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September 1973

Cerebellar Dysfunction in Hand-Schuller-Christian Disease

Author Affiliations

Bethesda, Md

From the Reproduction Research Branch, National Institute of Child Health and Human Development (Drs. Braunstein and Kohler) and the Medical Neurology Branch, National Institute of Neurological Disease and Stroke (Dr. Whitaker), National Institutes of Health, Bethesda, Md. Dr. Braunstein is now with Cedars-Sinai Medical Center, Los Angeles, Dr. Whitaker is at the Veterans Administration Hospital, Minneapolis, and Dr. Kohler is at Baylor College of Medicine, Houston.

Arch Intern Med. 1973;132(3):387-390. doi:10.1001/archinte.1973.03650090067012

Diabetes insipidus and growth retardation are common manifestations of hypothalamic involvement in patients with Hand-Schuller-Christian disease (HSCD). Involvement of other areas of the central nervous system (CNS) has been reported infrequently. We have studied 13 patients with HSCD. Four of these patients exhibited signs of extrahypothalamic CNS disease, manifested primarily by impairment of cerebellar function. One patient showed bilateral dentate nuclear calcification, a radiologic sign not previously reported in this disease. Although histologic examination was not possible, the nature of the clinical and radiologic signs suggest that intracerebellar involvement in patients with HSCD is more common than previously appreciated.

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