PATHOLOGY OF THE CENTRAL-NERVOUS-SYSTEM IN CHESTER-ERDHEIM-DISEASE - REPORT OF 3 CASES

Chester-Erdheim disease is a rare form of non-langerhans cell histiocy tosis consisting of disseminated xanthogranulomatous infiltration and fibrosis that primarily involves the bones, visceral organs and system ic fatty spaces. Involvement of the central nervous system is variable , and neuropathological features have seldom been documented. We repor t the neuropathological findings in 3 autopsy cases. One patient had r adiological and pathological bone changes characteristic of Chester-Er dheim disease. Neuropathology revealed multiple characteristic xanthog ranulomas disseminated in the cerebral hemispheres, hypothalamus, cere bellum, and brainstem. The second patient presented first with cutaneo us lesions characteristic of Langerhans cell histiocytosis. She subseq uently developed bone abnormalities suggestive of Chester-Erdheim dise ase, which was confirmed by autopsy, raising the possibility of a comm on spectrum of histiocytosis including both diseases. Gross examinatio n of the brain was normal; however, microscopy showed infiltration of the brain by characteristic non-langerhans cell xanthogranulomas. The third patient presented with systemic features characteristic of Chest er-Erdheim disease. Neurological signs included gait disturbance, seiz ures and confusion. Examination of the brain did not show any histiocy tic infiltration, but did show changes suggestive of Hallervorden-Spat z syndrome. Association of Chester-Erdheim disease and Hallervorden-Sp atz syndrome has not been previously reported. The relationship betwee n both conditions is unclear.