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Myastenia Gravis

Siti Nur Qomariah, S.Kep.Ns.,M.Kep

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DEFINITION

Autoimun disorders influence


neuromuscular transmission characterized
by weakness and fatigability on
sustained effort

• Antibodies directed against


acetylcholine receptor (AChR)

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INCIDENS

• Woman > man


• 40th >>

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Pathophysiology
• IgG interact with the postsynaptic AChR at
the nicotinic neuromuscular junction(NMJ).
• This reduces the number of functional
receptors by blocking Ach attachment, by
increasing the degradation of receptors and
by complement induced damage to the NMJ
• MG pts have a reduced AChR density and have
30% the normal number of AChR

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• The reduced AChR density leads to
decreased amplitude in the post
synaptic region leading to failure in
initiation in muscle fibre contraction.
• When it occurs at many NMJs
manifests as weakness of voluntary
muscle feature

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Classification
1. Ocular myastenia : influence eye muscle 
ptosis, diplopia
2. Mild generalized myastenia : spread out to
muscle skelet and bulbar
3. Moderate generalized myastenia :
inresponsive to drugs
4. Severe generalized myastenia :
- Acute fulmating myastenia : acute onset of
respiratory muscle weakness until 6 months
- Late severe myastenia : > 2 years
5. Myastenia crisis : recurent

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Manifestations

• Voluntary Descendens Muscle weakness, with


fatigability, relieved with rest
• 15% pts have disease confined to the eyes
• 85% generalised ocular, facial, bulbar, limb
• Respiratory muscles affected mildly however
in myasthenic crisis resp failure requiring
support may be required
• Symptoms of diplopia, ptosis, dysarthria,
regurgitation,dysphagia are all common.

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Dx test

• EMG
– Recording of compound muscle action
potentials(CMAP)
– Following repetitive stimulation of motor
nerve
– In MG this leads to reduction in CMAP
(>10%)
• Detection of anti-AChR antibodies
– In 80-85% cases
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Management

• Anticholinesterase drugs potentiate the


Ach at receptor sites (Pyridostigmine,
Prostigmin, mestinon)
• Thymectomy best results and advocated
early in all patients regardless of
severity or presence of thymoma.
Earlier remission, lower mortality,
greater delay in recurrences.

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• Immunosuppression - corticosteroids
mainstay
– effective in 70%
– best results when used high dose and
reduced
– average of 4months with some indefinitely
– Azothiprine and methotrexate effective
adjuncts, 80% helped, few remission

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ICU
• Frequent estimations of VC and inspiratory
force
• Consideration of mechanical ventilation given
to those with severe bulbar and respiratory
muscle involvement
• Deterioration of ABGs often late sign
• Good ICU care, maintaining K, Ca Mg levels
normal. Physio and NG feeding.
• If adjustment of medications not sufficient
to see improvement need to consider high
dose steroid and plasma exchange therapy.

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Nursing Dx

• Ineffective breating pattern


• Ineffective airway clearence
• Impaired gas exchange
• Impaired physical mobility
• Impaired skin integrity
• Anxiety
• Self care defisit

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