Optic Atrophy in Childhood

Hereditary optic atrophy with onset in early childhood.

Optic Atrophy.

BASIC CONSIDERATIONS Optic atrophy is a misleading term which, embedded by tradition and usage, resists precise definition. It is used when there is excessive pallor of the optic disc and is thus a physical sign and not a diagnosis. Since there are many causes of optic pallor, the term is usually reserved for those cases in which there is associated visual defect, either of fields or acuity, in...

Dominantly inherited optic atrophy.

LEBER'S disease, with its sudden onset in adolescence or early adult life, its rapid course, and puzzling mode of inheritance, has overshadowed other forms of inherited optic atrophy. The early literature contained many cases of "atypical" Leber's disease, but the continued emphasis on the established form, even in the monumental review by Bell (1931), hampered the emergence of other forms as s...

Gastric atrophy in childhood.

Chronic gastritis and gastric atrophy are common in later adult life. Hebbel (1943) investigated the incidence of chronic gastritis at necropsy in patients dying from a variety of causes but whose history revealed no evidence of gastric symptoms. He found that gastric changes of any degree were rare below the age of 30 years and severe changes uncommon below the age of 50 years. Atrophic gastri...

Dominant optic atrophy

UNLABELLED DEFINITION OF THE DISEASE: Dominant Optic Atrophy (DOA) is a neuro-ophthalmic condition characterized by a bilateral degeneration of the optic nerves, causing insidious visual loss, typically starting during the first decade of life. The disease affects primary the retinal ganglion cells (RGC) and their axons forming the optic nerve, which transfer the visual information from the pho...