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Glucocorticoid Insufficiency, Achalasia, Alacrima with Autonomic and Motor Neuropathy

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▸Requests for reprints should be addressed to Dr. B. G. Stuckey; Department of Endocrinology and Diabetes, Sir Charles Gairdner Hospital, Verdun Street; Nedlands, WA 6009, Australia.

Sir Charles Gairdner Hospital; Nedlands, Australia

Ann Intern Med. 1987;106(1):62-64. doi:10.7326/0003-4819-106-1-62
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We report the case of a patient with adrenal disease with achalasia and alacrima, which appear to be part of a widespread defect in adrenal and nervous system function.

A 21-year-old white woman at age 6 had had hypoglycaemia, hyperpigmentation, low plasma cortisol level (30 nmol/L; normal, 200 to 600), and a lack of cortisol response to cosyntropin. Alacrima had been noted since infancy but there were no other abnormal neurologic features. A roentgenogram of the chest at age 9 showed bilateral pulmonary opacities presumed due to aspiration; these have persisted. Nocturnal cough, vomiting of undigested food, and recurrent chest


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