • No standard therapy for ADEM has 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.  High dose regimens of
    corticosteroids have commonly been used to treat
    individuals with ADEM and generally are
    considered the mainstay of treatment.
    Corticosteroids have led to an improvement of
    symptoms in many cases. Corticosteroids are the
    most widely reported therapy for individuals with
    ADEM. Methyl prednisone is a specific
    corticosteroid that is commonly used for ADEM.

7
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