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April 1969

Localized Hypothalamic Histiocytosis X: Report of a Case

Author Affiliations

Stockton, Calif, and San Francisco; San Francisco
From the Department of Medical Education, San Joaquin General Hospital, Stockton, Calif, and the Department of Medicine, University of California Medical Center, San Francisco (Dr. Bernard), and the Department of Neuropathology, Pacific Medical Center, San Francisco (Dr. Aguilar).

Arch Neurol. 1969;20(4):368-372. doi:10.1001/archneur.1969.00480100044006

THE rarity of histiocytosis X and related disorders has made reporting and discussion of these syndromes of interest and importance to those concerned with such diseases. In the present case, localized hypothalamic histiocytosis X was associated with thyroiditis and cirrhosis of the liver. The patient's early death by pulmonary embolization made possible the extensive study of this example of a localized reticuloendotheliosis.

Report of a Case  A 20-year-old white man enjoyed normal health, growth, and development until 1960 when at age 13 he developed polyuria and polydipsia. Diabetes insipidus was diagnosed, and after an investigation, including pneumoencephalography, failed to reveal the etiology, he was treated with posterior pituitary extract by nasal insufflation. During the following year, he had a 18.2 kg (40-lb) weight gain and episodes of "dizziness," which occurred after sudden changes in body position. There was no family history of a similar disorder.He was seen at

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