Creutzfeldt-Jakob Disease and the Eye. II. Ophthalmic and Neuro-Ophthalmic Features
Creutzfeldt-Jakob c.J. LUECK, G.G. McILWAINE, M. ZEIDLER disease and the eye. II. Ophthalmic and neuro-ophthalmic features In this article, we discuss the various noted to be most marked in the occipital cortex. ophthalmic and neuro-ophthalmic A similar case involving hemianopia was manifestations of transmissible spongiform reported by Meyer et al.23 in 1954, and they encephalopathies (TSEs) as they affect man. coined the term 'Heidenhain syndrome'. This Such symptoms and signs are common, a term is now generally taken to describe any case number of studies reporting them as the third of CJD in which visual symptoms predominate most frequently presenting symptoms of in the early stages. Many studies suggest that Creutzfeldt-Jakob disease (CJD}.1,2 As a result, it the pathology of these cases is most marked in is likely that some patients will present to an the occipital lobes,1 2,22-33 and ophthalmologist. Recognition of these patients electroencephalogram (EEG) abnormalities may is important, not simply from the point of view also be more prominent over the OCcipital of diagnosis, but also from the aspect of 10bes.34 preventing possible transmission of the disease Many reports describe visual symptoms and 3 to other patients. The accompanying article signs in detail, and these will be dealt with provides a summary of our current below. In some cases, the description of the understanding of the molecular biology and visual disturbance is too vague to allow further general clinical features of the conditions. comment. Such descriptions include 'visual For ease of classification, the various disturbance',35-48 'visual problems',49 'visual symptoms and signs have been described in defects',5o 'vague visual difficulties',51 'failing three groups: those which affect vision, those vision',52 'visual loss',53,54 'distorted vision',25 which affect ocular motor function, and the c.J.
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