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Leber's optic atrophy
Also known as:
Leber’s acute optic neuropathy
Leber's atrophy
Leber's disease
Leber's hereditary optic atrophy
Leber’s syndrome I
Associated persons:
Theodor Karl Gustav von Leber
Description:
A rare hereditary form of optic atrophy that usually affects young males. Characterised by sudden bilateral cloudiness of vision, followed by scotoma, rapid deterioration of central vision, and occasional colour vision disorders. Associated with atrophy of the optic nerve fibres and the retinae. The pattern of transmission within a kindred are not in accordance with Mendelian principles and it is apparent that a complex mechanism of inheritance is in operation. The disease is never transmitted by affected men, but by affected women or, most frequently, by female carriers of the gene.
Bibliography:
- T. Leber:
Beiträge zur Kenntniss der atrophischen Veränderungen des Sehnerven nebst Bemerkungen über normale Structur des Nerven.
Archiv für Ophthalmologie, Berlin, 1868; 14: 164-176.
Über hereditäre und congenital-angelegte Sehnervenleiden.
[Albrecht von Grafes] Archiv für Ophthalmologie, Berlin, 1871, 17: 2 Abt, 249-291.
First description of his syndrome.
Die Krankheiten der Netzhaut.
In: Graefe-Saemisch Handbuch der gesammten Augenheilkunde, 2nd edition, page 1076. Leipzig, 1898-1915.
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