1. Diagnostic Tests • Electromyography (EMG) • Characteristic of MG: a rapid reduction in the amplitude of compound muscle action potentials in response to repetitive nerve stimulations • Motor nerve conduction velocity is normal • Anti-AchE medication is stopped 6 – 24 hrs. before testing • Best to test weak muscles or proximal muscle groups • Electric shocks are delivered at a rate of 2-3 sec to the appropriate nerve

2. Diagnostic Tests • AChR or MuSK receptor antibodies • Detection of anti-AChR antibodies provides a reasonably sensitive and highly specific test for the diagnosis of MG • juvenile myasthenics who do not have anti-ACh antibodies have instead MuSK R (tyrosine kinase receptor) antibodies, which also is localized at the NMJ • Measured levels of anti-AChR does not correspond well to the severity of MG • Negative test does not exclude the disease

3. Diagnostic Tests • Anticholinesterase Test • Anticholinesterase inhibitors rolong and exaggerate the effects of Ach in the synapse and thereby provide an increment in muscle power • Edrophonium chloride (5mg initial dose) is commonly used for diagnostic testing because of its rapid onset (30s) and short duration (5min) of its effect • Reserved for patients with clinical findings suggestive of MG but negative anti-AChR and EMG • False-positives occur in patients with other neurologic disorders (e.g. amyotrophic lateral sclerosis)

4. Diagnostic Tests • Anticholinesterase test (recommendations for infants <2yrs old) • A specific fatigable weakness should be measurable (e.g. ptosis of the eyelids) • Edrophonium is not recommended for use in infants because its effect is too brief for objective assessment and an increased incidence of acute cardiac arrhythmias is reported in infants • IM Neostigmine (0.5-1.5mg) is instead used • Dosage: 0.04 mg/kg; if the result is (-), another dose of 0.04 mg/kg may be administered after 4hrs). The peak effect is seen in 20–40 min • Should not be given IV because of risk of cardiac arrythmias

5. Reference • Nelson Textbook of Pediatrics, 18th ed. • 2013 block A trans