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Hypothalamic Atrophy with Progressive Hypopituitarism in an Adolescent Girl

Departments of Pediatrics and Radiological Sciences, UCLA Hospital and Clinics Los Angeles, California 90024

Address requests for reprints to: S. Anne Hendricks, M.D., Department of Pediatrics, UCLA School of Medicine, Los Angeles, California 90024.

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.

Received January 31, 1980.