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

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Acute Disseminated Encephalomyelitis (ADEM)
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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.

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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.

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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.

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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.

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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.

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