[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-444803-105":3,"detail-sidebar-cat-0-en-105":81,"doc-detail-444803-en":131},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":74,"head_meta":76,"extra_data":78,"updated_unix":80},105,"en","allgrove-syndrome-in-adults-a-case-of-an-atypical-presentation","Allgrove Syndrome in Adults - A Case of an Atypical Presentation","","Allgrove syndrome, or “Triple A” syndrome, is a rare autosomal recessive disorder defined by achalasia, alacrima, and ACTH-resistant adrenal insufficiency. Adult cases are uncommon and frequently missed due to atypical or slowly progressive features. A 29-year-old Moroccan woman presented with chronic dysphagia and multisystem complaints, and diagnosis was delayed for more than a decade. Endoscopic, radiologic, and manometric studies confirmed type 1 achalasia, adrenal insufficiency, and alacrima, and treatment with corticosteroids plus pneumatic dilation achieved a favorable outcome.",{"@graph":14,"@context":73},[15,34,56],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/healthcare/","Healthcare",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/allgrove-syndrome-in-adults-a-case-of-an-atypical-presentation/444803/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/allgrove-syndrome-in-adults-a-case-of-an-atypical-presentation/444803.png","ImageObject",300,407,{"name":42,"@type":43},"Alexandar","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-11-06","2026-09-29",true,{"@type":52,"interactionType":53,"userInteractionCount":55},"InteractionCounter",{"@type":54},"ViewAction",12,{"@type":57,"mainEntity":58},"FAQPage",[59,65,69],{"name":60,"@type":61,"acceptedAnswer":62},"What defines Allgrove (“Triple A”) syndrome?","Question",{"text":63,"@type":64},"It is characterized by a triad of achalasia, alacrima, and ACTH-resistant adrenal insufficiency.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"Why was the diagnosis delayed in this adult case?",{"text":68,"@type":64},"The patient had a long history of unexplored, slowly progressive dysphagia and adult presentations with atypical features are under-recognized.",{"name":70,"@type":61,"acceptedAnswer":71},"How was the diagnosis confirmed and what treatment was used?",{"text":72,"@type":64},"The diagnosis was confirmed with clinical, endoscopic, radiologic, and manometric findings showing achalasia and adrenal insufficiency, along with alacrima. Treatment included corticosteroids and pneumatic dilation, with a favorable outcome.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},444803,1791153628,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,101,106,110,115,120,123,127],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":89,"show_sort_weight":90,"slug":91},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":93,"show_sort_weight":94,"slug":95},"Exam",70,"exam",{"id":97,"doc_module":4,"doc_module_name":25,"category_name":98,"show_sort_weight":99,"slug":100},5,"Comic",60,"comic",{"id":102,"doc_module":4,"doc_module_name":25,"category_name":103,"show_sort_weight":104,"slug":105},6,"Technology",50,"technology",{"id":107,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":108,"slug":109},7,40,"healthcare",{"id":111,"doc_module":4,"doc_module_name":25,"category_name":112,"show_sort_weight":113,"slug":114},8,"Research & Report",30,"research-report",{"id":116,"doc_module":4,"doc_module_name":25,"category_name":117,"show_sort_weight":118,"slug":119},9,"Religion & Spirituality",20,"religion-spirituality",{"id":118,"doc_module":4,"doc_module_name":25,"category_name":121,"show_sort_weight":118,"slug":122},"World Cup","world-cup",{"id":124,"doc_module":4,"doc_module_name":25,"category_name":125,"show_sort_weight":124,"slug":126},10,"Lifestyle","lifestyle",{"id":128,"doc_module":4,"doc_module_name":25,"category_name":129,"show_sort_weight":97,"slug":130},19,"General","general",{"code":4,"msg":82,"data":132},{"doc_id":79,"user_id":133,"nickname":42,"user_avatar":134,"doc_module":4,"category_id":107,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":55,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":102,"language":140,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":12,"update_tm":144,"read_time":145,"preview_page_count":4},962090768658,"https://ap-avatar.wpscdn.com/davatar_6f874abed73319feea01a86fa6f0fab8","Review began 11/15/2025  \nReview ended 12/03/2025  \nPublished 12/07/2025  \n© Copyright 2025  \nMaizi et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited.  \nDOI: 10.7759/cureus.98638  \nOpen Access Case Report  \nAllgrove Syndrome in Adults: A Case of an Atypical Presentation  \nMeryem Maizi 1, 2 , Fatima Belabbes 1, 2, Hanane Delsa 1, 2, Saloua Elamari 3, 2, Imane Ben Elbarhdadi 1, 2  \n1. Gastroenterology and Hepatology, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR 2. Research Unit, Mohammed VI Center for Research and Innovation, Rabat, MAR 3. Endocrinology, Diabetology, Metabolic Disease, and Nutrition, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR  \nCorresponding author: Meryem Maizi, mmaizi@um6ss.ma  \nAbstract  \nAllgrove syndrome, or \"Triple A\" syndrome, is a rare autosomal recessive disorder characterized by achalasia, alacrima, and adrenal insufficiency. Typically diagnosed in childhood or adolescence, its adult presentation remains under-recognized and challenging. We report the case of a 29-year-old Moroccan woman with chronic dysphagia, who was ultimately diagnosed with Allgrove syndrome following the discovery of type 1 achalasia, primary adrenal insufficiency, and alacrima. The diagnosis was delayed, and symptoms had persisted for over a decade before appropriate evaluation. Clinical, endoscopic, radiologic, and manometric findings confirmed the diagnosis. Therapeutic management included corticosteroids and pneumatic dilation, with a favorable outcome. This case highlights the importance of considering Allgrove syndrome in adults with unexplained dysphagia and systemic symptoms.  \nCategories: Neurology, Endocrinology/Diabetes/Metabolism, Gastroenterology  \nKeywords: achalasia, adrenal insufficiency, adult, alacrima, allgrove syndrome, delayed diagnosis  \nIntroduction  \nAllgrove syndrome, also known as “Triple A” syndrome, is a rare autosomal recessive disorder characterized by a triad of achalasia, alacrima, and adrenocorticotropic hormone (ACTH)-resistant adrenal insufficiency [1,2] . Although most cases are identified during childhood or adolescence, adult presentations are rare and often manifest with atypical features, notably neurological involvement alongside the classic symptoms [3] .  \nWe report the case of a 29-year-old woman with Allgrove syndrome in whom the diagnosis was significantly delayed because of the insidious progression of long-standing, unexplored dysphagia. This case underscores the diagnostic challenges associated with adult-onset forms of the condition.  \nCase Presentation  \nA 29-year-old Moroccan woman, born to consanguineous parents (first-degree cousins), presented with a 13-year history of progressively worsening dysphagia. Her childhood history was notable for a foreign body impaction (a coin) that required endoscopic removal, along with chronic anemia of unknown etiology.  \nIntermittent dysphagia began at the age of 16 and gradually progressed over the years.  \nShe reported postprandial vomiting, nocturnal gastroesophageal reflux, unintentional weight loss, chronic fatigue, dry cough, blurred vision, and joint pain. Neurological symptoms included rhinolalia (nasal speech), orthostatic lightheadedness, and episodic dysarthria. Family members had observed generalized skin hyperpigmentation and melanonychia over the past decade.  \nOn physical examination, blood pressure measurements were within the normal range, and her body mass index (BMI) was 16.9 kg/m². She appeared clinically dehydrated and exhibited diffuse cutaneous and mucosal hyperpigmentation, particularly in the skin folds (Figure 1) .  \nHow to cite this article  \nMaizi M , Belabbes F , Delsa H , et","cbCaivhDXL8jgwBU","https://ap.wps.com/l/cbCaivhDXL8jgwBU","pdf",2490204,"English","# Abstract\n# Introduction\n# Case Presentation\n## History and Symptoms\n## Physical Examination and Neurological Findings\n## Ophthalmologic and Laboratory Findings","[{\"question\":\"What defines Allgrove (“Triple A”) syndrome?\",\"answer\":\"It is characterized by a triad of achalasia, alacrima, and ACTH-resistant adrenal insufficiency.\"},{\"question\":\"Why was the diagnosis delayed in this adult case?\",\"answer\":\"The patient had a long history of unexplored, slowly progressive dysphagia and adult presentations with atypical features are under-recognized.\"},{\"question\":\"How was the diagnosis confirmed and what treatment was used?\",\"answer\":\"The diagnosis was confirmed with clinical, endoscopic, radiologic, and manometric findings showing achalasia and adrenal insufficiency, along with alacrima. Treatment included corticosteroids and pneumatic dilation, with a favorable outcome.\"}]","Allgrove Syndrome in Adults - A Case of an Atypical Presentation | PDF",1790709315,15]