1. N J Andrews, C P Farrington, H J T Ward, HJ Ward, S N Cousens, P G Smith, A M Molesworth, R S G Knight, RS Knight, J W Ironside, and R G Will. Deaths from variant Creutzfeldt-Jakob disease in the UK. Lancet, 361(9359):751-2, March 2003.
   Keywords: Adolescent, Cause of Death, Child, Child Preschool, Cohort Studies, Comparative Study, Creutzfeldt-Jakob Syndrome, mortality, Cross-Sectional Studies, Great Britain, epidemiology, Human, Incidence, Population Surveillance, Support Non-U.S. Gov't.

   Abstract:

   In 2002, 17 people died from variant CJD (vCJD) in the UK, compared with 20 in 2001 and 28 in 2000. We analysed data for deaths from vCJD since 1995 and estimated the underlying trend in mortality. The trend had a quadratic component (p=0.005), suggesting that the increase was not exponential, and that the previously increasing trend is slowing down. The death rate peaked in 2000. These findings are encouraging, but mortality might increase again in the future.

   
   

   @ARTICLE{andrews:lancet:2003,
   AUTHOR = {N J Andrews and C P Farrington and H J T Ward and HJ Ward and S N Cousens and P G Smith and A M Molesworth and R S G Knight and RS Knight and J W Ironside and R G Will},
   JOURNAL = {Lancet},
   TITLE = {Deaths from variant Creutzfeldt-Jakob disease in the UK},
   YEAR = {2003},
   MONTH = {March},
   OPTNOTE = {},
   NUMBER = {9359},
   PAGES = {751-2},
   VOLUME = {361},
   KEYWORDS = {Adolescent, Cause of Death, Child, Child Preschool, Cohort Studies, Comparative Study, Creutzfeldt-Jakob Syndrome, mortality, Cross-Sectional Studies, Great Britain, epidemiology, Human, Incidence, Population Surveillance, Support Non-U.S. Gov't},
   ABSTRACT = {In 2002, 17 people died from variant CJD (vCJD) in the UK, compared with 20 in 2001 and 28 in 2000. We analysed data for deaths from vCJD since 1995 and estimated the underlying trend in mortality. The trend had a quadratic component (p=0.005), suggesting that the increase was not exponential, and that the previously increasing trend is slowing down. The death rate peaked in 2000. These findings are encouraging, but mortality might increase again in the future.} 
   }
   




2. D A Brown, M E Bruce, and J R Fraser. Comparison of the neuropathological characteristics of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) in mice. Neuropathol Appl Neurobiol, 29(3):262-72, June 2003.

   Abstract:

   Bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) belong to a group of diseases called the transmissible spongiform encephalopathies (TSEs). Transmission studies in inbred mice (strain typing) provided overwhelming evidence that vCJD arose from BSE. In this study, we compare the patterns of neuropathology in a panel of three inbred mouse strains (RIII, C57BL and VM) and one cross (C57BL x VM) infected with either vCJD or BSE. For each mouse strain, patterns of abnormal prion protein (PrPres) deposition, astrocytosis and vacuolation were similar in the vCJD- and BSE-challenged mice. Prion protein (PrP)-positive plaques were prominent in the VM and C57BL x VM mice in addition to diffuse PrPres accumulation, whereas only diffuse PrPres labelling was observed in the RIII and C57BL mice. The hippocampus was targeted in all mouse strains, as was the cochlear nucleus in the medulla, both showing consistent severe vacuolation and heavy PrPres deposition. Although the targeting of PrPres was similar in the BSE- and vCJD-infected brains, the amount and intensity of PrPres observed in the brains treated with formic acid during fixation was reduced considerably. The distribution of astrocytosis was similar to the targeting of PrPres deposition in the brain, although some differences were observed in the hippocampi of mice challenged with vCJD. We conclude that there are no significant differences in the targeting of neuropathological changes observed in the BSE- and vCJD-infected mice, consistent with the previous evidence of a link between BSE and vCJD.

   
   

   @ARTICLE{brown:nan:2003,
   AUTHOR = {D A Brown and M E Bruce and J R Fraser},
   JOURNAL = {Neuropathol Appl Neurobiol},
   TITLE = {Comparison of the neuropathological characteristics of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) in mice},
   YEAR = {2003},
   MONTH = {June},
   OPTNOTE = {},
   NUMBER = {3},
   PAGES = {262-72},
   VOLUME = {29},
   ABSTRACT = {Bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) belong to a group of diseases called the transmissible spongiform encephalopathies (TSEs). Transmission studies in inbred mice (strain typing) provided overwhelming evidence that vCJD arose from BSE. In this study, we compare the patterns of neuropathology in a panel of three inbred mouse strains (RIII, C57BL and VM) and one cross (C57BL x VM) infected with either vCJD or BSE. For each mouse strain, patterns of abnormal prion protein (PrPres) deposition, astrocytosis and vacuolation were similar in the vCJD- and BSE-challenged mice. Prion protein (PrP)-positive plaques were prominent in the VM and C57BL x VM mice in addition to diffuse PrPres accumulation, whereas only diffuse PrPres labelling was observed in the RIII and C57BL mice. The hippocampus was targeted in all mouse strains, as was the cochlear nucleus in the medulla, both showing consistent severe vacuolation and heavy PrPres deposition. Although the targeting of PrPres was similar in the BSE- and vCJD-infected brains, the amount and intensity of PrPres observed in the brains treated with formic acid during fixation was reduced considerably. The distribution of astrocytosis was similar to the targeting of PrPres deposition in the brain, although some differences were observed in the hippocampi of mice challenged with vCJD. We conclude that there are no significant differences in the targeting of neuropathological changes observed in the BSE- and vCJD-infected mice, consistent with the previous evidence of a link between BSE and vCJD.} 
   }
   




3. I Capek and V Vaillant. Creutzfeldt-Jakob disease and related diseases in France from 1998 to 2000. Euro Surveill, 8(1):14-8, January 2003.
   Keywords: Age Distribution, Codon, Creutzfeldt-Jakob Syndrome, epidemiology, France, epidemiology, Human, Mutation, Population Surveillance, Risk Factors.

   Abstract:

   In France, the number of reports of suspected CJD increased from 1998 to 2000, probably due to the increase in the requests for biological tests: respectively 459 in 1998, 590 in 1999, and 823 in 2000. For all three years, the distribution by sex is similar, with a sex ratio (M/F) of 1.05. The proportion of suspected cases aged under 50 remained stable (1617777761750f all reports in 1998-2000). The number of sporadic CJD, confirmed or probable, was stable, with a mortality ratio of 1.38 per one million in 1998, 1.56 in 1999, and 1.41 in 2000.

   
   

   @ARTICLE{capek:es:2003,
   AUTHOR = {I Capek and V Vaillant},
   JOURNAL = {Euro Surveill},
   TITLE = {Creutzfeldt-Jakob disease and related diseases in France from 1998 to 2000},
   YEAR = {2003},
   MONTH = {January},
   OPTNOTE = {},
   NUMBER = {1},
   PAGES = {14-8},
   VOLUME = {8},
   KEYWORDS = {Age Distribution, Codon, Creutzfeldt-Jakob Syndrome, epidemiology, France, epidemiology, Human, Mutation, Population Surveillance, Risk Factors},
   ABSTRACT = {In France, the number of reports of suspected CJD increased from 1998 to 2000, probably due to the increase in the requests for biological tests: respectively 459 in 1998, 590 in 1999, and 823 in 2000. For all three years, the distribution by sex is similar, with a sex ratio (M/F) of 1.05. The proportion of suspected cases aged under 50 remained stable (1617777761750f all reports in 1998-2000). The number of sporadic CJD, confirmed or probable, was stable, with a mortality ratio of 1.38 per one million in 1998, 1.56 in 1999, and 1.41 in 2000.} 
   }
   




4. Philippe Demaerel, Raf Sciot, Wim Robberecht, Rene Dom, Dirk Vandermeulen, Frederik Maes, and Guido Wilms. Accuracy of diffusion-weighted MR imaging in the diagnosis of sporadic Creutzfeldt-Jakob disease. J Neurol, 250(2):222-5, February 2003.
   Keywords: Aged, Autopsy, Brain, pathology, Creutzfeldt-Jakob Syndrome, pathology, Electroencephalography, Female, Human, Image Processing Computer-Assisted, Magnetic Resonance Imaging, Male, Middle Age, Prospective Studies, Support Non-U.S. Gov't.

   Abstract:

   The definitive diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) is based on brain autopsy. The 14-3-3 analysis in the CSF is considered a highly sensitive and specific procedure. Sensitivity, specificity and accuracy of EEG, the 14-3-3 assay and MR imaging in 12 patients referred for suspected sCJD were calculated. We suggest that diffusion-weighted MR imaging (DWI) should be included in the array of diagnostic tests because of the 100 (äÿ‘øM‚ensitivity and specificity.

   
   

   @ARTICLE{demaerel:jn:2003,
   AUTHOR = {Philippe Demaerel and Raf Sciot and Wim Robberecht and Rene Dom and Dirk Vandermeulen and Frederik Maes and Guido Wilms},
   JOURNAL = {J Neurol},
   TITLE = {Accuracy of diffusion-weighted MR imaging in the diagnosis of sporadic Creutzfeldt-Jakob disease},
   YEAR = {2003},
   MONTH = {February},
   OPTNOTE = {},
   NUMBER = {2},
   PAGES = {222-5},
   VOLUME = {250},
   KEYWORDS = {Aged, Autopsy, Brain, pathology, Creutzfeldt-Jakob Syndrome, pathology, Electroencephalography, Female, Human, Image Processing Computer-Assisted, Magnetic Resonance Imaging, Male, Middle Age, Prospective Studies, Support Non-U.S. Gov't},
   ABSTRACT = {The definitive diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) is based on brain autopsy. The 14-3-3 analysis in the CSF is considered a highly sensitive and specific procedure. Sensitivity, specificity and accuracy of EEG, the 14-3-3 assay and MR imaging in 12 patients referred for suspected sCJD were calculated. We suggest that diffusion-weighted MR imaging (DWI) should be included in the array of diagnostic tests because of the 100 (äÿÚ‘øM‚ensitivity and specificity.} 
   }
   




5. Oded Gonen. Higher Field Strength for Proton MR Spectroscopy. AJNR Am J Neuroradiol, 24(5):781-2, May 2003.
   

   @ARTICLE{gonen:aajn:2003,
   AUTHOR = {Oded Gonen},
   JOURNAL = {AJNR Am J Neuroradiol},
   TITLE = {Higher Field Strength for Proton MR Spectroscopy},
   YEAR = {2003},
   MONTH = {May},
   OPTNOTE = {},
   NUMBER = {5},
   PAGES = {781-2},
   VOLUME = {24} 
   }
   




6. D A Hilton and J W Ironside. Screening for variant Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry, 74(6):828-9, June 2003.
   

   @ARTICLE{hilton:jnnp:2003,
   AUTHOR = {D A Hilton and J W Ironside},
   JOURNAL = {J Neurol Neurosurg Psychiatry},
   TITLE = {Screening for variant Creutzfeldt-Jakob disease},
   YEAR = {2003},
   MONTH = {June},
   OPTNOTE = {},
   NUMBER = {6},
   PAGES = {828-9},
   VOLUME = {74} 
   }
   




7. Kiyotoshi Kaneko. [Creutzfeldt-Jakob disease (CJD), variant CJD, and BSE]. Nippon Rinsho, 61 Suppl 3:9-16, March 2003.
   

   @ARTICLE{kaneko:nr:2003,
   AUTHOR = {Kiyotoshi Kaneko},
   JOURNAL = {Nippon Rinsho},
   TITLE = {[Creutzfeldt-Jakob disease (CJD), variant CJD, and BSE]},
   YEAR = {2003},
   MONTH = {March},
   OPTNOTE = {},
   OPTNUMBER = {},
   PAGES = {9-16},
   VOLUME = {61 Suppl 3} 
   }
   




8. J L Laplanche, V Lepage, K Peoc'h, N Delasnerie-Laupretre, and D Charron. HLA in French patients with variant Creutzfeldt-Jakob disease. Lancet, 361(9356):531-2, February 2003.
   Keywords: Alleles, Creutzfeldt-Jakob Syndrome, genetics, France, Gene Frequency, HLA-DQ Antigens, genetics, Histocompatibility Antigens Class II, genetics, Histocompatibility Testing, Human, Tumor Necrosis Factor, genetics.
   

   @ARTICLE{laplanche:lancet:2003,
   AUTHOR = {J L Laplanche and V Lepage and K Peoc'h and N Delasnerie-Laupretre and D Charron},
   JOURNAL = {Lancet},
   TITLE = {HLA in French patients with variant Creutzfeldt-Jakob disease},
   YEAR = {2003},
   MONTH = {February},
   OPTNOTE = {},
   NUMBER = {9356},
   PAGES = {531-2},
   VOLUME = {361},
   KEYWORDS = {Alleles, Creutzfeldt-Jakob Syndrome, genetics, France, Gene Frequency, HLA-DQ Antigens, genetics, Histocompatibility Antigens Class II, genetics, Histocompatibility Testing, Human, Tumor Necrosis Factor, genetics} 
   }
   




9. Jennifer Martindale, Michael D Geschwind, Stephen De Armond, Armond S De, Geoffrey Young, W P Dillon, Roland Henry, Jane H Uyehara-Lock, David A Gaskin, and Bruce L Miller. Sporadic creutzfeldt-jakob disease mimicking variant creutzfeldt-jakob disease. Arch Neurol, 60(5):767-70, May 2003.

   Abstract:

   BACKGROUND: The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons. OBJECTIVE: To describe a patient with sporadic Creutzfeldt-Jakob disease (sCJD) who had a clinical history and initial electroencephalogram and magnetic resonance imaging findings consistent with variant CJD (vCJD). RESULTS: Results of a repeated electroencephalogram were suggestive of sCJD, and a subsequent brain biopsy confirmed this diagnosis. CONCLUSIONS: This case cautions against relying solely on T2- and diffusion-weighted pulvinar hyperintensity and clinical features to differentiate between vCJD and sCJD, and further supports established diagnostic criteria for vCJD.

   
   

   @ARTICLE{martindale:an:2003,
   AUTHOR = {Jennifer Martindale and Michael D Geschwind and De Armond, Stephen and Armond S De and Geoffrey Young and W P Dillon and Roland Henry and Jane H Uyehara-Lock and David A Gaskin and Bruce L Miller},
   JOURNAL = {Arch Neurol},
   TITLE = {Sporadic creutzfeldt-jakob disease mimicking variant creutzfeldt-jakob disease},
   YEAR = {2003},
   MONTH = {May},
   OPTNOTE = {},
   NUMBER = {5},
   PAGES = {767-70},
   VOLUME = {60},
   ABSTRACT = {BACKGROUND: The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons. OBJECTIVE: To describe a patient with sporadic Creutzfeldt-Jakob disease (sCJD) who had a clinical history and initial electroencephalogram and magnetic resonance imaging findings consistent with variant CJD (vCJD). RESULTS: Results of a repeated electroencephalogram were suggestive of sCJD, and a subsequent brain biopsy confirmed this diagnosis. CONCLUSIONS: This case cautions against relying solely on T2- and diffusion-weighted pulvinar hyperintensity and clinical features to differentiate between vCJD and sCJD, and further supports established diagnostic criteria for vCJD.} 
   }
   




10. Oscar E Mendez, Jingzi Shang, Charles A Jungreis, and Daniel I Kaufer. Diffusion-weighted MRI in Creutzfeldt-Jakob disease: a better diagnostic marker than CSF protein 14-3-3?. J Neuroimaging, 13(2):147-51, April 2003.
    Keywords: Aged, Case Report, Creutzfeldt-Jakob Syndrome, cerebrospinal fluid, Fatal Outcome, Female, Human, Magnetic Resonance Imaging, methods, Male, Support U.S. Gov't P.H.S., Tyrosine 3-Monooxygenase, cerebrospinal fluid.

    Abstract:

    Two middle-aged patients presented with rapidly progressive dementia and ataxia, nonspecific electroencephalography findings, and negative cerebrospinal fluid (CSF) protein 14-3-3. Both patients underwent brain magnetic resonance imaging (MRI) scans that demonstrated abnormalities on diffusion-weighted imaging (DWI) sequences, and both were later confirmed to have Creutzfeldt-Jakob disease. (CJD) by tissue examination. Because a recent position paper from the American Academy of Neurology characterized CSF protein 14-3-3 as a gold standard for clinically diagnosing CJD, the authors reviewed studies of CJD in which DWI-MRI imaging and CSF protein 14-3-3 studies were both performed. Among 19 reported cases of CJD with DWI-MRI lesions, CSF protein 14-3-3 was negative in 6 cases and positive in 2 others. The authors' findings suggest that multifocal cortical and subcortical hyperintensities confined to gray matter regions in DWI-MRI may be a more useful noninvasive diagnostic marker for CJD than CSF protein 14-3-3. These observations provide a compelling rationale for a prospective comparative study.

    
    

    @ARTICLE{mendez:jn:2003,
    AUTHOR = {Oscar E Mendez and Jingzi Shang and Charles A Jungreis and Daniel I Kaufer},
    JOURNAL = {J Neuroimaging},
    TITLE = {Diffusion-weighted MRI in Creutzfeldt-Jakob disease: a better diagnostic marker than CSF protein 14-3-3?},
    YEAR = {2003},
    MONTH = {April},
    OPTNOTE = {},
    NUMBER = {2},
    PAGES = {147-51},
    VOLUME = {13},
    KEYWORDS = {Aged, Case Report, Creutzfeldt-Jakob Syndrome, cerebrospinal fluid, Fatal Outcome, Female, Human, Magnetic Resonance Imaging, methods, Male, Support U.S. Gov't P.H.S., Tyrosine 3-Monooxygenase, cerebrospinal fluid},
    ABSTRACT = {Two middle-aged patients presented with rapidly progressive dementia and ataxia, nonspecific electroencephalography findings, and negative cerebrospinal fluid (CSF) protein 14-3-3. Both patients underwent brain magnetic resonance imaging (MRI) scans that demonstrated abnormalities on diffusion-weighted imaging (DWI) sequences, and both were later confirmed to have Creutzfeldt-Jakob disease. (CJD) by tissue examination. Because a recent position paper from the American Academy of Neurology characterized CSF protein 14-3-3 as a gold standard for clinically diagnosing CJD, the authors reviewed studies of CJD in which DWI-MRI imaging and CSF protein 14-3-3 studies were both performed. Among 19 reported cases of CJD with DWI-MRI lesions, CSF protein 14-3-3 was negative in 6 cases and positive in 2 others. The authors' findings suggest that multifocal cortical and subcortical hyperintensities confined to gray matter regions in DWI-MRI may be a more useful noninvasive diagnostic marker for CJD than CSF protein 14-3-3. These observations provide a compelling rationale for a prospective comparative study.} 
    }
    




11. Takashi Nishid, Aya M Tokumaru, Katsumi Doh-Ura, Akira Hirata, Kazuo Motoyoshi, and Keiko Kamakura. Probable sporadic Creutzfeldt-Jakob disease with valine homozygosity at codon 129 and bilateral middle cerebellar peduncle lesions. Intern Med, 42(2):199-202, February 2003.

    Abstract:

    We describe a 67-year-old Japanese man with probable sporadic Creutzfeldt-Jakob disease (CJD) who had valine homozygosity at codon 129, a rarity in the Japanese. T2-weighted magnetic resonance imaging (MRI) detected high-intensity lesions in the bilateral middle cerebellar peduncles and basal ganglia as well as cerebellar and cortical atrophy. He developed cerebellar ataxia and subsequent mental deterioration, myoclonus, and periodic synchronous discharge as shown in an electroencephalogram. Cerebrospinal fluid examination showed a high level of neuron-specific enolase and a positive immunoassay for the 14-3-3 protein. He died of pneumonia 10 months after the initial symptoms appeared. Whether or not the genetic polymorphism increased his susceptibility to sporadic CJD is not clear because valine homozygosity at codon 129 is less than 1 2147476456n the normal Japanese population. Although there is no convincing evidence in the present case, the MRI findings of cerebellar peduncle changes, which are rare in CJD, suggest a kind of degeneration, demyelination, or both.

    
    

    @ARTICLE{nishid:im:2003,
    AUTHOR = {Takashi Nishid and Aya M Tokumaru and Katsumi Doh-Ura and Akira Hirata and Kazuo Motoyoshi and Keiko Kamakura},
    JOURNAL = {Intern Med},
    TITLE = {Probable sporadic Creutzfeldt-Jakob disease with valine homozygosity at codon 129 and bilateral middle cerebellar peduncle lesions},
    YEAR = {2003},
    MONTH = {February},
    OPTNOTE = {},
    NUMBER = {2},
    PAGES = {199-202},
    VOLUME = {42},
    ABSTRACT = {We describe a 67-year-old Japanese man with probable sporadic Creutzfeldt-Jakob disease (CJD) who had valine homozygosity at codon 129, a rarity in the Japanese. T2-weighted magnetic resonance imaging (MRI) detected high-intensity lesions in the bilateral middle cerebellar peduncles and basal ganglia as well as cerebellar and cortical atrophy. He developed cerebellar ataxia and subsequent mental deterioration, myoclonus, and periodic synchronous discharge as shown in an electroencephalogram. Cerebrospinal fluid examination showed a high level of neuron-specific enolase and a positive immunoassay for the 14-3-3 protein. He died of pneumonia 10 months after the initial symptoms appeared. Whether or not the genetic polymorphism increased his susceptibility to sporadic CJD is not clear because valine homozygosity at codon 129 is less than 1 2147476456n the normal Japanese population. Although there is no convincing evidence in the present case, the MRI findings of cerebellar peduncle changes, which are rare in CJD, suggest a kind of degeneration, demyelination, or both.} 
    }
    




12. H G Pandya, S C Coley, I D Wilkinson, and P D Griffiths. Magnetic resonance spectroscopic abnormalities in sporadic and variant Creutzfeldt-Jakob disease. Clin Radiol, 58(2):148-53, February 2003.
    Keywords: Adult, Aged, Aspartic Acid, analogs & derivatives, Basal Ganglia, metabolism, Case Report, Creatine, metabolism, Creutzfeldt-Jakob Syndrome, diagnosis, Fatal Outcome, Female, Human, Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy, Thalamus, metabolism.

    Abstract:

    AIM: To study the proton MR spectroscopic findings in Creutzfeldt-Jakob disease (CJD) (sporadic and variant). MATERIALS AND METHODS: MR imaging and proton MR spectra were acquired in two patients with sporadic CJD (biopsy proven) and one patient with variant CJD. RESULTS: The two patients with sporadic CJD demonstrated MR signal change within the basal ganglia and thalami and reduced N-acetylaspartate (NAA):creatine ratios. The patient with variant CJD showed characteristic signal change within the pulvinar of the thalami and a markedly reduced N-acetylaspartate:creatine ratio. CONCLUSION: All three patients with CJD demonstrated evidence of reduced N-acetylaspartate: creatine ratios on MR spectroscopy. These changes imply that neuronal loss and/or dysfunction is a consistent finding in established CJD.

    
    

    @ARTICLE{pandya:cr:2003,
    AUTHOR = {H G Pandya and S C Coley and I D Wilkinson and P D Griffiths},
    JOURNAL = {Clin Radiol},
    TITLE = {Magnetic resonance spectroscopic abnormalities in sporadic and variant Creutzfeldt-Jakob disease},
    YEAR = {2003},
    MONTH = {February},
    OPTNOTE = {},
    NUMBER = {2},
    PAGES = {148-53},
    VOLUME = {58},
    KEYWORDS = {Adult, Aged, Aspartic Acid, analogs & derivatives, Basal Ganglia, metabolism, Case Report, Creatine, metabolism, Creutzfeldt-Jakob Syndrome, diagnosis, Fatal Outcome, Female, Human, Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy, Thalamus, metabolism},
    ABSTRACT = {AIM: To study the proton MR spectroscopic findings in Creutzfeldt-Jakob disease (CJD) (sporadic and variant). MATERIALS AND METHODS: MR imaging and proton MR spectra were acquired in two patients with sporadic CJD (biopsy proven) and one patient with variant CJD. RESULTS: The two patients with sporadic CJD demonstrated MR signal change within the basal ganglia and thalami and reduced N-acetylaspartate (NAA):creatine ratios. The patient with variant CJD showed characteristic signal change within the pulvinar of the thalami and a markedly reduced N-acetylaspartate:creatine ratio. CONCLUSION: All three patients with CJD demonstrated evidence of reduced N-acetylaspartate: creatine ratios on MR spectroscopy. These changes imply that neuronal loss and/or dysfunction is a consistent finding in established CJD.} 
    }
    




13. Cecilia Parazzini, S Mammi, M Comola, and G Scotti. Magnetic resonance diffusion-weighted images in Creutzfeldt-Jakob disease: case report. Neuroradiology, 45(1):50-2, January 2003.
    Keywords: Brain, pathology, Case Report, Creutzfeldt-Jakob Syndrome, diagnosis, Diffusion Magnetic Resonance Imaging, Female, Human, Middle Age.

    Abstract:

    We describe the diffusion-weighted MRI findings and follow-up in a case of autopsy-proven Creutzfeldt-Jakob disease that revealed abnormal hyperintensity in the cortex and basal ganglia.

    
    

    @ARTICLE{parazzini:neuroradiology:2003,
    AUTHOR = {Cecilia Parazzini and S Mammi and M Comola and G Scotti},
    JOURNAL = {Neuroradiology},
    TITLE = {Magnetic resonance diffusion-weighted images in Creutzfeldt-Jakob disease: case report},
    YEAR = {2003},
    MONTH = {January},
    OPTNOTE = {},
    NUMBER = {1},
    PAGES = {50-2},
    VOLUME = {45},
    KEYWORDS = {Brain, pathology, Case Report, Creutzfeldt-Jakob Syndrome, diagnosis, Diffusion Magnetic Resonance Imaging, Female, Human, Middle Age},
    ABSTRACT = {We describe the diffusion-weighted MRI findings and follow-up in a case of autopsy-proven Creutzfeldt-Jakob disease that revealed abnormal hyperintensity in the cortex and basal ganglia.} 
    }
    




14. Peter G Smith. The epidemics of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: current status and future prospects. Bull World Health Organ, 81(2):123-30, 2003.

    Abstract:

    The large epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom has been in decline since 1992, but has spread to other countries. The extensive control measures that have been put in place across the European Union and also in Switzerland should have brought the transmission of BSE under control in these countries, provided that the measures were properly enforced. Postmortem tests on brain tissue enable infected animals to be detected during the late stages of the incubation period, but tests that can be performed on live animals (including humans) and that will detect infections early are urgently needed. The number of infected animals currently entering the food chain is probably small, and the controls placed on bovine tissues in the European Union and Switzerland should ensure that any risks to human health are small and diminishing. Vigilance is required in all countries, especially in those in which there has been within-species recycling of ruminant feed. Fewer than 150 people, globally, have been diagnosed with variant Creutzfeldt-Jakob disease (vCJD), but there are many uncertainties about the future course of the epidemic because of the long and variable incubation period. Better control measures are necessary to guard against the possibility of iatrogenic transmission through blood transfusion or contaminated surgical instruments. These measures will required sensitive and specific, diagnostic tests and improved decontamination methods.

    
    

    @ARTICLE{smith:bwho:2003,
    AUTHOR = {Peter G Smith},
    JOURNAL = {Bull World Health Organ},
    TITLE = {The epidemics of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: current status and future prospects},
    YEAR = {2003},
    OPTMONTH = {},
    OPTNOTE = {},
    NUMBER = {2},
    PAGES = {123-30},
    VOLUME = {81},
    ABSTRACT = {The large epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom has been in decline since 1992, but has spread to other countries. The extensive control measures that have been put in place across the European Union and also in Switzerland should have brought the transmission of BSE under control in these countries, provided that the measures were properly enforced. Postmortem tests on brain tissue enable infected animals to be detected during the late stages of the incubation period, but tests that can be performed on live animals (including humans) and that will detect infections early are urgently needed. The number of infected animals currently entering the food chain is probably small, and the controls placed on bovine tissues in the European Union and Switzerland should ensure that any risks to human health are small and diminishing. Vigilance is required in all countries, especially in those in which there has been within-species recycling of ruminant feed. Fewer than 150 people, globally, have been diagnosed with variant Creutzfeldt-Jakob disease (vCJD), but there are many uncertainties about the future course of the epidemic because of the long and variable incubation period. Better control measures are necessary to guard against the possibility of iatrogenic transmission through blood transfusion or contaminated surgical instruments. These measures will required sensitive and specific, diagnostic tests and improved decontamination methods.} 
    }
    




15. Robert Turner and Terry Jones. Techniques for imaging neuroscience. Br Med Bull, 65:3-20, 2003.
    Keywords: Brain, physiology, Human, Image Interpretation Computer-Assisted, Magnetic Resonance Imaging, instrumentation, Neurosciences, methods, Tomography Emission-Computed, instrumentation.

    Abstract:

    In the last 20 years, a number of non-invasive spatial mapping techniques have been demonstrated to provide powerful insights into the operation of the brain during task performance. These are, in order of their emergence as robust technologies: positron emission tomography, source localization with EEG and MEG, and functional magnetic resonance imaging. The imaging neuroscience study areas represented in this volume use the first or last of these - PET and fMRI. The physical principles underlying both of these techniques are outlined, and the important assumptions and limitations are made explicit. The range of applications for each is briefly indicated.

    
    

    @ARTICLE{turner:bmb:2003,
    AUTHOR = {Robert Turner and Terry Jones},
    JOURNAL = {Br Med Bull},
    TITLE = {Techniques for imaging neuroscience},
    YEAR = {2003},
    OPTMONTH = {},
    OPTNOTE = {},
    OPTNUMBER = {},
    PAGES = {3-20},
    VOLUME = {65},
    KEYWORDS = {Brain, physiology, Human, Image Interpretation Computer-Assisted, Magnetic Resonance Imaging, instrumentation, Neurosciences, methods, Tomography Emission-Computed, instrumentation},
    ABSTRACT = {In the last 20 years, a number of non-invasive spatial mapping techniques have been demonstrated to provide powerful insights into the operation of the brain during task performance. These are, in order of their emergence as robust technologies: positron emission tomography, source localization with EEG and MEG, and functional magnetic resonance imaging. The imaging neuroscience study areas represented in this volume use the first or last of these - PET and fMRI. The physical principles underlying both of these techniques are outlined, and the important assumptions and limitations are made explicit. The range of applications for each is briefly indicated.} 
    }
    




16. H J T Ward, HJ Ward, M W Head, R G Will, and J W Ironside. Variant Creutzfeldt-Jakob disease. Clin Lab Med, 23(1):87-108, March 2003.

    Abstract:

    Variant CJD is a novel human prion disease that represents the first known occasion in which animal prion diseases have been transmitted to humans. There are many uncertainties concerning vCJD, including the mechanism of transmission between species, the extent of human exposure to the BSE agent, the infectious dose for humans, and the future burden of human disease. It is hoped that continuing scientific research may lead to answers to some of these questions and that further understanding of the mechanism of prion replication may lead to the development of effective treatment. Indeed a recent publication has suggested that the drugs quinacrine or chloropromazine may be candidates for the treatment of human prion diseases [42].

    
    

    @ARTICLE{ward:clm:2003,
    AUTHOR = {H J T Ward and HJ Ward and M W Head and R G Will and J W Ironside},
    JOURNAL = {Clin Lab Med},
    TITLE = {Variant Creutzfeldt-Jakob disease},
    YEAR = {2003},
    MONTH = {March},
    OPTNOTE = {},
    NUMBER = {1},
    PAGES = {87-108},
    VOLUME = {23},
    ABSTRACT = {Variant CJD is a novel human prion disease that represents the first known occasion in which animal prion diseases have been transmitted to humans. There are many uncertainties concerning vCJD, including the mechanism of transmission between species, the extent of human exposure to the BSE agent, the infectious dose for humans, and the future burden of human disease. It is hoped that continuing scientific research may lead to answers to some of these questions and that further understanding of the mechanism of prion replication may lead to the development of effective treatment. Indeed a recent publication has suggested that the drugs quinacrine or chloropromazine may be candidates for the treatment of human prion diseases [42].} 
    }
    

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