Review journal myasthenia gravis and treatment

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Review journal myasthenia gravis and treatment

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• 28 /2/2008

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case

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?????????????????

• Full conscious
• Other with in normal
• Neurological sign
• Ptosis both eyes
• Motor power gr 0 all
• Reflex gr 0 all
• BBK normal
• Clonus negative

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• Progress respiratory failure on ET with
  ventilation
• DDx 1.myasthenia gravis 2.GBS
• Prostigmine test positive
• Definite diagnosis
• generalized myasthenia gravis
• Treatment
• Mestinon

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Myasthenia gravis

• CLINICAL FEATURES
• ptosis or diplopia
• weakness
• bulbar symptoms
• dysphagia
• DIAGNOSIS
• pyridostigmine test

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Management

• improving the availability of Ach in the NMJ to
  improve neuromuscular transmission
• Symptomatic
• Cholinesterase inhibitors
• Pyridostigmine
• Neostigmine

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Progress case

• After treatment by mestinon
• Clinical not improvement to extubation
• Cause
• 1.severe myasthenia gravis
• 2.cholinergic crisis

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Questions?

• What is an alternative treatment in myasthenic
  crisis patients who have not respond to
  anticholinergic drug?

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Treatment

• Acute care setting
• Cholinesterase inhibitors
• Plasma exchange
• Intravenous immunoglobulin
• Long term management
• Cholinesterase inhibitors
• Immunotherapy
• Corticosteroid treatment

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Role of IVIG and Plasma exchange in Myasthenia
crisis?

• Plasma exchange
• removal of the pathogenic antibodies
• antinicotinic ACh receptor, anti-MUSK antibodies
• IVIG
• mechanism of action in autoimmune diseases is
  quite complex and not fully understood

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Literature searching
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• Plasma exchange for myasthenia gravis
• IVIG for myasthenia gravis

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• Objectives
• Efficacy of plasma exchange in the short- and
  long-term treatment of myasthenia gravis
• Search strategy
• Term myasthenia gravis and plasma exchange
• Cochrane Neuromuscular Disease Group register,
  MEDLINE and EMBASE
• randomised trials

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• Types of participants
• The diagnosis of myasthenia gravis was based on
  the following three criteria
• Two categories of patients
• Patients with major exacerbations of generalised
  myasthenia gravis, defined as the appearance or
  reappearance of at least one of the following
  symptoms
• difficulty in swallowing
• acute respiratory failure (needing mechanical
  ventilation)
• major functional disability responsible for the
  discontinuationof physical activity.
• Patients with chronic generalised myasthenia

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• The primary outcome measures
• patients treated for exacerbation
• change in a specific muscle score after plasma
  exchange
• patients treated for chronic myasthenia gravis
• change in a functional scale

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• Types of intervention
• treatment with plasma exchange alone
• plasma exchange associated with steroids and/or
  immunosuppressive drugs
• Data collection and analysis
• One author extracted the data and a second author
  checked them

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conclusions

• No adequate randomised controlled trials
• Many case series studies report short-term
  benefit from plasma exchange in myasthenia gravis

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• Objective
• Efficacy of intravenous immunoglobulin for
  treating exacerbations of myasthenia gravis or
  for chronic myasthenia gravis
• Search strategy
• Cochrane Neuromuscular Disease GroupTrials
  Register, MEDLINE and EMBASE
• Term myasthenia gravis and intravenous
  immunoglobulin
• RCT

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• Types of participants
• The diagnosis of myasthenia gravis was based on
  the following three criteria
• Two categories of patients
• Patients with major exacerbations of generalised
  myasthenia gravis, defined as the appearance or
  reappearance of at least one of the following
  symptoms
• difficulty in swallowing
• acute respiratory failure (needing mechanical
  ventilation)
• major functional disability responsible for the
  discontinuationof physical activity.
• Patients with chronic generalised myasthenia

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• Primary outcome measure
• Exacerbation
• change in a specific score
• before VS 7 to 15 day after start of treatment
• chronic (severe but stable)MG
• before VS least 6 months after start of
  treatment

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• Secondary outcome measures
• In participants treated for exacerbation
• least one grade in a functional scale
• Weaning fromventilation before day 15 after start
  of treatment
• Absolute mean reduction in circulating
  concentrations of anti-AChR antibodies after
  treatment.

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• Data collection and analysis
• One review author extracted the data
• Two others checked these data
• no formal meta-analysis was performed
• 6 RCT

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Day 0 to 42
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conclusions

• one randomised controlled trial of IVIg versus
  placebo
• no significant difference between IVIg and plasma
  exchange.
• no significant difference between 1 g/kg and 2
  g/kg of IVIg
• no significant difference between IVIg and oral
  methylprednisolone.

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Conclusion of review

• No evidence based clinical data to support the
  use of plasma exchange and IVIG treatment for
  myasthenic exacerbations
• Efficacy?, dose?, duration?
• Small data of RCT
• New randomized controlled clinical studies will
  be needed to study

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• But a new choose to be use
• Clarify risk and benefits