Infranuclear Ophthalmoplegia

Abstract

Infranuclear ophthalmoplegia is characterized by global weakness of the extraocular and levator muscles. It has a broad differential diagnosis that varies depending on the tempo of onset. In this chapter, we begin by describing how to differentiate nuclear-infranuclear ophthalmoplegia from supranuclear ophthalmoplegia at the bedside. We next list the common causes of acute onset infranuclear ophthalmoplegia, which include Miller Fisher syndrome, Guillain-Barré syndrome, stroke, and ocular myasthenia. We then list the common causes of chronic progressive infranuclear ophthalmoplegia, which include mitochondrial disorders, oculopharyngeal muscular dystrophy, and myotonic dystrophy. We discuss the clinical features and diagnostic workup of chronic progressive external ophthalmoplegia due to mitochondrial disease. Lastly, we briefly discuss the management of ptosis and diplopia in the setting of chronic progressive external ophthalmoplegia.