Leber's congenital amaurosis

**Leber's congenital amaurosis**
*Classifications and external resources*
ICD-10	H53.0
ICD-9	362.76

Leber's congenital amaurosis is a rare inherited eye disease that appears at birth or in the first few months of life, typically characterized by nystagmus, sluggish or no pupillary responses, and severe vision loss or blindness. It is an autosomal recessive disorder thought to be caused by abnormal development of photoreceptors.

Leber's congenital amaurosis is sometimes confused with Leber's hereditary optic neuropathy, which is a different disease also first described by Theodore Leber in the 19th century.

Project 3000, a foundation started by Chicago Cubs first baseman Derrek Lee and Boston Celtics owner Wyc Grousbeck, works to have the approximately 3,000 people in the United States with the disease genetically tested.