ABSTRACT
PURPOSE: To assess histopathological findings in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). DESIGN: Case reports and histopathological evaluation of enucleated eyes. METHODS: Four eyes from two CADASIL patients were enucleated at autopsy and prepared for histopathological analysis using light and electron microscopy. RESULTS: Thickening of arterial walls with fibrosis, eosinophilic Periodic acid Schiff-positive basement membrane material and loss of vascular smooth muscle cells (VSMC) in the central retinal artery and its branches, the leptomeninges, the ocular adnexa, and the optic disk were observed. On electron microscopy, numerous deposits of granular, osmiophilic material in arterial walls as well as VSMC and pericyte degeneration were noted. In contrast to retinal vessels, the choroid was not affected. CONCLUSION: Our findings suggest a differential involvement of small blood vessels in CADASIL, depending on the angioarchitecture and support autopsy data of nervous tissue describing that loss of VSMCs is most pronounced in tissues depending on blood-tissue barriers.
Subject(s)
Dementia, Multi-Infarct/pathology , Retinal Artery/ultrastructure , Retinal Diseases/pathology , Bruch Membrane/ultrastructure , Eosinophils/pathology , Fibrosis , Humans , Male , Middle Aged , Muscle, Smooth, Vascular/pathology , Pedigree , Pericytes/pathology , Pigment Epithelium of Eye/ultrastructureABSTRACT
PURPOSE: Recurrent or persistent corneal erosions and ulcerations are typical complications of atopic keratoconjunctivitis. Toxic eosinophil granule proteins such as major basic protein (MBP) and eosinophil cationic protein (ECP) may be involved in this pathogenetic process. This study was designed to demonstrate the presence of toxic eosinophil granule proteins in corneal tissue from a patient with corneal complications of atopic keratoconjunctivitis. DESIGN: Observational case report. METHODS: Three corneal buttons of a patient with atopic keratoconjunctivitis associated ulcerations or scarring were examined by light microscopy and by immunofluorescence technique. RESULTS: A linear deposition of eosinophil granular substance was detected subepithelially above Bowman's membrane in all corneal buttons. Indirect immunofluorescence identified this material as MBP and ECP. The deposits were not limited to the area of ulceration, but were also found underneath intact corneal epithelium. Multiple eosinophils were present in the upper corneal stroma. Normal corneas and negative control sections of the pathologic buttons revealed only minimal nonspecific staining at the surface of the epithelium. CONCLUSIONS: Both MBP and ECP are known to affect human corneal epithelial cell viability and morphology in vitro. Moreover, MBP was shown to inhibit epithelial migration and protein synthesis. These toxic eosinophil proteins may also be responsible for corneal instability, recurrent and persistent corneal epithelial defects and ulcerations in patients with atopic keratoconjunctivitis.
