“Crying without tears” as an early diagnostic sign-post of triple A (Allgrove) syndrome: two case reports
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چکیده
منابع مشابه
“Crying without tears” as an early diagnostic sign-post of triple A (Allgrove) syndrome: two case reports
BACKGROUND Triple A syndrome (or Allgrove syndrome) is a rare autosomal recessive disorder characterized by alacrima, achalasia, adrenal insufficiency and autonomic/neurological abnormalities. The majority of cases are caused by mutations in the AAAS gene located on chromosome 12q13. However, the clinical picture as well as genetic testing may be complex since symptomatology is variable and mut...
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Backgrounds: Crying seizures is a rare type of seizure. Some scholars believe that crying seizures are mainly associated with lesions in the anterior temporal lobe, typically the non-dominant hemisphere, which has close connections with negative emotions. However, the role of amygdaloid lesions in crying seizures is not fully understood. Methods: By a patient with crying seizures and lesions in...
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Triple A (Allgrove) syndrome was first described by Allgrove in 1978 in two pairs of siblings. Since then, about 100 cases have been reported, all of them displaying an autosomal recessive pattern of inheritance. Clinical picture is characterized by achalasia, alacrimia and ACTH-resistant adrenal failure. A progressive neurological syndrome including central, peripheral and autonomic nervous sy...
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ژورنال
عنوان ژورنال: BMC Pediatrics
سال: 2018
ISSN: 1471-2431
DOI: 10.1186/s12887-017-0973-y