Hand-Schüller-Christian Disease in a Girl with Cerebellar Ataxia: Case Report

A case of Hand-Schuller-Christian disease with involvement of the central nervous system is reported. The patient was a 13-year-old girl, admitted with cerebellar ataxia, polydipsia and polyuria, exophthalmos and visual impairment. Computerized tomography (CT) scan with contrast enhancement revealed a high-density mass at the left cerebellopontine angle, brachium pontis, cerebellar hemisphere, ambient cistern, superior cerebellar cistern, quadrigeminal cistern and posterior third ventricle. High-density masses along the bilateral optic nerves were also revealed. Left vertebral angiography showed no tumor stain. Suboccipital craniectomy was performed after the Stein's approach, and the tumor which adhered strongly to the cerebellum was partially removed. The tumor was yellowish in color, fibrous, and elastic hard. Histological examination showed histiocytosis X. It was supposed from the clinical course that the disease began in the hypothalamic region and spread through the subarachnoid and Virchow-Robin spaces to the optic nerve sheath and posterior cranial fossa. Only twenty-five cases of Hand Schüller-Christian disease with cerebellar signs or cerebellar involvement have been reported in the literature. CT findings of Hand Schuller-Christian disease with cerebellar involvement has not been reported.