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S.M., sometimes referred to as SM-046, is an American woman with a peculiar type of brain damage that physiologically reduces her ability to feel fear.First described by scientists in 1994, [1] she has had exclusive and complete bilateral amygdala destruction since late childhood as a consequence of Urbach–Wiethe disease.
Urbach–Wiethe disease is inherited in an autosomal recessive manner. Urbach–Wiethe disease is a very rare recessive genetic disorder, with approximately 400 reported cases since its discovery. [1] [2] [3] It was first officially reported in 1929 by Erich Urbach and Camillo Wiethe, [4] [5] although cases may be recognized dating back as ...
Urbach-Wiethe disease (UWD) is a rare genetic disorder characterized by early damage to the basolateral amygdala. Perhaps more than 50% of the world's population of UWD live in South Africa, and together with his doctoral student, Helena Thornton, Stein initiated work on the neuropsychology of UWD.
In fear conditioning, the main circuits that are involved are the sensory areas that process the conditioned and unconditioned stimuli, certain regions of the amygdala that undergo plasticity (or long-term potentiation) during learning, and the regions that bear an effect on the expression of specific conditioned responses.
Lipoid proteinosis, also known as Urbach–Wiethe disease This page was last edited on 29 December 2019, at 06:13 (UTC). Text is available under the Creative Commons ...
Other types of damage include Klüver–Bucy syndrome and Urbach–Wiethe disease. In Klüver–Bucy syndrome, a temporal lobectomy, or removal of the temporal lobes, results in changes involving fear and aggression. Specifically, the removal of these lobes results in decreased fear, confirming its role in fear recognition and response.
The English suffixes -phobia, -phobic, -phobe (from Greek φόβος phobos, "fear") occur in technical usage in psychiatry to construct words that describe irrational, abnormal, unwarranted, persistent, or disabling fear as a mental disorder (e.g. agoraphobia), in chemistry to describe chemical aversions (e.g. hydrophobic), in biology to describe organisms that dislike certain conditions (e.g ...
Hypochondrogenesis is one of the most severe conditions in a spectrum of disorders caused by mutations in the COL2A1 gene.The protein made by this gene forms type II collagen, a molecule found mostly in cartilage and in the clear gel that fills the eyeball (the vitreous).