Myasthenia Gravis

Normal repeated Contraction

When the nerve impulse orignating in the brain arrives at the nerve
ending, it releases a chemical called acetylcholine.

Acetylcholine travels across the space to the muscle fiber side of

the neuromuscular junction where it attaches to many receptor
sites. The muscle contracts when enough of the receptor sites have
been activated by the acetylcholine.

Then the acetylcholinease decompose the acetylcholine in order to

prepare for the following contractions.
 Myasthenia Gravis
 Autoimmune disease
 Post-synaptic disorder of neuromuscular junction
 Antibodies against post-synaptic acetlcholine
receptors (Anti-AChR)
 block, bind, modulate AChR reversibly
 receptor lysis
 membrance lysis by associated complement
cascades

Results in fatigable muscle weakness

 Clinical features
 Weakness with fluctuation and variability over
the course of a day or over months
 True fatigue -- ＞ weakness after use
 Orbicularis oculi and extra-ocular muscles
typically involve early and more severely
 Variable ptosis and diplopia
 Mimic cranial nerve or gaze palsies
 May be the sole manifestation “ocular MG”
 Clinical features
 Limb weak : proximal worse than distal -- ＞
axial / truncal muscles, test neck flex/ext.
 Bulbar weak: nasal speech, dysphagia -- ＞
aspiration pneumonia, malnutrition
 Diaphragmatic weak: respiratory failure
 Monitor forced vital capacity by serial simple
spirometry
 Diagnosis

 Bedside edrophonium (Tensilon) test

 Electrophysiological evaluation
 Serum anti-AChR assays
 “ice test”
Tensilon test
 Electrophysiological evaluation
Electromyography studies
 Repetitive nerve stimulation is used to check for
a pattern of response that is characteristic of MG
 Serum anti-AChR assays
 A blood test for the abnormal acetylcholine
receptor antibody, when detected with an
elevated concentration , strongly indicate
the MG.
Ice test
 Associated Conditions

 Thymic abnormality (thymic hyperplasia or

thymoma) by CT thorax
 Thyroid disorder, check serum sTSH
 Other autoimmune disorders if clinically
suspected
 Treatment
For Ocular MG
 Anti-cholinesterase drug - pyridostigmine

For generalized MG (add below)

 Steroid - prednisolone
 Thymectomy - For younger patients
 Other immunomodulating drug - azathioprine if
still refractory to treatment
 Treatment
MG “crisis”
 Rapid worsening of symptoms, in particular
respiratory failure, dysphagia, and severe limb
weakness
 A short course of plasma exchange usually
useful (action within days and lasted for few
weeks) “buy time”
 Long-term medications for control
Watch out for respiratory failure!!
Pathophysiology of respiratory failure
 Profound respiratory muscle dysfunction
 Hypoxaemia because of hypoventilation and
microatelectasis arising from retention of secretions
 Hypercapnia because of late signify and impending
respiratory arrest
 Bulbar involvement cause upper airway obstruction
and significant pulmonary aspiration
 Infection of the lower respiratory tract cause furter
deteriorate gas exchange
 Clinical diagnosis
 Progressive weakness.
 Weakness and fatigue but does NOT appear wheezy or
cyanosed
 Prefers to sit up or lie still in bed, becomes breathless on
talking, uses accessory muscles of respiration
 Paradoxical abdominal movememt if diaphragm weakness
 Diaphragm paralysis is paticular prone to hypoventilation
during sleep or supine position
 Impaired intercostal and abdominal muscle function may have
respiratory distress or failure in upright position
 Risk of aspiration
 Monitoring
 Forced vital capacity (NOT peak expiratory flow
rate)
 ＜ 30ml/kg impaired clearance of secretions
 ＜ 10ml/kg frank ventilatory failure
 Pulse oximetry, especially during sleep
 Arterial blood gas - late changes