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DIAGNOSIS AND TREATMENT OF MYASTHENIA

GRAVIS

Lecturer Adviser :
Dr.Donny H Hamid.SpS

Relanfa Farando
110201234

Clinical Science of Neurology


General Hospital Pasar Rebo East Jakarta
11 September 2017 16 October 2017
Definition

Myasthenia gravis (MG) is the most common primary disorder of


neuromuscular transmission. It is characterised clinically by
fluctuating painless muscle weakness, which worsens with exercise
and towards the end of the day, and improves with rest. It is usually
caused by antibodies to postsynaptic proteins, of which three
namely nicotinic acetylcholine receptor (AChR), muscle-specific
tyrosine kinase (MuSK) and low-density lipoprotein receptor-related
protein 4 (LRP4)
Epidemiology

200/ Million case Young Adult :


Females
Male/Females Risk
Increase with Age
Classification

Subtypes of MG According to
Antibodies

1. early-onset MG: age at onset <50 years. Thymic hyperplasia, usually


2. late-onset MG: age at onset >50 years. Thymic atrophy, mainly
3. thymoma-associated MG (10%15%)
4. MG with anti-MUSK antibodies
5.ocularMG (oMG): symptoms only affecting extraocular muscles
6.MG with no detectable AChR and muscle-specific tyrosine kinase (MuSK)
Other
Classification

Osserman Classification of Myasthenia Gravis (modified from MGFA)


Class I pure ocular
Any ocular weakness, may have weakness of eye closure;
Strength of all other muscles is normal.
Class II ocular plus other deficits
IIa Mild weakness aff ecting limbsbulbar muscles;
IIb Predominantly aff ecting limbs oropharyngeal.
Class III moderate generalized
IIIa Moderate weakness Predominantly aff ecting limb, axial muscles, or both;
IIIb Moderate generalized weakness predominantly aff ecting oropharyngeal,
respiratory muscles, or both.
Class IV severe generalized
IVa Severe weakness aff ecting limbs;
IVb Severe weakness aff ecting limbs. Predominantly aff ects bulbar muscles.
Class V
Ventilator dependent except when used during routine postoperative management
Etiopathogenesis

Autoantibodies

Thymus

Genetic

Enviromental
Pathofisiologi

Antibodies to AChR protein:


85 % of patients with generalized
myasthenia and 60% of those with
ocular myasthenia shows AChR
Antibodies
Anti-MuSK Ab(40% of seronegative
cases)

An immune response to muscle-


specific kinase (MuSK) can also result
in myasthenia gravis, possibly by
interfering with AChR clustering
1. Anti-AChR antibodies bind and crosslink
the AChRs, resulting in increased
endocytosis and degradation of AChRs by
the muscle cell.
2.anti-AChRs bind complement factors at
the post - synaptic membrane, leading to
focal lysis of the postsynaptic folds at the
neuromuscular junction by the membrane
attack complex.
3.the destruction of other AChR-associated
proteins, such as utrophin, rapsyn and
voltage-gated sodium channels, impairs
neuromuscular transmission as these
proteins are involved in neuromuscular
junction formation and maintenance
Clinical Manifestastion

Levator palpebrae weakness, may be unilateral, N


Ptosis III,IV,V1 weakness, Difficulty with eye closure

Facial muscle & an expressionless face, or a snarl when the patient


Jaw Weakness tries to smile, mastication,

dysphagia, weakness of the pharyngeal and tongue


dysarthria ,choking, musculature, laryngeal weakness
dysphonia

Upper commoner than lower extremity


extremity weakness
weakness
Ocular involvement (A), myasthenic snarl (B)
Diagnosis

Anamnesis : Physical Examination :


Ocular (Ptosis, Peek sign)
Insidious onset Frontalis (Worried/surprised look,
Unilateral)
Symptoms may be worsened Oropharyngeal (Chewing and swallowing,
by some agents changes in the voice
Laryngeal (hoarseness, choking, dysphagia,
Slowly progressive course respiratory dysfunction)
Symptoms often fluctuate in Facial appearance (myasthenic snarl)
Weak trunk or limb muscle (Neck flexors ,
intensity during the day triceps and extensors of the wrist and
fingers and ankle dorsiflexors )
Muscle weakness in MG can be exacerbated by emotional upset, hot
temperature, infection, menstruation, physical exertion, pregnancy, surgery,
thyroid disease (hyperthyroidism or hypothyroidism) and many classes of
drugs
Antibodies Test

Repetitive nerve stimulation (RNS)


Neurophysiology single-fibre electro myography
(SFEMG)

edrophonium chloride, a short-acting


acetylcholinesterase inhibitor It is prudent
to administer atropine prophylactically
Edhorphonium Test because of the risk of bradykinesia and
cardiac arrest.

ice cube is placed over the drooping


eyelid for about two minutes, and if
Ice Pack Test there is improvement in the ptosis, it
suggests a neuro muscular transmission
disorder
Differential Diagnosis
Treatment
Emergency in
Myasthenia Gravis

Myasthenic Crisis (MC)


Severe weakness of the bulbar (innervated by cranial nerves) and/or
respiratory muscles, enough to cause inability to maintain adequate
ventilation and/or permeability of upper airways, causing respiratory failure
that requires artificial airway or ventilatory support.
Epidemiology

The median time to first 15-20% of myasthenic


myasthenic crisis form onset Women : Men
of MG ranges form 8-12 patients are affected by
myasthenic crisis 2 :1
months

Patients in crisis requiring


< 50 years old affect endotracheal intubation
woman : man 4 :1 spend a median of 17 days in
the hospital Currently,
> 55 years old affect mortality is 4% and is
women : man equally primarily the result of
comorbid medical conditions
Warning Symptoms !!

1.Abdominal muscle paradox 11.Orthopnea


2.Accessory muscle use 12.Pausing during speech to take a
3.Cough after swallowing breath
4.Difficulty in clearing secretions 13.Rapid shallow breathing
5.Dysphagia 14.Staccato speech or a nasal
6.Hypophonia quality to speech
7.Inability to raise the head due to 15.Single breath count of < 15
neck muscle weakness 16.Stridor
8.Forehead sweating 17.Tachypnea
9.Jaw weakness (jaw closure weaker 18.Tongue weakness
than jaw opening) 19.Weak cough
10.Nasal regurgitation 20. Wet, gurgling voice
Diagnosis

FVC <1 L, negative inspiratory force of 20 cm H2O or less, and the need for
ventilatory support
Arterial blood gas analysis commonly shows hypercarbia before hypoxia
Patients with previous diagnosed Myasthenia gravis
Patients without a previous diagnosis of Myasthenia gravis. Suddenly show a
severe respiratory distress, facial weakness, airway collapse, and muscle
failure. Initially, oxygenation is preserved.
1.Admit to ICU
2.Assessment of respiratory function
3.Elective intubation
4.Rapid therapy IV IG ,
5.Plasmapheresis
6.Immunomodulating therapy
7.Careful weaning
8.Taking care of complications
9.Anticholinesterase inhibitors
10.Thymectomy