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Related to Leber's hereditary optic neuropathy: Leber's congenital amaurosis


Any disease affecting neurons.
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.
The following article is from The Great Soviet Encyclopedia (1979). It might be outdated or ideologically biased.



a functional weakness of the nervous system accompanied by a decrease in the absolute threshold (also called the stimulus, or sensitivity, threshold); the condition is frequently congenital.

Neuropathy arises as a disturbance in the function of the autonomic nervous system owing to deleterious influences on the fetus as a whole (for example, infection in the pregnant mother, trauma, or poisoning) or on the individual embryonic cells (as occurs when the parents suffer from alcoholism). Family circumstances, upbringing, and illnesses suffered by the infant play a role in the pathogenesis of neuropathy. The symptoms usually emerge in childhood or adolescence. When neuropathy occurs in early childhood, disturbances of sleep and appetite are most characteristic; the infant has difficulty in falling asleep, awakes easily, suckles poorly, and frequently spits up. Later, vomiting occurs, and either diarrhea or constipation develop.

From the preschool years on, increased general excitability and the rapid onset of fatigue and exhaustion are observed, in addition to continued disturbances of sleep and appetite. Children who suffer from neuropathy blush easily in response to irritation. Motor hyperactivity, nervous tics, and stammering are frequent. In adolescence, autonomic-vascular instability becomes more acute, as evidenced by frequently occurring nervous palpitation, abrupt shifts in blood pressure, headaches, dizzy spells, and fainting spells. The prognosis is favorable, with the neuropathic symptoms usually disappearing with age.


Simson, T. Nevropatii, psikhopatii i reaktivnye sostoianiia mladencheskogo vozrasta. Moscow-Leningrad, 1929.
Sukhareva, G. E. Klinicheskie lektsii po psikhiatrii detskogo vozrasta, vol. 2. Moscow, 1959.


The Great Soviet Encyclopedia, 3rd Edition (1970-1979). © 2010 The Gale Group, Inc. All rights reserved.
References in periodicals archive ?
Ma et al., "Evaluation of Leber's hereditary optic neuropathy patients prior to a gene therapy clinical trial," Medicine (Baltimore), vol.
Wahlstrom, "Leber's hereditary optic neuropathy and complex I deficiency in muscle," Annals of Neurology, vol.
(2003) Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose medium.
A mitochondrial DNA mutation as a cause of Leber's hereditary optic neuropathy. N Engl J Med.
* The report reviews key players involved in the therapeutics development for Leber's Hereditary Optic Neuropathy (LHON) (Leber optic atrophy) and enlists all their major and minor projects
Leber's hereditary optic neuropathy: The mitochondrial connection revisited.
Mutation-specific PCR: a rapid and inexpensive diagnostic method as exemplified by mitochondrial DNA analysis in Leber's hereditary optic neuropathy. DNA Cell Biol 1993;12:54952.
Wallace and his colleagues discovered that people suffering Leber's hereditary optic neuropathy (LHON) have a defect in one mitochondrial gene that codes for a protein involved in the first step of ATP production.
Santhera out-licensed ex-North American rights to its first approved product, Raxone (idebenone), for the treatment of Leber's hereditary optic neuropathy (LHON) to Chiesi Group.
In conjunction, BridgeBio has formed the subsidiary company, called Fortify Therapeutics, to further develop this chemistry for local treatment of Leber's Hereditary Optic Neuropathy (LHON), with an initial financial commitment of USD20m.
The MAA was submitted as a Type II variation of the company's existing marketing authorization for Raxone for the treatment of visual impairment in patients with Leber's hereditary optic neuropathy (LHON).
Santhera's lead product Raxone[R] is authorized in the European Union for the treatment of Leber's hereditary optic neuropathy (LHON).

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