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


Any disease affecting neurons.



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.


References in periodicals archive ?
Neuropathology of white matter disease in Leber's hereditary optic neuropathy," Brain, vol.
2001) alpha- Tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation.
This report provides comprehensive information on the therapeutic development for Leber's Hereditary Optic Neuropathy (LHON) (Leber optic atrophy), complete with comparative analysis at various stages, therapeutics assessment by drug target, mechanism of action (MoA), route of administration (RoA) and molecule type, along with latest updates, and featured news and press releases.
Late onset of Leber's hereditary optic neuropathy in HIV infection.
Santhera's lead product Raxone is authorized in the European Union, Norway, Iceland and Liechtenstein for the treatment of Leber's hereditary optic neuropathy (LHON).
Tylock has faced many challenges since being diagnosed in eighth grade with Leber's Hereditary Optic Neuropathy, a rare genetic disorder that causes loss of vision.
The Committee's opinion on Santhera's Marketing Authorization Application (MAA) for Raxone in the treatment of Leber's Hereditary Optic Neuropathy (LHON) is now expected to be obtained in early 2013.
Acquired disorders of the optic nerve include anterior ischaemic optic neuropathy (AION), optic neuritis, papilloedema (swelling of the optic nerve head secondary to raised intracranial pressure), toxic optic neuropathies, tumours of the optic nerve, and genetic optic neuropathies (such as Leber's hereditary optic neuropathy and autosomal dominant optic atrophy), and, of course, glaucoma.
Raxone was approved in September 2015 for the treatment of visual impairment in adolescent and adult patients with Leber's hereditary optic neuropathy (LHON) in all EU member states, Norway, Iceland and Liechtenstein.
Stewart, 23, has Leber's hereditary optic neuropathy (LHON), a degeneration of cells in the optic nerve, which relays visual information from the eyes to the brain.

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