Histiocytosis-X: causes, symptoms, diagnosis, treatment

Histiocytosis-X is a rare granulomatous disease of unknown etiology. Its clinical variety is Hand-Schüller-Christian syndrome, or disease. In 50% of cases of this disease, the clinical picture is dominated by symptoms of diabetes insipidus, which may manifest itself monosymptomically for several years. Histiocytosis-X should be excluded primarily in the case of diabetes insipidus in early childhood. Destruction of the hypothalamus and the upper part of the pituitary stalk as a result of neoplastic or granulomatous infiltration can cause the development of the clinical picture of diabetes insipidus. Destruction of the posterior pituitary gland is an extremely rare cause of diabetes insipidus. This is explained by the fact that some axons of the supraoptic-pituitary tract terminate in blood vessels located in the upper part of the stalk or in the median eminence of the hypothalamus. Neoplastic damage to the hypothalamus can be either a primary tumor or a metastatic process.

CNS involvement by sarcoidosis is a relatively rare disease. Diabetes insipidus in sarcoidosis is often combined with galactorrhea due to hyperprolactinemia and partial or complete insufficiency of the anterior pituitary gland. X-ray examination of the skull, fundus and visual field analysis, lumbar puncture, and computed tomography are necessary to exclude a neoplastic or granulomatous process.

Diagnosis of histiocytosis-X is facilitated by systemic manifestations of the disease, neurological concomitant symptoms, and characteristic changes in the cerebrospinal fluid.

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