What is CNS LCH?

Three major clinical groups of CNS LCH can be distinguished, but the occurrence of more than one type of lesion in the same patient is common. The classification of the lesions according to their MRI appearance is given in Table 1. Hypothalamic pituitary involvement (type IV) is seen in about 10 percent of LCH patients.

The incidence ranges from 5 to 50 percent in the different reports, according to the various stratification systems or treatment approaches. Polyuria and polydipsia as symptoms of posterior pituitary dysfunction are the most frequent finding in about 90 percent of patients with morphological changes in this region. However, other endocrine deficiencies like growth failure, precocious or delayed puberty, amenorrhea, hypothyroidism, or hypocortisolism resulting from anterior pituitary failure, are seen in about 50 percent of the DI patients.

In rare cases of hypothalamic tumors, disturbances of appetite and social behavior with binge or rage attacks and dysregulation of ... more.

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