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4 A Syndrome

Sujoy Ghosh, Debmalya Sanyal, Sarmistha Mukherjee, Moutusi Raychowdhuri, Satinath Mukherjee, Subhankar Chowdhury

Am J Case Rep 2008; 9:329-332

ID: 865123

Background: Allgrove syndrome is a rare inherited familial disorder with variable age of onset and presentation. It is usually diagnosed in the first decade of life with features of primary adrenal insufficiency characterized by hypoglycaemic seizures, shock and pigmentation. Less frequently, a child may be present with dysphagia due to achalasia or with ocular symptoms of dry eyes. Manifestations in adult patients are rarely reported.
Case Report: We report a case of an 18-year-old boy with undiagnosed adrenal insufficiency and neurological problems associated with deficient tear secretion (alacrima) and asymptomatic achalasia.
Conclusions: This case highlights the spectrum of abnormalities associated with Allgrove syndrome and that the diagnosis of Allgrove syndrome should be considered in all patients presenting with any one of the classic symptoms. Other features, even if asymptomatic should be explored.

Keywords: adrenal insufficiency, achalasia, alacrima, adrenal myeloneuropathy

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