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spongiform encephalopathy (SSE) It would be desirable to keep them distinct from one another, or at least, whenever one speaks of Creutzfeldt-Jakob disease, to specify what is meant either SSE or the slower progressive dementia with signs of pyramidal and extrapyramidal affection originally described by Creutzfeldt and Jakob (the latter condition is discussed on page 913) However, decades of use makes the term Creutzfeld-Jakob disease and its acronym, CJD, virtually impossible to displace Precision in de nition of these states is of greater importance than before, since it was shown by Gibbs and Gajdusek that brain tissue from patients with SSE, injected into chimpanzees, can transmit the disease after an incubation period of a year or longer Epidemiology and Pathogenesis The disease appears in all parts of the world and in all seasons, with an annual incidence of 1 to 2 cases per million of population The incidence is higher in Israelis of Libyan origin, in immigrants to France from North Africa, and in Slovakia Although the reported incidence of SSE is somewhat higher in urban than in rural areas, temporospatial clustering of cases has not been observed, at least in the United States A small proportion of all series of cases is familial varying from 5 percent reported by Cathala and associates to 15 percent of 1435 cases analyzed by Masters and coworkers The occurrence of familial cases, not in the same household, suggests a genetic susceptibility to infection, although the possibility of common early exposure to the transmissible agent cannot be dismissed A small number of conjugal cases have also been reported The only clearly demonstrated mechanism of spread of SSE is iatrogenic, having occurred in a few cases after transplantation of corneas or dural grafts from infected individuals, after implantation of infected electroencephalographic depth electrodes, and after the injection of human growth hormone or gonadotropins that had been prepared from pooled cadaveric sources of these hormones At least one neurosurgeon is known to have acquired the disease Individuals exposed to scrapie-infected sheep and to patients with SSE are not disproportionately affected Recently, a great deal of attention has been drawn to an epidemic of prion disease among cows in the British Isles ( mad cow disease ) The epidemic began in 1985, with putative transmission of the disease to some two dozen humans These patients have been younger (average age of onset, 27 years) than those with typical SSE (65 years) and manifested curious psychiatric and sensory symptoms as the rst sign of illness; they did not exhibit the usual EEG ndings even as the illness advanced to its later stages (Will et al) This has been called new variant Creutzfeldt-Jakob disease It has recently been shown that the prion strain in these patients is identical to the one from affected cattle The mode of transmission, presumed to be the ingestion of infected meat, is reminiscent of the propagation of kuru in New Guinea by ceremonial ingestion of brain tissue from infected individuals Spongiform encephalopathy has now been fairly rmly associated with the conversion of a normal cellular protein, PrPc to an abnormal isoform, PrPSc The transformation involves a change in the physical conformation of the protein in which its helical proportion diminishes and the proportion of the pleated sheet increases (see reviews by Prusiner) Current theory holds that the infectivity of prions and their propagation in brain tissue may result from the susceptibility of the native prion protein to alter shape as a result of physical exposure to the abnormal protein, a so-called conformational disease Conformationally altered prions have a tendency to aggregate, and this may be the mode of cellular.

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There is a problem with the type of layout shown here if you start using this technique for more complex databases What happens if the data you are trying to store contains a colon As far as the Perl script is concerned, the colon specifies the end of one field and the beginning of the next An additional colon would only upset the information stored in the record Even worse, what would happen if you wanted to record multiple lines of text in the database Individual records are often stored on individual lines; multiline fields would confuse the script again You could use separate record delimiters that don t rely on the newline character, but again, this adds more complexity There are ways around these problems You could use a different character for the field and record separators, although this is still open to the same abuse and possible results You could just remove the field and record separator characters from the source before you put them in the database, but this reduces the utility of being able to store the information in the first place The simplest solution is to ignore any field or record separators and instead use fixed-length records to store the information The use of fixed-length records implies that you know the maximum size of the data that you are storing before you place it into the database, and therefore you need to know the format of the database before you write to and read from it Using fixed-length records would allow you to store any sort of information in the database, including multiple-line text, without worrying about how the data may affect the database layout The only problem with fixed-length databases is that you not only restrict the amount of information you can store, but you also increase the size of the data file for small records, as the individual fields are padded to make up the fixed lengths To complicate matters further, you may have trouble choosing a padding character that won t affect the contents of the fields you are storing Null characters, for example, produce all sorts of results when they are included as the text within a browser window when writing CGI programs In these situations, you can usually augment the fixed-length structure by also specifying the field length for each field in each record But this too increases the size of the database To add more complexity, you will also need to overcome the problems of searching the file if you are using it as a random-access database If you know the record number you want to access, then with either form of text database, you should be able to move to the location relatively quickly But if you don t know the record number, the time taken to find the data will be a factor of the database size This is why, practically, text databases are only good for small-scale installations or in situations where the information flow is basically one way Using a text database for storing log information is an example of a one-way information flow One or more programs will add data to the file without referring back to the data they have written Another script will be responsible for taking in the.