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Urbach‑Wiethe, the Rare ECM1 Disorder That Abolishes External Fear, Comes Into Focus

New reporting explains that amygdala damage removes responses to outside threats, with CO2 still triggering panic through another circuit.

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El síndrome de Urbach-Wieth es una extraña enfermedad genética que puede causar daños a la amígdala, la región del cerebro que, entre otras cosas, desempeña un papel fundamental en el procesamiento del miedo.
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La enfermedad de Urbach-Wiethe es muy rara, pero puede identificarse con la incapacidad para sentir miedo. Imagen hecha con IA.

Overview

  • Researchers recount that Urbach‑Wiethe can eliminate outward fear by destroying the amygdala while leaving other emotions largely intact.
  • The condition is extremely rare, with about 400 diagnosed cases worldwide according to the U.S. National Institutes of Health.
  • Caused by loss‑of‑function mutations in the ECM1 gene, the disorder leads to abnormal deposits in skin, laryngeal tissue and, in roughly half of cases, the amygdala.
  • Studies of the patient known as SM show no fear to snakes, spiders or horror films and an inability to recognize fearful faces, alongside unusually short interpersonal distance and heightened vulnerability.
  • Management remains symptomatic with individualized care, including retinoids and CO2 laser for lesions; life expectancy can be normal, though risks include airway obstruction and neurologic complications.