A common presentation includes exophthalmos with strabismus of the affected eye, associated with a loss of visual acuity, visual field defects, optic atrophy, and a relative afferent pupillary defect. Not infrequently, an acquired nystagmus is the first clinical sign. The most common sign is optic atrophy on the affected side, and bilateral involvement is not uncommon. The mass grows slowly and advanced stages of enlargement are commonly associated with diencephalic disorders, including diabetes insipidus, adiposity, delayed sexual maturation, and somnolence.
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