Histiocytosis-X: causes, symptoms, diagnosis, treatment

Histiocytosis-X refers to a fairly rare granulomatous disease of an unknown etiology. Its clinical variety is a syndrome, or a disease, Hyunda-Schuiller-Christen. In this disease, in 50% of cases in the clinical picture the symptoms of diabetes insipidus are leading, which can manifest monosymptomatically within a few years. Histiocytosis-X should be excluded first of all in the picture of diabetes insipidus in early childhood. The 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 a clinical picture of diabetes insipidus. The destruction of the posterior pituitary gland is an extremely rare cause of diabetes insipidus. This is due to the fact that some axons of the supraoptic-hypophyseal tract terminate on blood vessels located in the upper part of the stem or in the middle elevation of the hypothalamus. Neoplastic damage to the hypothalamus is possible both as a primary tumor and as a metastatic process.

CNS lesions are a relatively rare disease in sarcoidosis. Non-diabetes mellitus in sarcoidosis is often combined with galactorrhea due to hyperprolactinaemia and with partial or complete failure of the anterior lobe of the pituitary gland. To exclude the neoplastic or granulomatous process, an x-ray examination of the skull, analysis of the fundus and visual fields, lumbar puncture, computed tomography are necessary.

Diagnosis of histiocytosis-X is facilitated by systemic manifestations of diseases, neurological concomitant symptomatology, characteristic changes in cerebrospinal fluid.

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