XANTHOMA DISSEMINATUM WITH MASSIVE INTRACRANIAL INVOLVEMENT

We present the case of a man who died 11 years after the onset of xant homa disseminatum, His course was marked by numerous, large recurrent intracranial dural-based xanthomatous tumors requiring repeated neuros urgical intervention. Late in his course there was progressive quadrip aresis, ataxia, ophthalmoplegia and bulbar palsy as a result of intrap arenchymal brainstem involvement, At autopsy, there was extensive meni ngeal and intraparenchymal CNS disease and widespread systemic infiltr ates, The gross and microscopic pathology are presented with immunohis tochemical, ultrastructural and biochemical details, The present case is discussed in the context of the current classification scheme and t he recent literature, where it is exceptional for the extent of CNS di sease. Xanthoma disseminatum is currently classified as a non-X histio cytopathy, the pathogenesis of which remains uncertain, CNS involvemen t carries a poor prognosis in this otherwise benign condition.