Table 1

Signs of fatiguable muscle weakness in myasthenia gravis, how to elicit them and sensitivities and specificities from the published literature47–51

SignHow to elicitSensitivitySpecificity
Fatiguable ptosis on upgaze (Simpson’s test)Measure the marginal reflex distance (see figure 3) before and after 30 s of looking up. Photography of the examination may help73% (when combined with ice-pack test)97%
(when combined with ice-pack test)
Peek signAsk the patient to close the eyes gently. Separation of the eyelid margins and visibility of the sclera within 30 s is a positive finding4%–12%98%–99%
Curtain sign and enhanced ptosisPassive elevation of the ptotic eyelid unmasks ptosis on the other side (curtain sign); passive elevation of the non-ptotic eyelid results in worsening ptosis on the affected side (enhanced ptosis). These phenomena occur due to Hering’s law of equal innervation and are not specific to myasthenia gravisUnknownUnknown
Cogan’s lid twitchAsk the patient to look down for 30 s followed by quick movement into primary gaze. Transient improvement in ptosis with brief lid retraction is a positive finding50%–99%75%–99%
Quiver movements and glissadesCogan also described characteristic high-velocity short-amplitude eye movements (quivers), often followed by slow corrective drifts to a target (glissades)UnknownUnknown
Lid-hopping signAsk the patient to perform alternating horizontal saccades (ie, look left, look right). Bilateral transient lid twitch is a positive findingUnknownUnknown
Ice-pack testMeasure ptosis before and after application of ice over the eyelid for 2 min, ideally also with a warm pack control to eliminate the effect of rest. Improvement of ptosis ≥2 mm is a positive finding20%–90%30%–100%
Bienfang’s testMeasure ptosis in primary gaze before and after 30 s of asking the patient to squeeze the eyelids tightly. Transient improvement in ptosis is a positive finding94%91%
Fatiguable limb weaknessAssess muscle strength (eg, shoulder abduction) before and after 30 s of repetitive exercise. Blind the examiner to the side exercisedUnknownUnknown