DOI: 10.36347/sjmcr.2015.v03i05.017

Pages: 407-410

Allgrove syndrome or Triple A syndrome is a rare autosomal recessive disorder. The mutation affects the AAAS gene localized on chromosome 12. It combines alacrima, achalasia and adrenal insufficiency. Neurological signs can be associated. It usually occurs during the first decade of life. Treatment is symptomatic based on hormone replacement, artificial tears and surgery. We report two cases of a brother and a sister, whose parents are first cousins and have the full form of this disease with neurological signs. We will study physiopathological, clinical, genetic and therapeutic aspects of this syndrome.