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February 5, 1982

Multiple Endocrine Adenomatosis Type I: Occurrence in an Octogenarian With High Levels of Circulating Pancreatic Polypeptide

JAMA. 1982;247(5):665-666. doi:10.1001/jama.1982.03320300069029

MULTIPLE endocrine adenomatosis (MEA) is a multisystem familial disorder involving hypersecretion of polypeptide hormones by adenomatous or hyperplastic tissues. In well-documented cases, uniform involvement of the parathyroid glands, pituitary gland, and pancreas has been found in type I disease.1 Pancreatic involvement may be signaled by the presence of elevated levels of pancreatic polypeptide (PP) in the serum of both symptomatic and asymptomatic individuals.2 We describe a patient in whom MEA developed in the ninth decade with hypoglycemia and in whom PP levels were greatly elevated. A further unusual finding was the presence of generalized islet hyperplasia.

Report of a Case  An 81-year-old woman was well until the onset of unsteadiness of gait one month before admission, when nausea, vomiting, and dizziness appeared. Nystagmus and truncal ataxia were noted on examination, and a diagnosis of probable brainstem infarct was made. The patient was admitted to the hospital for convalescence,