What's new AAPOS 2008 - The Private Eye Clinic

This review article summarizes current knowledge on normal retinal physiology, the
investigative techniques used, and the range of clinical disorders in which there is
predominantly inner retinal dysfunction. Inner retinal dysfunction is encountered in a
number of retinal disorders, either inherited or acquired, as a primary or predominant
defect. Fundus examination is rarely diagnostic in these disorders, although some
show characteristic features, and careful electrophysiological assessment of retinal
function is needed for accurate diagnosis. The ERG in inner retinal dysfunction typically
shows a negative waveform with a preserved a-wave and a selectively reduced b-wave.
Advances in retinal physiology and molecular genetics have led to a greater
understanding of the pathogenesis of these disorders. This paper includes a detailed
discussion of inherited disorders such as congenital stationary night blindness, X-linked
retinoschisis, vitreoretinal dystrophies, systemic diseases such as neuronal ceroid
lipofuscinosis and Refsum disease; and several acquired conditions.
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