TableĀ 3

Differentiating congenital myasthenic syndromes from autoimmune myasthenia gravis

Suggestive of autoimmune myasthenia gravisSuggestive of congenital myasthenic syndromes
Precipitous onset from normal
although mild previous abnormalities may have been missed
Onset at birth or infancy
Marked asymmetry of ptosis and fluctuation in ophthalmoplegia with diplopiaAnkle dorsiflexion weakness
common in congenital myasthenic syndromes but almost never a feature of myasthenia gravis
Response to immunotherapiesOphthalmoplegia not responsive to treatment
Spontaneous resolution especially in childrenFamily history or consanguinity in parents
Dysmorphic features due to early in utero weakness