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Dominant optic atrophy was first described clinically by Batten in 1896 and named Kjer’s optic neuropathy in 1959 after Danish ophthalmologist Poul Kjer, who studied 19 families with the disease. [3] Although dominant optic atrophy is the most common autosomally inherited optic neuropathy (i.e., disease of the optic nerves), it is often ...
Generally, diseases outlined within the ICD-10 codes H46-H48 within Chapter VII: Diseases of the eye, adnexa should be included in this category. Pages in category "Disorders of optic nerve and visual pathways"
Paul Kjer is a Danish ophthalmologist who studied a condition in nineteen families that was characterized by infantile optic atrophy along with a dominant inheritance mode. In 1959, the condition was named Kjer's optic neuropathy in his honor.
Optic neuropathy is damage to the optic nerve from any cause. The optic nerve is a bundle of millions of fibers in the retina that sends visual signals to the brain. Damage and death of these nerve cells, or neurons, leads to characteristic features of optic neuropathy.
HSP is marked by slowly progressive lower limb spasticity and weakness. HSP can be classified into pure and complicated forms, depending on whether additional clinical features are present besides spastic paraplegia, such as optic atrophy, ataxia, peripheral neuropathy, extrapyramidal deficits, and cognitive decline. [22]
Kjer may refer to: In people: Eva Kjer Hansen (born 1964), the Danish Minister for Food, Agriculture and Fisheries; Bodil Kjer (1917–2003), a Danish actress; Paul Kjer, Danish ophthalmologist who first described the condition now known as Kjer's optic neuropathy; In other uses: Kjer's optic neuropathy, dominant optic atrophy
Behr syndrome is characterized by the association of early-onset optic atrophy with spinocerebellar degeneration resulting in ataxia, pyramidal signs, peripheral neuropathy and developmental delay. [1] [2] Although it is an autosomal recessive disorder, heterozygotes may still manifest much attenuated symptoms. [3]
Non-arteritic anterior ischemic optic neuropathy (NAION) is a medical condition characterized by loss of vision caused by damage to the optic nerve as a result of ischemia, or insufficient blood supply. The key symptom of NAION is optic disc swelling, which typically resolves within 2 months, but often leads to optic atrophy. The likelihood of ...