Neuropathy

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

neuropathy

[nu̇′räp·ə·thē]
(medicine)
Any disease affecting neurons.

Neuropathy

 

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.

REFERENCES

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.

L. M. SHMAONOVA

References in periodicals archive ?
Leber's hereditary optic neuropathy (LHON) is a heritable genetic disease causing blindness.
The report provides a snapshot of the global therapeutic landscape of Leber's Hereditary Optic Neuropathy (LHON) (Leber optic atrophy)
Occurrence of a multiple sclerosis-like illness in women who have a Leber's hereditary optic neuropathy mitochondrial DNA mutation," Brain, vol.
Pedigree analysis in Leber hereditary optic neuropathy families with a pathogenic mtDNA mutation.
2003) The epidemiology of Leber hereditary optic neuropathy in the North East of England.
Santhera develops Catena/Raxone as treatment for patients with Leber's Hereditary Optic Neuropathy, Duchenne Muscular Dystrophy and Primary Progressive Multiple Sclerosis.
They include muscle-wasting mitochondrial myopathies, Leber's Hereditary Optic Neuropathy which leads to blindness, nerve illnesses similar to multiple sclerosis, and conditions that damage organs, including the heart.
Stuart Carroll, who, with his uncle Les McGuinness of Canley, has Lebers hereditary optic neuropathy which has left him with 1/60th of his eyesight, said: "Treatment for our condition is only in its development stages, so it could be years before we can have our operations.
CONTEXT: Leber hereditary optic neuropathy (LHON) is a maternally inherited loss of central vision related to pathogenic mutations in the mitochondrial genome, which are a necessary but not sufficient condition to develop the disease.
16) In that same year, specific human diseases (Leber hereditary optic neuropathy [LHON], mitochondrial myopathy, and Kearns-Sayre syndrome) were first linked to mtDNA mutation.
The first two diseases shown to have mutations in mitochondrial genes included Leber's hereditary optic neuropathy (LHON), a sudden-onset, young-adult blindness (Wallace et al.
Since the discovery that Leber hereditary optic neuropathy (LHON) results from mutations in mitochondrial DNA (mtDNA), considerable attention has been focused on this alternative genome and on development of the scientific tools needed to study this remarkable genetic pathway (1, 2).

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