CENTRAL-NERVOUS-SYSTEM HISTIOCYTOSIS-X - IMAGING AND RESPONSES TO CHEMOTHERAPY

Histiocytosis X is the term first coined by Lichtenstein in 1953(1) to describe a heterogeneous group of disorders which is considered now t o include Hand-Schuller-Christian disease, Letterer-Siwe disease and E osinophilic Granuloma of bone. Gagel, in 1941, first described involve ment of the central nervous system (CNS) in Histiocytosis X2 - in this case the hypothalamus and posterior pituitary were the areas principa lly affected. CNS involvement outwith these areas is rare, generally d ifficult to diagnose,3 and little information on treatment is availabl e. In this case we describe a man with cranial histiocytosis X who was treated with intrathecal and systemic chemotherapy and cranial irradi ation, and we comment upon the value of magnetic resonance imaging (MR I) in this condition.