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    Neuroprotective treatment promising for macular telangiectasia

    CNTF found effective in slowing vision loss from photoreceptor cell death in animal models

     

    Lowy Medical Research Institute

    Research into macular telangiectasia type 2 took off in 2005, when funding by the Lowy Family Group, owners of the Westfield Corp., initiated the MacTel Project.

    The project has since grown, taking the form of the Lowy Medical Research Institute (LMRI), which is devoted to full-time investigation of the disease and related neurological, vascular, and glial degenerative disease of the retina. LMRI resides next to the campus of The Scripps Research Institute in La Jolla.

    In addition to its own full-time staff, the institute supports the activities of macular telangiectasia-related research through grants to about a dozen laboratories and 22 clinics worldwide. Along with basic preclinical basic research, it has sponsored programs in adaptive optics, OCT, metabolomics, visual psychophysics, and genetics.

    In its early days—under the direction of Emily Chew, MD, of the National Eye Institute, and Alan Bird, MD, of University College London and Moorfields Eye Hospital—the project undertook a multicentered, international natural history study of MacTel type 2 and identified several key features:

    • Loss of luteal pigment centrally comes as one of the first signs, and changes from this loss can be imaged using fundus autofluorescence with a confocal laser ophthalmoscope. Abnormalities in this and blue light reflectance were shown by Frank Holz, MD, University of Bonn in Germany; Daniel Pauleikhoff, MD, medical faculty of the University of Duisburg in Essen, Germany, and Alain Gaudric, MD, at the University of Paris.

    • Abnormalities in photoreceptors—visible on optical coherence tomography (OCT) early in the disease—often comes with significant loss of scotopic and photopic function beginning nasal to fixation. It can lead to pre-fixational blindness. 

    • Cone loss also characterizes the early disease, and is visible with adaptive optics imaging. The photoreceptor loss may be functional rather than structural, with two independent studies from Austin Roorda, PhD, and Brandon Lujon, MD, University of California, Berkeley; Jacque Duncan, MD, University of California, San Francisco; and Joseph Carroll, PhD, Medical College of Wisconsin in Milwaukee, showing that affected cones may lose their outer segments but preserve inner segments.

    • Anti-vascular endothelial growth factor (VEGF) treatment reverses the blood vessel changes but does not influence progression of photoreceptor cell loss or functional loss.

    • The retina is thinner than normal unlike other macular conditions such as diabetic retinopathy.

    • Dye leaks during fluorescein angiography of eyes with macular telangiectasia type 2.  Opacification of the retina occurs very early in disease but is not due to retinal edema. Histopathological examination of several post mortem eyes taken from patients with MacTel shows the presence of sub-retinal deposits and an abnormality of Müller glia.

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