Grisanti S.

Zentrum für Augenheilkunde, Abt. für Netzhaut- und Glaskörperchirurgie, Eberhard-Karls-Universität Tübingen

Bovine spongiform encephalopathy (BSE) belongs to a group of neurodegenerative disorders. Though the pathogenesis of this disease is not completely understood yet, "infectious proteins" called prions seem to be causative. These pathogenic agents are characterized by novel modes of replication and transmission mechanisms. The most important members of this pathological entity are BSE ( 'mad cow disease') of cattle, scrapie of sheep and Creutzfeldt-Jakob disease (CJD) of man. Despite their rarity, human prion diseases have recently been covered extensively in the media because of the likely connection between a new variant of human CJD (vCJD) and BSE and the possibility of contamination of human tissue by the vCJD agent. The BSE crisis as an important impact on ophthalmology, since the eye, a part of the central nervous system, may contain very high levels of prions. This implicates an higher risk of infectivity both in transplantation (e.g. cornea), as well as in other surgical interventions. Since prions resist routine sterilisation, contamination of surgical instruments is not excluded and requires the adaptation of more effective sterilisation techniques. This paper reviews the basic biological properties of prions and the modes of replication and transmission mechanisms, followed by a discussion about the implications and necessitating precautions in ophthalmology.

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