CENTRAL-NERVOUS-SYSTEM DISEASE IN LANGERHANS CELL HISTIOCYTOSIS

Central nervous system (CNS) disease in Langerhans cell histiocytosis (LCH) is a poorly understood complication of yet unknown frequency. By far the most common manifestation is in the hypothalamic-pituitary sy stem with diabetes insipidus as the leading sign, followed by other en docrinopathies and hypothalamic dysfunction. However, essentially all other parts of the CNS may be involved. On the one hand, space-occupyi ng histiocytic infiltrates may lead to size-and site-depending symptom s, extending from adjacent bone lesions or arising from the meninges o r choroid plexus. On the other hand, a progressive neurological deteri oration can occur with mainly cerebellar-pontine symptoms. Ln this art icle, these clinical patterns are described in correlation with the mo rphology on MR imaging and histopathology. Further, the therapeutic st rategies are reviewed critically, and guidelines for the management of patients with LCH-related CNS disease are presented.