XANTHOMA DISSEMINATUM WITH MASSIVE INTRACRANIAL INVOLVEMENT

Citation
Rr. Hammond et Lra. Mackenzie, XANTHOMA DISSEMINATUM WITH MASSIVE INTRACRANIAL INVOLVEMENT, Clinical neuropathology, 14(6), 1995, pp. 314-321
Citations number
32
Categorie Soggetti
Neurosciences,Pathology
Journal title
ISSN journal
07225091
Volume
14
Issue
6
Year of publication
1995
Pages
314 - 321
Database
ISI
SICI code
0722-5091(1995)14:6<314:XDWMII>2.0.ZU;2-9
Abstract
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.