Myasthenia Gravis

• Autoimmune disease is characterized by muscle

weakness that fluctuates, worsening with
exertion, and improving with rest.
• Miastenia Gravis (MG) adalah penyakit dimana
target antibodinya adalah neuromuscular junction
dari otot rangka.
Prevalensi
• 20 per 100,000 in the US population.
• Two-thirds of the patients, the involvement of
extrinsic ocular muscles (EOMs) presents as the
initial symptom, usually progressing to involve
other bulbar muscles and limb musculature,
resulting in generalized myasthenia gravis.
• Below 40 years of age, female : male ratio is about
3 : 1; however, between 40 and 50 years as well as
during puberty, it is roughly equal. Over 50 years, it
occurs more commonly in males.
• Childhood MG is uncommon in Europe and North
America, comprising 10% to 15% of MG cases.
• In Asian countries though, up to 50% of patients
have onset below 15 years of age, mainly with
purely ocular manifestations.
Klasifikasi MG
1. Early-onset MG: age at onset <50 years. Thymic
hyperplasia, usually females.
2. Late-onset MG: age at onset >50 years. Thymic
atrophy, mainly males.
3. Thymoma-associated MG (10%–15%).
4. MG with anti-MUSK antibodies.
5. Ocular MG (oMG): symptoms only affecting
extraocular muscles.
6. MG with no detectable AChR and muscle-specific
tyrosine kinase (MuSK) antibodies.
• MG patients with Thymoma almost always have
detectable AChR antibodies in serum.
• 15% of generalized MG patients do not have
antiAChR antibodies in current lab assays.
• They have atypical clinical features like selective
facial, bulbar, neck, or respiratory muscle weakness
with occasional marked muscle.
• Atrophy and with relative sparing of the ocular
muscles. Respiratory crises are more common with
involvement of muscle groups like paraspinal and
upper esophageal muscles.
• Class I MG:
• Any ocular muscle weakness
• Weakness of eye closure
• All other muscle strengths are normal
• Class II MG:
• Mild weakness affecting muscles other than ocular
muscles
• Ocular muscle weakness of any severity.
• Class IIa MG:
• Predominantly affecting limb, axial muscles, or both
• May also have lesser involvement of oropharyngeal
muscles.
• Class IIb MG:
• Predominantly affecting oropharyngeal, respiratory
muscles, or both, (ii) may also have lesser or equal
involvement of limb, axial muscles, or both.
• Class III MG:
• Moderate weakness affecting muscles other than ocular
muscles
• May also have ocular muscle weakness of any severity.
• Class IIIa MG:
• Predominantly affecting limb, axial muscles, or both.
• May also have lesser involvement of oropharyngeal muscles.
• Class IIIb MG:
• Predominantly affecting oropharyngeal, respiratory muscles, or
both
• May also have lesser or equal involvement of limb, axial
muscles, or both.
• Class IV MG:
• Severe weakness affecting muscles other than ocular muscles
• may also have ocular muscle weakness of any severity.
• Class IVa MG:
• Predominantly affecting limb, axial muscles, or both
• May also have lesser involvement of oropharyngeal muscles.
• Class IVb MG:
• Predominantly affecting oropharyngeal, respiratory muscles or both
• May also have lesser or equal involvement of limb, axial muscles, or both.
• Class V MG:
• Intubation with or without mechanical ventilation, except when employed
during routine postoperative management
• The use of feeding tube without intubation places the patient in class IVb.
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Myasthenia Gravis : clinical feature
• Hallmark of myasthenia gravis : fatigable muscle weakness
• 40% cases begin with ocular muscles with various combinations (diplopia + ptosis);
often asymmetric; pupils are normal.
• oropharyngeal weakness - dysphagia and dysarthria; nasal regurgitations, aspirations;
vocal cords are only exceptionally affected
• limb & postural muscles are generally less affected; limbs (upper > lower; proximal >
distal; may be asymmetric) are never affected alone!
• weakness becomes generalized in majority (15% remain confined to ocular muscles);
examination of neck flexors is most sensitive in demonstrating generalized disease
(holding head up from surface of examining table while lying supine - gravity cannot
be overcome for more than few seconds).
• Fluctuating Nature
• weakness varies in course of single day (sometimes within minutes), and from day to
day (or over longer periods); constant weakness may occur.
• during physical stress (esp. respiratory infection, surgery) precipitous worsening may
occur!
• symptoms are exacerbated by heat and improved by cold (e.g. ptosis may improve
after cooling of eyelid with ice pack).
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 Class of Myasthenia Gravis

• Class I: Any ocular muscle weakness; may have weakness of eye closure; all other muscle
strength is normal
• Class II: Mild weakness affecting other than ocular muscles; may also have ocular muscle
weakness of any severity
• Class IIa: Predominantly affecting limb, axial muscles, or both; may also have lesser involvement
of oropharyngeal muscles
• Class IIb: Predominantly affecting oropharyngeal, respiratory muscles, or both; may also have
lesser or equal involvement of limb, axial muscles, or both
• Class III: Moderate weakness affecting other than ocular muscles; may also have ocular muscle
weakness of any severity
• Class IIIa: Predominantly affecting limb, axial muscles, or both; may also have lesser involvement
of oropharyngeal muscles
• Class IIIb: Predominantly affecting oropharyngeal, respiratory muscles, or both; may also have
lesser or equal involvement of limb, axial muscles, or both
• Class IV: Severe weakness affecting other than ocular muscles; may also have ocular muscle
weakness of any severity
• Class IVa: Predominantly affecting limb, axial muscles, or both; may also have lesser involvement
of oropharyngeal muscles
• Class IVb: Predominantly affecting oropharyngeal, respiratory muscles, or both; may also have
lesser or equal involvement of limb, axial muscles, or both; use of a feeding tube without
intubation
• Class V: Defined by the need for intubation, with or without mechanical ventilation, except when
used during routine postoperative management
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