Multiple Endocrine Adenomatosis Type I

Abstract

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.¹Pancreatic involvement may be signaled by the presence of elevated levels of pancreatic polypeptide (PP) in the serum of both symptomatic and asymptomatic individuals.²We 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. <h3>Report of a Case</h3> 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,