====== Ocular Myasthenia Gravis ====== ===== Clinical Features ===== * Triad: Ptosis, Oculomotor paresis and Obicularis oculi weakness * **Ptosis** may be unilateral or bilateral * Alternating ptosis is specific for this condition * **Eyelid fatigue** * Ptosis worsens with prolonged upgaze or upon return to primary gaze * **Cogan's lid twitch** * After brief but sustained downgaze, a saccade to primary gaze results in the eyelid quickly rising and falling (1 mm or more typically) * **Eyelid Curtaining** * When lifting the more ptotic eyelid, the contralateral eyelid will droop more because of Hering's law * Resting for 5 minutes with the eyes closed will cause improvement in ptosis * Strabismus that fluctuates or appears fatiguable * Any type of strabismus possible * prolonged or sustained gaze in the field of action of the affected muscle may show increasing paresis * Lagophthalmos is rare * attempt to open the eyelid against forced closue- if the eyelid can be opened it suggest obicularis weakness ===== Diagnostic Tests ===== ==== Antibody Tests ==== * **Acetylcholine Receptor Antibody** * sensitivity 50% in Ocular myasthenia vs 90% in Generalized myasthenia * The most specific test, no false positives have been reported * Muscle specific tyrosine kinase antibodies (MuSK) * Rare cares of this antibody present in the setting of ocular myathenia without acetylcholine receptor antibodies * LRP4 antibodies * 3 cases reported these present in setting of ocular myasthenia ==== Other tests ==== * **Ice Pack Test** * Place a bag of ice on the ptotic lid for 1 minute * Immediately asses ptosis when ice removed * improvement is transient (<1 min) * sensitivity 80% if prominent ptosis present * **Tensilon test** * Edrophonium- inhibits acetylcholinesterase * transiently reverses muscle weakness * Sensitivity 85-95% * Electrophysiology * Repetitive nerve stimlation- reduction in amplitudes with repetitive stimulation * Single-fiber EMG- temoral variability of adjacent motor nerve fibers known as "jitter".