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Related to hereditary optic neuropathy: Leber hereditary optic neuropathy, LHON


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 ?
Santhera's lead product Raxone is authorized in the European Union for the treatment of Leber's Hereditary Optic Neuropathy (LHON).
Santhera currently has four clinical-stage development programs, three of which are investigating its lead compound, SNT-MC17 (INN: idebenone), in the treatment of Friedreich's Ataxia (FRDA), Duchenne Muscular Dystrophy (DMD) and Leber's Hereditary Optic Neuropathy (LHON).
These findings are consistent with a diagnosis of Leber hereditary optic neuropathy (LHON), a maternally inherited loss of central vision that preferentially affects young men (Man et al.
Deficient NADH:coenzyme Q oxidoreductase in Leber's hereditary optic neuropathy.
It also reviews key players involved in the therapeutic development for Leber's Hereditary Optic Neuropathy (LHON) (Leber optic atrophy).
Late onset of Leber's hereditary optic neuropathy in HIV infection.
Phylogenic analysis of the mitochondrial genomes from Leber hereditary optic neuropathy pedigree.
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 Telegraph has told previously how Stuart Carroll, aged 26, of Jeliff Street, Tile Hill, sees the world as a blur because he has Lebers hereditary optic neuropathy.
The most common point mutations are A3243G, accounting for 80% of patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS); A8344G, which underlies myoclonic epilepsy, ragged red fibers (MERRF); T8993G/C, leading to neuropathy, ataxia, retinitis pigmentosa (NARP); and G11778A, found in >50% of patients with Leber hereditary optic neuropathy (LHON).
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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