糖尿病と下垂体機能障害を合併し,高度の痴呆を呈したKlinefelter症候群の1例

Abstract

We report a case of Klinefelter's syndrome associated with diabetes mellitus, pituitary gland dysfunction, and severe dementia. The patient was a 70-year-old man who had been under medical treatment for diabetes mellitus for twenty years. In March 1998, he developed cerebral infarction and symptoms of dementia. Physical exami- nation revealed projection of both forehead and lower jaw, hypoplasia of the genitals, and blood flow insufficiency beyond the bilateral popliteal arteries. Chromosomal analysis showed a 46, XY/46, XX/47, XXY mosaic pattern. HbA1c rose to 8.4% and ΔIRI/ΔBS was 0.02 ng/ml, indicating decreased insulin secretion. A pituitary gland dysfunction compli- cated the clinical scenario in this patient, the secretion of growth hormone, adrenocor- ticotropic hormone, prolactin, and cortisol was increased, and that of luteinizing hormone, follicle-stimulating hormone, and testosterone was decreased. Magnetic resonance images of the head showed a normal-appearing pituitary gland and cerebral infarction at both the middle and posterior cerebral artery. Therefore, our patient was considered to have vascular dementia. In this report, we discuss the relationship between Klinefelter's syndrome and either diabetes mellitus or pituitary gland dysfunction.