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Purpose To assess histopathological findings in patients with cerebral autosomal dominant arteriopathy with sub-cortical infarcts and leukoencephalopathy(CADASIL).Design Case reports and histopathological evaluation of enucleated eyes.Methods Four eyes f rom two CADASIL patients were enucleated at autopsy and prepared for histopathological analysis using l ight and electron mi-croscopy.Results Thickening of arterial walls with fibro-sis,eosinophilic Periodic acid Sch iffpositive basement membrane material and loss of vascul ar smoothmuscle cells(VSMC)in the central retinal artery and its branches,the leptomeninges,the ocular adnexa,a nd the optic disk were observed.On electron microscopy,n umerous deposits of granular,osmiophilic material in a rterial walls as well as VSMC and pericyte degeneration were noted.In contrast to retinal vessels,the choroid was n ot affected.Conclu-sion Our findings suggest a differen tial involvement of small blood vessels in CADASIL,depending on the an-gioarchitecture and support autopsy data of nervous tissue describing that loss of VSMCs is most pronounced in tis-sues depending on bloodtissue barriers.
Purpose To assess histopathological findings in patients with cerebral autosomal dominant arteriopathy with sub-cortical infarcts and leukoencephalopathy (CADASIL). Design Case reports and histopathological evaluation of enucleated eyes. Methods Four eyes f rom two CADASIL patients were enucleated at autopsy and prepared for histopathological analysis using l ight and electron mi-croscopy. Results Thickening of arterial walls with fibro-sis, eosinophilic Periodic acid Sch iff positive basement membrane material and loss of vascul ar smoothmuscle cells (VSMC) in the central retinal artery and its branches, the leptomeninges, the ocular adnexa, a nd the optic disk were observed. On electron microscopy, n urous deposits of granular, osmiophilic material in a rterial walls as well as VSMC and pericyte degeneration were. In contrast to retinal vessels, the choroid was n ot affected. Conclu-sion Our findings suggest a differen tial involvement of small blood vessels in CADASIL, depending on the an-gio architecture and support autopsy data of nervous tissue describing that loss of VSMCs is most pronounced in tis-sues depending on bloodtissue barriers.