Extensive mixed vascular malformation clinically imitating multiple sclerosis--case report.
Vascular malformations usually develop as a result of influence of teratogenic factor(s) acting in the defined embryonic/fetal period. However, in the case examined by us, various types of vascular malformations formed in different periods of the ontogenic development were found. They were seen in all parts of the central nervous system and clinically mimicked multiple sclerosis. On the background of generalized ischemic lesions of the CNS, certain kinds of vascular malformations were seen: cavernous or fetallike vessels within meninges, superficially located capillary angioma penetrating into the brain and spinal cord white matter, and arterio-venous pathological conglomerates forming meningeal angiomatosis. In pathological vessels, immunocytochemical assessment of vascular endothelium with antibodies against antigens CD31, CD34, von Willebrand factor and lectin Ulex europaeus was normal but examination of the vascular basal membrane compounds revealed poor immunoreactivity to laminin and fibronectin. There were no disturbances in expression of angiopoietin, platelet-derived growth factor, transforming growth factor beta and vascular endothelial growth factor receptors Tie-1/2, PDGFR-alpha/beta, endoglin and Flk-1, respectively. The presence of various types of pathological vessels originating from different ontogenic periods indicates remittent or prolonged influence of teratogenic factor(s) in all periods of fetal vessel development.
Rafalowska
J
J
Department of Experimental and Clinical Neuropathology, Medical Research Centre, Polish Academy of Sciences, Pawinskiego str. 5, 02-106 Warsaw, Poland. ddziewul@amwaw.edu.pl
Dziewulska
D
D
Podlecka
A
A
Zakrzewska-Pniewska
B
B
D000015:Abnormalities, Multiple / Q000473:pathology*; D000293:Adolescent; D000328:Adult; D001921:Brain / Q000098:blood supply* / Q000196:embryology* / Q000473:pathology*; D020785:Central Nervous System Vascular Malformations / Q000175:diagnosis* / Q000378:metabolism; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D004730:Endothelium, Vascular / Q000196:embryology / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000175:diagnosis*; D013341:Sturge-Weber Syndrome / Q000473:pathology