Hypothalamic atrophy with progressive hypopituitarism in an adolescent girl

The present report describes a 19-yr-old female with progressive hypopituitarism and diabetes insipidus. Pneumoencephalography demonstrated gross atrophy of the hypothalamus and a small pituitary gland. In the face of documented hypothyroidism and hypogonadism, basal pituitary trophic hormones were consistently detectable and responded briskly to releasing factor administration. This combination of an atrophic lesion of the hypothalamus with gradually evolving hypopituitarism but detectable and stimulable anterior pituitary hormones appears to represent a unique form of hypothalamic failure.