Question 979
{"accessible":false,"alternatives":[{"id":4906,"text":"asymmetric cortical and subcortical gyriform calcifications"},{"id":4907,"text":"asymmetric scattered intraparenchymal calcifications"},{"id":4908,"text":"intracranial calcifications are not a feature of this disease"},{"id":4909,"text":"symmetric calcifications in the amygdalae "},{"id":4910,"text":"symmetric calcifications in the basal ganglia and thalamus"}],"archived":false,"correctAlternativeId":4909,"explanation":"\u003cp\u003e\u003ca title=\"Urbach-Wiethe disease\" class=\"internal\" href=\"/articles/urbach-wiethe-disease\"\u003eUrbach-Wiethe disease\u003c/a\u003e\u0026nbsp;is a rare autosomal recessive genodermatosis that primarily affects the skin, upper respiratory tract, and central nervous system (CNS). In the CNS, it manifests clinically with neuropsychiatric symptoms relating to amygdala destruction (such as absence of fear and memory loss), and \u003ca title=\"Temporal lobe epilepsy\" class=\"internal\" href=\"/articles/temporal-lobe-epilepsy\"\u003etemporal lobe epilepsy\u003c/a\u003e. Non-contrast CT brain classically demonstrates bilateral symmetric intracranial calcifications affecting the amygdalae. These calcifications are present in 50-75% of patients over the age of 10 with the disease.\u003c/p\u003e\u003cp\u003eThe other options in this question describe other aetiologies of \u003ca title=\"Multiple intracranial calcifications\" class=\"internal\" href=\"/articles/multiple-intracranial-calcifications\"\u003emultiple intracranial calcifications\u003c/a\u003e, such as \u003ca title=\"Sturge-Weber syndrome\" class=\"internal\" href=\"/articles/sturge-weber-syndrome-1\"\u003eSturge-Weber syndrome\u003c/a\u003e, \u003ca title=\"Congenital TORCH infections\" class=\"internal\" href=\"/articles/congenital-infections-mnemonic\"\u003eTORCH infections\u003c/a\u003e, and \u003ca title=\"Fahr syndrome\" class=\"internal\" href=\"/articles/fahr-syndrome-1\"\u003eFahr syndrome\u003c/a\u003e.\u003c/p\u003e","id":979,"imageUrl":null,"imageAttribution":null,"imageAttributionCaseInfo":null,"firstQuestionPath":"/questions/979","nextQuestionPath":null,"relatedArticles":[{"id":57147,"title":"Urbach-Wiethe disease","link":"/articles/urbach-wiethe-disease?lang=gb"}],"alsoUsedIn":[],"stem":"\u003cp\u003eWhich of the following best describes the pattern of intracranial calcification seen in\u0026nbsp;Urbach-Wiethe disease?\u003c/p\u003e","menuLinks":[{"text":"Report problem with question","url":"https://docs.google.com/forms/d/e/1FAIpQLSfO3soWYhOjJ7yErSysyCe5V4A1CqW7WK3rDA7MtAkecMGqNw/viewform?entry.1624461248\u0026entry.553583435=https://radiopaedia.org/questions/979"}],"attemptsPercentages":[{"alternativeId":"4910","percentage":11},{"alternativeId":"4907","percentage":8},{"alternativeId":"4909","percentage":61},{"alternativeId":"4908","percentage":8},{"alternativeId":"4906","percentage":11}],"promptToLogin":false,"questionManager":false,"articleId":"multiple-intracranial-calcifications"}