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@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.}
}
@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.}
}
@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 (16% of 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{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 %
sensitivity and specificity.}
}
@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}
}
@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}
}
@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}
}
@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}
}
@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.}
}
@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.}
}
@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% in
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{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.}
}
@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.}
}
@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.}
}
@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.}
}
@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].}
}
@ARTICLE{alperovitch:crasi:2002,
AUTHOR = {Annick Alperovitch and Robert G Will},
JOURNAL = {C R Acad Sci III},
TITLE = {Predicting the size of the vCJD epidemic in France},
YEAR = {2002},
MONTH = {January},
OPTNOTE = {},
NUMBER = {1},
PAGES = {33-6},
VOLUME = {325},
KEYWORDS = {Animal, Cattle, Creutzfeldt-Jakob Syndrome,
epidemiology, Encephalopathy Bovine Spongiform, transmission, France,
epidemiology, Human, Meat},
ABSTRACT = {More than 5 years after the description of the first
cases of variant Creutzfeldt-Jakob disease (vCJD), there is still
great uncertainty about the size of the vCJD epidemic in the United
Kingdom (UK), although the most recent predictions based on
statistical modelling are more optimistic than the previous ones. The
number of vCJD cases in France is far too small to attempt any direct
modelling of the vCJD epidemic in the French population. Comparative
assessment of the level of exposure to the bovine spongiform
encephalopathy (BSE) agent in the UK and France could help to
estimate the size of the vCJD epidemic in France. Data on imports of
beef products from the UK between 1985 and 1996, BSE epidemic in
French cattle, and travel of French people to the UK suggest that the
French population was much less exposed to the BSE agent than the UK
population. The France/UK ratio of vCJD incidence is currently
approximately equal to 0.05. Further studies are needed to estimate
accurately the exposure ratio between UK and France and to examine
whether comparative data about exposure and incidence are fully
consistent. The temporal pattern of exposure in UK and France, and
possible differences in exposure to high risk bovine tissues because
of food habits or risk reduction measures should be carefully
considered.}
}
@ARTICLE{beekes:dmw:2002,
AUTHOR = {M Beekes and R Kurth},
JOURNAL = {Dtsch Med Wochenschr},
TITLE = {[BSE and Creutzfeldt-Jakob disease. Implication on
health politics in Germany and Europe]},
YEAR = {2002},
MONTH = {February},
OPTNOTE = {},
NUMBER = {7},
PAGES = {335-40},
VOLUME = {127},
KEYWORDS = {Adolescent, Adult, Aged, Animal, Belgium, Cats, Cattle,
Comparative Study, Creutzfeldt-Jakob Syndrome, epidemiology,
Encephalopathy Bovine Spongiform, epidemiology, Europe, Forecasting,
France, Germany, Health Policy, Human, Incidence, Meat,
adverse effects, Middle Age, Occupations, Prospective Studies,
Research, Risk Factors, Sheep}
}
@ARTICLE{begic:ma:2002,
AUTHOR = {Lejla Begic and Adaleta Mulaomerovic and Selma Berbic and
Zlata Zigic},
JOURNAL = {Med Arh},
TITLE = {[New findings on the diagnosis and therapy of prion
diseases]},
YEAR = {2002},
OPTMONTH = {},
OPTNOTE = {},
NUMBER = {5-6},
PAGES = {305-11},
VOLUME = {56},
KEYWORDS = {English Abstract, Human, Prion Diseases, diagnosis},
ABSTRACT = {Spongiform encephalopathies are the fatal diseases, that
affect the brain tissue of mammals. They are caused by a
conformational changed prion protein. There is no adequate diagnostic
test for in vivo identification of prion protein. Disease can be
diagnosed only by clinical sings and EEG in new variant of
Creutzfeldt-Jakob disease. Post mortem, histopathological examination
of brain tissue reveals spongiform changes and immunohistochemistry
detects disease-related prion protein. Appropriate diagnostic in vivo
tests are not developed yet; therefore extensive researches are
ongoing aimed to introduce such methods. This review describes a few
promising experimental methods, which may develop into diagnostic
tests in the future: detection of prions in urine samples, PMCA
(protein misfolding cyclic amplification), DATAS (differential
analysis of transcripts with alternative splicing), SELEX (in vitro
selection), detection of prions in tonsils and detection of copper
and manganese dysbalance in tissues. Current therapy strategy is
based on testing of some known drugs (quinacrine, chlorpromazine),
and antioxidant and antibody treatments. The detection of NSE
(neuron-specific enolase) and cholesterol in meat products reveals
the presence of brain and spinal cord tissue. The spreading of
spongiform encephalopathies can be diminished by utilising the
adequate in vivo diagnostic tests, effective therapy strategy and
preventive steps.}
}
@ARTICLE{belay:clm:2002,
AUTHOR = {Ermias D Belay and Lawrence B Schonberger},
JOURNAL = {Clin Lab Med},
TITLE = {Variant Creutzfeldt-Jakob disease and bovine spongiform
encephalopathy},
YEAR = {2002},
MONTH = {December},
OPTNOTE = {},
NUMBER = {4},
PAGES = {849-62},
VOLUME = {22},
KEYWORDS = {Animal, Cattle, Creutzfeldt-Jakob Syndrome, diagnosis,
Encephalopathy Bovine Spongiform, diagnosis, Human},
ABSTRACT = {Strong epidemiologic and laboratory evidence indicate
that a novel, variant form of Creutzfeldt-Jakob disease (vCJD) first
reported in the United Kingdom in 1996 is causally linked with bovine
spongiform encephalopathy (BSE). BSE was first identified in the
early 1980s in the United Kingdom, and has since spread to other
European countries and recently to Japan and Israel. Although the
United Kingdom BSE epizootic is on the decline, widespread exposure
of humans to infected cattle products may have already occurred,
raising concerns about the ultimate magnitude of the vCJD outbreak
which, as of October 2002, has already affected 138 patients
worldwide, including 128 patients in the United Kingdom.}
}
@ARTICLE{bocquet:ami:2002,
AUTHOR = {Patrick Bocquet and De Stampa, Matthieu and Stampa M De and
Celine Foucher and Marie-Pierre Delporte and Joelle Martigny},
JOURNAL = {Ann Med Interne (Paris)},
TITLE = {[Sporadic Creutzfeldt-Jakob disease and isolated
dementia. Contribution of brain MRI]},
YEAR = {2002},
MONTH = {February},
OPTNOTE = {},
NUMBER = {1},
PAGES = {62-7},
VOLUME = {153},
KEYWORDS = {Case Report, Creutzfeldt-Jakob Syndrome, diagnosis,
Dementia, diagnosis, English Abstract, Fatal Outcome, Female, Human,
Magnetic Resonance Imaging, methods, Middle Age,
Sensitivity and Specificity},
ABSTRACT = {Creutzfeldt-Jakob disease (CJD) is the most frequent
transmissible spongiform encephalopathy. Its definite diagnosis is
ascertained by cerebral neuropathological exam. However, diagnosis of
a probable or possible case of CJD can be evoked on the basis of
Masters'classification which is based on the association of different
clinical and electroencephalographical criteria. We report the case
of a 58-year-old woman who died in a geriatric unit of autopsy proven
sporadic CJD. The clinical course over 15 months was rapidly
progressive dementia without characteristic clinical and EEG signs.
The case presentation did not meet the criteria of probable or
possible CJD, according to Masters'classification. However, 4 months
after the onset of the disease, t-Flair MRI revealed an increased
signal intensity in the right frontal and occipital cortex which
could suggest the diagnosis of CJD. This case therefore stresses the
contribution of MRI, especially diffusion-weighted imaging, for early
diagnosis of CJD. It shows also the short comings of
Masters'classification which does not always enable diagnosis of CJD
even though control measures would have to be rapidly undertaken,
specially the decontamination of medico-surgical equipment. Finally,
this case illustrates the great importance of post mortem exam in
such context. In light of this clinical observation, we discuss this
rare diagnosis which should be considered in geriatrics when
confronted with a rapidly progressive dementia}
}
@ARTICLE{bradley:ane:2002,
AUTHOR = {Ray Bradley},
JOURNAL = {Acta Neurobiol Exp (Warsz)},
TITLE = {Bovine spongiform encephalopathy. Update},
YEAR = {2002},
OPTMONTH = {},
OPTNOTE = {},
NUMBER = {3},
PAGES = {183-95},
VOLUME = {62},
KEYWORDS = {Animal, Animals Wild, Cats, Cattle, Cattle Diseases,
epidemiology, Encephalopathy Bovine Spongiform, epidemiology,
European Union, Great Britain, epidemiology, Human,
Legislation Medical},
ABSTRACT = {Bovine spongiform encephalopathy (BSE) is a zoonosis
being the origin of variant Creutzfeldt-Jakob disease and an
important cattle disease in its own right. Countries have been slow
to learn the importance of protecting, not only their cattle
populations, but also their human populations. Since 2000, several
additional European countries have reported BSE in native-born stock
and this has led to a concern about the BSE status of countries that
have imported cattle and cattle products from infected countries.
Extensive feed and offal bans and application of newly-developed,
'Rapid' tests for prion protein in central nervous tissue of
targeted, high-risk animals and slaughter cattle over 30 months old
now provides the tools whereby the public are fully protected and BSE
can be eradicated.}
}
@ARTICLE{caboclo:an:2002,
AUTHOR = {Luis Otavio Sales Ferreira Caboclo and LO Caboclo and
Nancy Huang and Guilherme Alves Lepski and Jose Antonio Livramento and
Carlos Alberto Buchpiguel and Claudia Sellitto Porto and
Ricardo Nitrini},
JOURNAL = {Arq Neuropsiquiatr},
TITLE = {Iatrogenic Creutzfeldt-Jakob disease following human
growth hormone therapy: case report},
YEAR = {2002},
MONTH = {June},
OPTNOTE = {},
NUMBER = {2-B},
PAGES = {458-61},
VOLUME = {60},
KEYWORDS = {Adult, Blotting Western, Case Report,
Cerebrospinal Fluid Proteins, analysis, Creutzfeldt-Jakob Syndrome,
cerebrospinal fluid, Human, Human Growth Hormone, adverse effects,
Iatrogenic Disease, Magnetic Resonance Imaging, Male,
Tyrosine 3-Monooxygenase, cerebrospinal fluid},
ABSTRACT = {We report the case of a 41-year-old man with iatrogenic
Creutzfeldt-Jakob disease (CJD) acquired after the use of growth
hormone (GH) obtained from a number of pituitary glands sourced from
autopsy material. The incubation period of the disease (from the
midpoint of treatment to the onset of clinical symptoms) was rather
long (28 years). Besides the remarkable cerebellar and mental signs,
the patient exhibited sleep disturbance (excessive somnolence) from
the onset of the symptoms, with striking alteration of the sleep
architecture documented by polysomnography. 14-3-3 protein was
detected in the CSF, and MRI revealed increased signal intensity
bilaterally in the striatum, being most evident in diffusion-weighted
(DW-MRI) sequences. This is the second case of iatrogenic CJD
associated with the use of GH reported in Brazil.}
}
@ARTICLE{cooper:jcep:2002,
AUTHOR = {J D Cooper and S M Bird},
JOURNAL = {J Cancer Epidemiol Prev},
TITLE = {UK dietary exposure to BSE in head meat: by birth cohort
and gender},
YEAR = {2002},
OPTMONTH = {},
OPTNOTE = {},
NUMBER = {2},
PAGES = {71-83},
VOLUME = {7},
KEYWORDS = {Adult, Animal, Brain, virology, Cattle, Cohort Studies,
Computer Graphics, Consumer Product Safety, standards,
Creutzfeldt-Jakob Syndrome, epidemiology,
Encephalopathy Bovine Spongiform, epidemiology, Female,
Food Contamination, Food Handling, standards, Great Britain,
epidemiology, Head, Human, Male, Meat, virology, Meat Products,
virology, Middle Age, Sex Distribution, Support Non-U.S. Gov't},
ABSTRACT = {BACKGROUND: UK dietary exposure in 1980-1996 to the
bovine spongiform encephalopathy (BSE) infectious agent through the
consumption of beef mechanically recovered meat (MRM) contained in
burgers, sausages and other meat products has already been quantified
by birth cohort (born pre-1940, 1940-1969 or post-1969) and gender.
In this paper, similar quantification is undertaken for the
consumption of bovine head meat. METHODS: Synthesis of evidence on
clinical BSE bovines, on bovines slaughtered in the last year of
their BSE incubation period, brain contamination during head meat
production, brain infectivity (option 1: 1-year preclinical bovine
54% as infectious as clinical BSE bovine; option 2: 1-year
pre-clinical bovine as infectious as clinical BSE bovine) and
1980-1996 UK dietary consumption of head meat in burgers, sausages
and other meat products. FINDINGS: Median infectivity consumed in
head meat was 49 900 (67 800 for infectivity option 2), 96 200 (126
900) and 24950 (32 800) bovine oral (Bo) ID 50 units for the
post-1969, 1940-1969 and pre-1940 birth cohorts in 1980-1989; and 143
950 (266 550 for infectivity option 2), 150 900 (279 500) and 38 350
(71 250) Bo ID50 units in 1990-1996. Males consumed almost 58% of
infectivity in 1980-1996. For all three birth cohorts, exposure to
BSE in head meat was higher in 1990-96 for both infectivity options.
Median infectivity consumed in head meat and beef MRM was 83 150 (109
000 for infectivity option 2), 161 900 (207 450) and 39 300 (50 450)
Bo ID50 units for the post-1969, 1940-1969 and pre-1940 birth cohorts
in 1980-1989; and 188 200 (348 700), 190 600 (353 050) and 47 200 (87
550) Bo ID50 units in 1990-1996. INTERPRETATION: Males consumed
almost 58% of BSE infectivity in head meat and beef MRM, which is
consistent with 60 males of 113 variant Creutzfeldt-Jakeb disease
(vCJD) onsets to 30 November 2001. If vCJD onsets to that date had
all been infected in 1980-1989, 65 of 113 vCJD onsets in the
post-1969 cohort are not consistent with its BSE exposure in
1980-1989 unless the vCJD incubation period or susceptibility depends
on age, or another exposure is involved. Experimental data are needed
to identify which brain material contaminates head meat, and further
pathogenesis data are needed to determine the corresponding
infectivity. Other salient sensitivity issues are highlighted.}
}
@ARTICLE{daignaux:ije:2002,
AUTHOR = {Jerome Huillard D'Aignaux and Simon N Cousens and
Nicole Delasnerie-Laupretre and Jean-Philippe Brandel and
Dominique Salomon and Jean-Louis Laplanche and Jean-Jacques Hauw and
Annick Alperovitch},
JOURNAL = {Int J Epidemiol},
TITLE = {Analysis of the geographical distribution of sporadic
Creutzfeldt-Jakob disease in France between 1992 and 1998},
YEAR = {2002},
MONTH = {April},
OPTNOTE = {},
NUMBER = {2},
PAGES = {490-5},
VOLUME = {31},
KEYWORDS = {Adult, Aged, Aged 80 and over, Cluster Analysis,
Creutzfeldt-Jakob Syndrome, epidemiology, Female, France,
epidemiology, Human, Male, Middle Age, Support Non-U.S. Gov't},
ABSTRACT = {BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rare
fatal dementia caused by a transmissible agent. However, the
mechanism leading to the disease is unknown in the majority of cases.
The presence of geographically clustered cases might indicate a
common environmental exposure to the transmissible agent, or
case-to-case transmission of the agent. This study sought evidence of
clustering of cases of sporadic CJD in France. METHODS: A total of
402 individuals who died from definite or probable sporadic CJD in
France between 1992 and 1998 were analysed. The geographical
distribution of cases was analysed using three different clustering
methods. An analysis of the distribution of the distances between
pairs was performed to look for evidence of clustering. Then, two
methods of cluster detection were used to identify the locations of
clusters. RESULTS: Each of our analyses found some evidence of
clustering, though the extent of that clustering differed between
approaches. The strongest evidence, statistically, related to three
cases living in a small rural area in South-West France (P = 0.001).
Two of the three cases lived in the same area throughout life. They
had also both undergone surgery on several occasions. Little
information is available on the third case. CONCLUSION: Some sporadic
CJD cases in France may be aetiologically linked. There was strong
evidence that three cases in South-West France formed a cluster but
the precise mechanism underlying this cluster of cases remains
unclear. The potentially long incubation period of the disease makes
the identification of links between such cases difficult.}
}
@ARTICLE{dearmond:toxicology:2002,
AUTHOR = {Stephen J DeArmond and Essia Bouzamondo},
JOURNAL = {Toxicology},
TITLE = {Fundamentals of prion biology and diseases},
YEAR = {2002},
MONTH = {December},
OPTNOTE = {},
OPTNUMBER = {},
PAGES = {9-16},
VOLUME = {181-182},
KEYWORDS = {Animal, Human, Models Molecular, Prion Diseases,
classification, Prions, chemistry, Protein Conformation,
Support Non-U.S. Gov't, Support U.S. Gov't P.H.S.},
ABSTRACT = {One of the most remarkable changes in medicine during
the last 20 years of the 20th century was the shift from the
clinical-neuropathological classification of Creutzfeldt-Jakob
disease (CJD) and related disorders as 'transmissible spongiform
encephalopathies' to a molecular-etiologic classification as 'prion
diseases'. We now know that these diseases are caused by
abnormalities of the prion protein (PrP). Specifically, CJD is caused
by the conversion of the normal, protease-sensitive PrP isoform,
designated PrP(C), to a protease resistant isoform, designated
PrP(Sc). PrP(Sc) forms into an infectious particle, named a 'prion',
that can transmit the disease. Accumulation of PrP(Sc) in the brain
causes neurodegeneration. The main goals of this review are to
summarize our understanding of the attributes of the PrP molecule
that give it the properties of an infectious agent and to describe
how different alterations of the PrP molecule cause the multiple
known prion disease variants. Finally, the emergence of a new variant
of CJD in Great Britain and to a lesser extent in Europe and its
relationship to the emergence of a particularly virulent form of
bovine spongiform encephalopathy will be discussed.}
}
@ARTICLE{donnelly:crb:2002,
AUTHOR = {Christl A Donnelly},
JOURNAL = {C R Biol},
TITLE = {BSE in France: epidemiological analysis and predictions},
YEAR = {2002},
MONTH = {July},
OPTNOTE = {},
NUMBER = {7},
PAGES = {793-806},
VOLUME = {325},
KEYWORDS = {Animal, Cattle, Creutzfeldt-Jakob Syndrome,
epidemiology, Encephalopathy Bovine Spongiform, epidemiology, France,
epidemiology, Human, Incidence, Probability, Support Non-U.S. Gov't},
ABSTRACT = {Attention throughout Europe continues to focus on bovine
spongiform encephalopathy (BSE) with increasing evidence linking it
to the new variant of Creutzfeldt-Jakob disease (vCJD) in humans. The
age- and cohort-specific incidence of BSE in French cattle was
modelled as a function of the survival distribution, the
cohort-specific incidence of BSE infection, the underreporting rate
of BSE cases, and the age-specific probability, conditional on
survival, that an infected animal would experience clinical onset.
The results reveal that thousands of French cattle were infected with
BSE over the course of the epidemic. However, case incidence is
predicted to decline in future years.}
}
@ARTICLE{donnelly:prslbbs:2002,
AUTHOR = {Christl A Donnelly and Neil M Ferguson and Azra C Ghani and
Roy M Anderson},
JOURNAL = {Proc R Soc Lond B Biol Sci},
TITLE = {Implications of BSE infection screening data for the
scale of the British BSE epidemic and current European infection
levels},
YEAR = {2002},
MONTH = {November},
OPTNOTE = {},
NUMBER = {1506},
PAGES = {2179-90},
VOLUME = {269},
KEYWORDS = {Animal, Cattle, Creutzfeldt-Jakob Syndrome,
epidemiology, Disease Outbreaks, veterinary,
Encephalopathy Bovine Spongiform, epidemiology,
Epidemiologic Factors, Europe, epidemiology, Female, Great Britain,
epidemiology, Human, Maternal-Fetal Exchange, Models Statistical,
Pregnancy, Support Non-U.S. Gov't},
ABSTRACT = {The incidence of confirmed clinical cases of bovine
spongiform encephalopathy (BSE) in Great Britain continues to
decline, but the recent discovery of cases in previously unaffected
countries (including Israel, Japan, Poland, Slovenia and Spain) has
heightened concerns that BSE transmission was more intense and
widespread than previously thought. We use back-calculation methods
to undertake an integrated analysis of data on infection prevalence
in apparently healthy cattle and the incidence of confirmed clinical
disease. The results indicate substantial underascertainment of
clinical cases over the course of the British epidemic, and
consequently that two- to fourfold more animals were infected than
previously estimated. Upper bounds on the predicted size of the new
variant Creutzfeldt-Jakob Disease (vCJD) epidemic are unaffected, as
the prediction methods employed fit to observed vCJD mortality data,
and are not sensitive to estimates of the absolute magnitude of past
human exposure to BSE-infected cattle, only to relative changes in
exposure through time. We also estimate the per-head incidence of
infection in cattle born between 1993 and 1997 in other European
Union countries, using data on the testing of apparently healthy
cattle slaughtered for consumption. Infection incidence for cattle
born after mid-1996 was highest in Greece, Italy and Belgium, with
Spain and The Netherlands having intermediate levels, and estimates
for Great Britain, Germany and France being comparably low.}
}
@ARTICLE{dormont:ijfm:2002,
AUTHOR = {Dominique Dormont},
JOURNAL = {Int J Food Microbiol},
TITLE = {Prions, BSE and food},
YEAR = {2002},
MONTH = {September},
OPTNOTE = {},
NUMBER = {1-2},
PAGES = {181-9},
VOLUME = {78},
KEYWORDS = {Animal, Biological Markers, Cattle,
Consumer Product Safety, Creutzfeldt-Jakob Syndrome,
Encephalopathy Bovine Spongiform, Human, Prion Diseases,
prevention & control, Prions, isolation & purification,
Risk Assessment, Zoonoses},
ABSTRACT = {Biochemical and biophysical properties of prions
including possible inactivation methods are reviewed. Possible
molecular markers of transmissible spongiform encephalopathy (TSE)
and mechanisms behind infectivity and correlation with clinical
symptoms are discussed. The risk of Bovine Spongiform Encephalopathy
(BSE) for humans i.e. variant Creutzfeldt-Jakob Disease (cCJD) is
addressed in detail. The consequences of the emergence of the new
cCJD and the lack of information on the infectivity of cCJD at the
clinical stage of the disease in relation to the need to reconsider
the biological concepts currently used in microbiology.}
}
@ARTICLE{eschweiler:nervenarzt:2002,
AUTHOR = {G W Eschweiler and H Wormstall and U Widmann and
T Naegele and M Bartels},
JOURNAL = {Nervenarzt},
TITLE = {[Correlation of diffusion-weighted magnetic resonance
imaging with neurological deficits in sporadic Creutzfeldt-Jakob
Disease]},
YEAR = {2002},
MONTH = {September},
OPTNOTE = {},
NUMBER = {9},
PAGES = {883-6},
VOLUME = {73},
KEYWORDS = {Case Report, Creutzfeldt-Jakob Syndrome, diagnosis,
Dementia, diagnosis, Diagnosis Differential,
Diffusion Magnetic Resonance Imaging, methods,
Electroencephalography, English Abstract, Fatal Outcome,
Follow-Up Studies, Hemiplegia, diagnosis, Human, Male, Middle Age,
Neurologic Examination, Neuropsychological Tests, Occipital Lobe,
pathology, Perceptual Disorders, diagnosis, Temporal Lobe, pathology,
Tomography Emission-Computed, Vertigo, etiology},
ABSTRACT = {This case report describes the sporadic
Creutzfeldt-Jakob disease (CJD) of a 53-year-old man who initially
complained about vertigo and dizziness. Within 18 weeks, he developed
impaired memory, hemineglect, and sensory impairment of the left half
of the body. A CSF tap was positive for 14-3-3 protein and showed
increased tau protein, neuron-specific enolase (NSE), and the
astroglial protein S-100 B. The EEG showed right temporal sharp waves
without periodicity. Diffusion-weighted MRI revealed hyperintensities
in the right temporo-occipital cortex which corresponded well with
hypometabolic areas in a PET scan and the neurological and
neuropsychological deficits. The morphological FLAIR T2 MRI showed no
pathological changes. Within 20 weeks, the patient developed severe
dementia with decreased spatial orientation and myoclonia, became
incontinent, and was confined to bed. He died within 22 weeks after
the first presentation of symptoms.}
}
@ARTICLE{galanaud:jn:2002,
AUTHOR = {D Galanaud and D Dormont and D Grabli and P Charles and
J J Hauw and C Lubetzki and J P Brandel and C Marsault and
P J Cozzone},
JOURNAL = {J Neuroradiol},
TITLE = {MR spectroscopic pulvinar sign in a case of variant
Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {December},
OPTNOTE = {},
NUMBER = {4},
PAGES = {285-7},
VOLUME = {29},
KEYWORDS = {Adult, Aspartic Acid, analogs & derivatives, Biopsy,
Case Report, Case-Control Studies, Choline, analysis, Creatine,
analysis, Creutzfeldt-Jakob Syndrome, diagnosis, Female, Human,
Inositol, analysis, Magnetic Resonance Spectroscopy, methods,
Phosphocreatine, analysis, Prognosis, Pulvinar, chemistry,
Sensitivity and Specificity},
ABSTRACT = {We report MR spectroscopic findings in a patient
hospitalized with biopsy-proven variant Creutzfeldt-Jakob (vCJD)
disease. N-acetyl aspartate was markedly decreased in the
postero-medial part of the thalami (pulvinar) but was not diminished
in the parieto-occipital white matter and cortical grey matter. These
observations, which are in accordance with the pathological findings
in this disease, suggest that MR spectroscopy, a highly sensitive
method for the detection of subtle brain metabolic dysfunction, could
be of interest for the diagnosis, prognosis and therapeutic follow-up
of vCJD.}
}
@ARTICLE{ghani:crasi:2002,
AUTHOR = {Azra C Ghani and Christl A Donnelly and Neil M Ferguson and
Roy M Anderson},
JOURNAL = {C R Acad Sci III},
TITLE = {The transmission dynamics of BSE and vCJD},
YEAR = {2002},
MONTH = {January},
OPTNOTE = {},
NUMBER = {1},
PAGES = {37-47},
VOLUME = {325},
KEYWORDS = {Age Factors, Animal, Cattle, Creutzfeldt-Jakob Syndrome,
epidemiology, Encephalopathy Bovine Spongiform, epidemiology, Europe,
epidemiology, Great Britain, epidemiology, Human, Meat,
Northern Ireland, epidemiology, Prions, genetics,
Support Non-U.S. Gov't},
ABSTRACT = {The bovine spongiform encephalopathy (BSE) epidemic in
cattle has had a huge economic impact on the agricultural industries
across Europe. Furthermore, scientific evidence now strongly
supporting a link between a new variant of Creutzfeldt-Jakob disease
(vCJD) and consumption of BSE-infected animals has further heightened
the need both to understand the transmission of these new diseases
and to improve control measures to protect public health. In this
paper we review work undertaken by our group using epidemiological
models to understand the transmission dynamics of BSE and vCJD. We
present new estimates of the future number of cases of BSE and the
number of infected animals slaughtered for consumption for Great
Britain, and summarise similar analyses undertaken for Northern
Ireland, Ireland, Portugal and France. We also consider the
epidemiological determinants of the future course of the vCJD
epidemic, including the age and genetic characteristics of the
confirmed cases, and present predictions of future case numbers.}
}
@ARTICLE{haik:an:2002,
AUTHOR = {Stephane Haik and Didier Dormont and Baptiste A Faucheux and
Claude Marsault and Jean-Jacques Hauw},
JOURNAL = {Ann Neurol},
TITLE = {Prion protein deposits match magnetic resonance imaging
signal abnormalities in Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {June},
OPTNOTE = {},
NUMBER = {6},
PAGES = {797-9},
VOLUME = {51},
KEYWORDS = {Brain, pathology, Brain Chemistry,
Creutzfeldt-Jakob Syndrome, metabolism, Human,
Magnetic Resonance Imaging, Prions, analysis, Support Non-U.S. Gov't}
}
@ARTICLE{henkel:jn:2002,
AUTHOR = {Karsten Henkel and Inga Zerr and Andreas Hertel and
Klaus-F Gratz and Andreas Schroter and Henriette J Tschampa and
Heiner Bihl and Udalrich Bull and Frank Grunwald and
Alexander Drzezga and Jorg Spitz and Sigrid Poser},
JOURNAL = {J Neurol},
TITLE = {Positron emission tomography with [(18)F]FDG in the
diagnosis of Creutzfeldt-Jakob disease (CJD)},
YEAR = {2002},
MONTH = {June},
OPTNOTE = {},
NUMBER = {6},
PAGES = {699-705},
VOLUME = {249},
KEYWORDS = {Adult, Aged, Atrophy, etiology, Brain, metabolism,
Cerebrovascular Circulation, physiology, Creutzfeldt-Jakob Syndrome,
metabolism, Down-Regulation, physiology, Electroencephalography,
Energy Metabolism, physiology, Female, Fludeoxyglucose F 18,
diagnostic use, Glucose, metabolism, Human, Laterality, physiology,
Magnetic Resonance Imaging, Male, Middle Age, Radiopharmaceuticals,
diagnostic use, Support Non-U.S. Gov't, Tomography Emission-Computed},
ABSTRACT = {The aim of this study was to explore the sites of
metabolic changes with [(18)F]2-fluoro-2-desoxy-D-glucose (FDG) and
positron emission tomography (PET) in patients with Creutzfeldt-Jakob
disease and to correlate the findings with clinical symptoms. Static
[(18)F]FDG-PET studies of eight patients with the diagnosis of
confirmed or probable CJD were retrospectively analysed by two
physicians from departments of nuclear medicine independently with a
strong interrater agreement (kappa=0,98). The clinical data of the
patients, based on a standardized evaluation by physicians from the
German Creutzfeldt-Jakob disease surveillance study, was correlated
with the PET findings. [(18)F]FDG-PET shows widespread hypometabolism
in CJD. All patients had a reduction of cerebral glucose metabolism
in at least one temporal or parietal region. Additionally in 7 of our
own 8 cases and 3 of 4 cases from the literature the occipital lobe,
the cerebellum or the basal ganglia were involved. These findings
differ from typical patterns of hypometabolism in Alzheimer's disease
and other neurodegenerative disorders. In two thirds of the cases the
distribution was markedly asymmetric. Myoclonus was present in five
out of our eight own cases. Our data suggest that myoclonus might
correlate with metabolic impairment of contralateral parietal and
temporal lobes. In three of four patients with visual symptoms FDG
uptake was reduced in the visual cortex bilaterally. Typical
hyperintensities on MRI were only found in two of the eight cases at
the time of PET-studies. Our results demonstrate that [(18)F]FDG-PET
appears to be a sensitive investigation in CJD and could be useful to
differentiate CJD from other neurodegenerative disorders.}
}
@ARTICLE{henry:rmv:2002,
AUTHOR = {Colm Henry and Richard Knight},
JOURNAL = {Rev Med Virol},
TITLE = {Clinical features of variant Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {May-Jun},
OPTNOTE = {},
NUMBER = {3},
PAGES = {143-50},
VOLUME = {12},
KEYWORDS = {Adult, Age Factors, Comparative Study,
Creutzfeldt-Jakob Syndrome, diagnosis, Depression, diagnosis,
Disease Progression, Great Britain, epidemiology, Human,
Magnetic Resonance Imaging, Thalamus, pathology},
ABSTRACT = {Since 1996, over one hundred cases of variant
Creutzfeldt-Jakob disease have appeared, mostly in the United
Kingdom. In this review, we summarise the major clinical features of
this progressive neurodegenerative condition and compare them with
those of sporadic Creutzfeldt-Jakob disease. We emphasise the young
age (median 26 years) at presentation and the dominant
psychiatric/behavioural features, particularly depression. Sensory
symptoms are present initially in half the cases and florid
psychiatric symptoms, such as delusions or hallucinations, are also
common. Given these symptoms, many patients present in clinical
practice initially to a psychiatrist but are referred to neurologists
when neurological signs become apparent. Although the definitive
diagnosis remains neuropathological, a confident pre-mortem diagnosis
is now possible when the 'pulvinar sign' is seen on magnetic
resonance imaging studies.}
}
@ARTICLE{krapfenbauer:electrophoresis:2002,
AUTHOR = {Kurt Krapfenbauer and Byong Chul Yoo and
Michael Fountoulakis and Eva Mitrova and Gert Lubec},
JOURNAL = {Electrophoresis},
TITLE = {Expression patterns of antioxidant proteins in brains of
patients with sporadic Creutzfeldt-Jacob disease},
YEAR = {2002},
MONTH = {August},
OPTNOTE = {},
NUMBER = {15},
PAGES = {2541-7},
VOLUME = {23},
KEYWORDS = {Aged, Antioxidants, isolation & purification,
Blotting Western, Case-Control Studies, Creutzfeldt-Jakob Syndrome,
genetics, Electrophoresis Gel Two-Dimensional, methods, Female,
Gene Expression, Human, Male, Middle Age, Nerve Tissue Proteins,
genetics, Peroxidases, genetics,
Spectrometry Mass Matrix-Assisted Laser Desorption-Ionization,
Support Non-U.S. Gov't},
ABSTRACT = {Using two-dimensional gel electrophoresis (2-DE) and
Western blot analysis, we were able to identify and quantify six
antioxidant proteins, peroxiredoxin (Prx) I, Prx II, Prx III, 1-Cys
Prx, putative peroxisomal antioxidant enzyme (PLP), and mitochondrial
Mn superoxide dismutase (Mn-SOD) in two individual brain regions,
cerebellum and frontal cortex of patients with sporadic
Creutzfeldt-Jacob (sCJD). Among six antioxidant proteins, 1-Cys Prx
showed significant increase (P > 0.05) in sCJD frontal cortex whereas
Prx I was decreased (P > 0.01). In cerebellum, levels of all
antioxidant proteins studied were comparable to those of controls.
Our findings provide evidence for the link between aberrant
expression of antioxidant proteins, 1-Cys Prx and Prx I and CJD
neuropathogenesis and we discuss the neuropathological meaning of
these dysregulated antioxidant proteins in sCJD brain.}
}
@ARTICLE{kubicki:hrp:2002,
AUTHOR = {Marek Kubicki and Carl-Fredrik Westin and
Stephan E Maier and Hatsuho Mamata and Melissa Frumin and
Hal Ersner-Hershfield and Ron Kikinis and Ferenc A Jolesz and
Robert McCarley and Martha E Shenton},
JOURNAL = {Harv Rev Psychiatry},
TITLE = {Diffusion tensor imaging and its application to
neuropsychiatric disorders},
YEAR = {2002},
MONTH = {Nov-Dec},
OPTNOTE = {},
NUMBER = {6},
PAGES = {324-36},
VOLUME = {10},
KEYWORDS = {Anisotropy, Brain, pathology, Diffusion, Human,
Image Processing Computer-Assisted, Magnetic Resonance Imaging,
methods, Mental Disorders, diagnosis, Nervous System Diseases,
diagnosis, Support Non-U.S. Gov't, Support U.S. Gov't Non-P.H.S.,
Support U.S. Gov't P.H.S.},
ABSTRACT = {Magnetic resonance diffusion tensor imaging (DTI) is a
new technique that can be used to visualize and measure the diffusion
of water in brain tissue; it is particularly useful for evaluating
white matter abnormalities. In this paper, we review research studies
that have applied DTI for the purpose of understanding
neuropsychiatric disorders. We begin with a discussion of the
principles involved in DTI, followed by a historical overview of
magnetic resonance diffusion-weighted imaging and DTI and a brief
description of several different methods of image acquisition and
quantitative analysis. We then review the application of this
technique to clinical populations. We include all studies published
in English from January 1996 through March 2002 on this topic,
located by searching PubMed and Medline on the key words "diffusion
tensor imaging" and "MRI." Finally, we consider potential future uses
of DTI, including fiber tracking and surgical planning and follow-up.}
}
@ARTICLE{kulldorff:ije:2002,
AUTHOR = {Martin Kulldorff},
JOURNAL = {Int J Epidemiol},
TITLE = {Geographical distribution of sporadic Creutzfeldt-Jakob
Disease in France},
YEAR = {2002},
MONTH = {April},
OPTNOTE = {},
NUMBER = {2},
PAGES = {495-6},
VOLUME = {31},
KEYWORDS = {Creutzfeldt-Jakob Syndrome, epidemiology, France,
epidemiology, Human}
}
@ARTICLE{lehmann:jsb:2002,
AUTHOR = {Sylvain Lehmann},
JOURNAL = {J Soc Biol},
TITLE = {[The prion protein]},
YEAR = {2002},
OPTMONTH = {},
OPTNOTE = {},
NUMBER = {4},
PAGES = {309-12},
VOLUME = {196},
KEYWORDS = {Animal, Cattle, English Abstract,
Glycosylphosphatidylinositols, Human, Mice, Mice Transgenic, Neurons,
metabolism, Nuclear Magnetic Resonance Biomolecular, PrPC Proteins,
chemistry, PrPSc Proteins, metabolism, Prion Diseases, genetics,
Protein Conformation, Protein Structure Tertiary, Scrapie, genetics,
Sheep, Signal Transduction, Structure-Activity Relationship},
ABSTRACT = {Transmissible spongiform encephalopathies form a group
of fatal neurodegenerative disorders represented principally by
Creutzfeldt-Jakob disease in humans, and by scrapie and bovine
spongiform encephalopathy in animals. Also called prion diseases,
these disorders have the property of being infectious, sporadic or
genetic in origin. Although the nature of the responsible agent of
these diseases is uncertain, it is clear that a protein called PrPSc
has a central role in their pathology. PrPSc is a conformational
variant of a normal protein called PrPC. PrPC is a glycoprotein
expressed by most tissues and is attached on the cell membrane by a
glycosyl-phosphatidylinositol anchor which would be consistent with
roles in cell adhesion, ligand uptake, or transmembrane signaling.
NMR studies revealed that the protein has a globular domain and a
long amino-terminal tail that contains repeated octapeptide domains
which bind metal ions with high affinities. PrPC is localized on the
cell membrane in detergent resistant microdomains and may be part of
functional complexes with other molecules. This is particularly
relevant, knowing the possible role of the molecule in signal
transduction, resistance to oxidative stress and neuronal survival.
In conclusion, it appears that the understanding of the biology of
PrP is essential for the understanding of the physiological function
of the protein as well as for its pathological conversion considering
that trafficking of this molecule governs generation of PrPSc.}
}
@ARTICLE{macleod:jn:2002,
AUTHOR = {M-A Macleod and G E Stewart and M Zeidler and R Will and
R Knight},
JOURNAL = {J Neurol},
TITLE = {Sensory features of variant Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {June},
OPTNOTE = {},
NUMBER = {6},
PAGES = {706-11},
VOLUME = {249},
KEYWORDS = {Creutzfeldt-Jakob Syndrome, complications,
Diagnosis Differential, Diagnostic Errors,
Evoked Potentials Somatosensory, physiology, Extremities,
physiopathology, Female, Human, Magnetic Resonance Imaging, Male,
Pain, etiology, Paresthesia, etiology, Pulvinar, pathology,
Referral and Consultation, Sensation Disorders, etiology, Specialism,
statistics & numerical data, Support Non-U.S. Gov't},
ABSTRACT = {OBJECTIVE: Sensory symptoms are a prominent feature of
variant Creutzfeldt-Jakob disease (vCJD), occurring at an early stage
of the illness. They are persistent and can be troublesome. Here,
they are described in detail and a possible anatomical basis is
discussed. METHODS: The first 50 cases of vCJD confirmed by the
National CJD Surveillance Unit (NCJDSU) were reviewed. Where possible
the patients and their relatives were interviewed and case notes were
examined. The presence and nature of sensory symptoms and signs were
noted. Results of investigation and types of treatment offered were
also reviewed. RESULTS: Of 50 definite cases, 64 % had persistent
sensory symptoms, 16 % had no sensory symptoms and 18 % were
uncertain. In 2 % there was insufficient information. Of the 32 with
definite symptoms, 31 % were symptomatic from the onset of the
illness. The symptoms were varied and some patients complained of
more than one type of symptom. Limb pain was described in 63 % cases.
This was the most common symptom and was often non-specific and
poorly localised, usually occurring in the lower limbs. Other
symptoms included cold feelings (25 % patients), dysaesthesia (28 %
patients), paraesthesia (31 % patients) and numbness (25 % patients).
The symptoms were lateralised in 31 % of patients. CONCLUSIONS:
Sensory symptoms are a prominent feature of vCJD, occurring in nearly
two thirds of cases. They may help distinguish variant from sporadic
CJD. They are likely to be of thalamic origin but the recognised MRI
changes in vCJD do not correlate with the presence or absence of
sensory symptoms. Neuropathological changes in the thalamus, however,
show marked astrocytosis and neuronal loss.}
}
@ARTICLE{mangin:mia:2002,
AUTHOR = {J-F Mangin and C Poupon and C Clark and Le Bihan, D and
Bihan D Le and I Bloch},
JOURNAL = {Med Image Anal},
TITLE = {Distortion correction and robust tensor estimation for
MR diffusion imaging},
YEAR = {2002},
MONTH = {September},
OPTNOTE = {},
NUMBER = {3},
PAGES = {191-8},
VOLUME = {6},
KEYWORDS = {Artifacts, Brain, anatomy & histology,
Comparative Study, Diffusion Magnetic Resonance Imaging, methods,
Human, Image Enhancement, methods, Models Statistical,
Quality Control, Reproducibility of Results,
Sensitivity and Specificity, Statistics, Stochastic Processes},
ABSTRACT = {This paper presents a new procedure to estimate the
diffusion tensor from a sequence of diffusion-weighted images. The
first step of this procedure consists of the correction of the
distortions usually induced by eddy-current related to the large
diffusion-sensitizing gradients. This correction algorithm relies on
the maximization of mutual information to estimate the three
parameters of a geometric distortion model inferred from the
acquisition principle. The second step of the procedure amounts to
replacing the standard least squares-based approach by the
Geman-McLure M-estimator, in order to reduce outlier-related
artefacts. Several experiments prove that the whole procedure highly
improves the quality of the final diffusion maps.}
}
@ARTICLE{molko:jcn:2002,
AUTHOR = {N Molko and L Cohen and J F Mangin and F Chochon and
S Lehericy and Le Bihan, D and Bihan D Le and S Dehaene},
JOURNAL = {J Cogn Neurosci},
TITLE = {Visualizing the neural bases of a disconnection syndrome
with diffusion tensor imaging},
YEAR = {2002},
MONTH = {May},
OPTNOTE = {},
NUMBER = {4},
PAGES = {629-36},
VOLUME = {14},
KEYWORDS = {Adult, Anisotropy, Brain, pathology, Brain Mapping,
Case Report, Dominance Cerebral, Dyslexia, diagnosis, Human,
Magnetic Resonance Imaging, Male, Neural Pathways, physiopathology,
Reading, Verbal Behavior},
ABSTRACT = {Disconnection syndromes are often conceptualized
exclusively within cognitive box-and-arrow diagrams unrelated to
brain anatomy. In a patient with alexia in his left visual field
resulting from a posterior callosal lesion, we illustrate how
diffusion tensor imaging can reveal the anatomical bases of a
disconnection syndrome by tracking the degeneration of neural
pathways and relating it to impaired fMRI activations and behavior.
Compared to controls, an abnormal pattern of brain activity was
observed in the patient during word reading, with a lack of
activation of the left visual word form area (VWFA) by left hemifield
words. Statistical analyses of diffusion images revealed a damaged
fiber tract linking the left ventral occipito-temporal region to its
right homolog across the lesioned area of corpus callosum and
stopping close to the areas found active in fMRI. The behavioral
disconnection syndrome could, thus, be related functionally to
abnormal fMRI activations and anatomically to the absence of a
connection between those activations. The present approach, based on
the "negative tracking" of degenerated bundles, provides new
perspectives on the understanding of human brain connections and
disconnections.}
}
@ARTICLE{murata:aajn:2002,
AUTHOR = {Takaki Murata and Yusei Shiga and Shuichi Higano and
Shoki Takahashi and Shunji Mugikura},
JOURNAL = {AJNR Am J Neuroradiol},
TITLE = {Conspicuity and evolution of lesions in
Creutzfeldt-Jakob disease at diffusion-weighted imaging},
YEAR = {2002},
MONTH = {August},
OPTNOTE = {},
NUMBER = {7},
PAGES = {1164-72},
VOLUME = {23},
KEYWORDS = {Aged, Aged 80 and over, Brain, blood supply,
Comparative Study, Creutzfeldt-Jakob Syndrome, diagnosis,
Disease Progression, Female, Follow-Up Studies, Human,
Image Processing Computer-Assisted, Magnetic Resonance Imaging,
methods, Male, Middle Age, Sensitivity and Specificity,
Severity of Illness Index, Time Factors},
ABSTRACT = {BACKGROUND AND PURPOSE: Diffusion-weighted imaging can
disclose distinct hyperintense lesions in Creutzfeldt-Jakob disease
(CJD). However, these findings and chronologic changes of CJD at
diffusion-weighted imaging have not been fully investigated. Our
purpose was to assess the diagnostic value of diffusion-weighted
imaging in depicting CJD-related lesions and in tracking the
evolution of these lesions. We also compared the sensitivity of
diffusion-weighted imaging in depicting CJD-related lesions to that
of fluid-attenuated inversion recovery (FLAIR) imaging. METHODS: We
reviewed findings in 13 patients with a diagnosis of CJD who
underwent MR imaging, including diffusion-weighted imaging. Nine
patients were initially examined within 4 months of onset of symptoms
(early stage), and eight were examined 4 months or later (late
stage). We evaluated four items: 1) distribution of lesions at
diffusion-weighted imaging, 2) conspicuity of lesions at
diffusion-weighted imaging and FLAIR imaging, 3) chronologic changes
in lesions at diffusion-weighted imaging, and 4) chronologic changes
in lesions revealed by apparent diffusion coefficient (ADC) maps.
RESULTS: Patients had striatal lesions or cerebral cortical lesions
or both. The thalamus was involved in only one patient, and the
globus pallidus was spared in all patients. The sensitivity of
diffusion-weighted imaging in depicting lesions was superior or at
least equal to that of FLAIR imaging. Hyperintense lesions at
diffusion-weighted imaging changed in extent and intensity over time.
Unlike infarction, lesional ADC decreased for 2 weeks or longer.
CONCLUSION: The progressively hyperintense changes in the striata and
cerebral cortices at diffusion-weighted imaging are considered
characteristic of CJD. Diffusion-weighted imaging may be useful for
the early diagnosis of CJD.}
}
@ARTICLE{nakada:nts:2002,
AUTHOR = {Tsutomu Nakada},
JOURNAL = {No To Shinkei},
TITLE = {[Diffusion tensor analysis: principles and applications]},
YEAR = {2002},
MONTH = {April},
OPTNOTE = {},
NUMBER = {4},
PAGES = {290-7},
VOLUME = {54},
KEYWORDS = {Brain, anatomy & histology, Diffusion, English Abstract,
Human, Image Processing Computer-Assisted,
Magnetic Resonance Imaging, methods, Sensitivity and Specificity},
ABSTRACT = {Diffusion tensor analysis represents a versatile yet
powerful application of magnetic resonance imaging (MRI). Early
applications of diffusion weighted imaging(DWI), especially those
related to ischemic brain disease were quickly overshadowed by more
advanced applications of DTA, namely, those dealing with anisotropy
analysis. Considering the array of possibilities, from neuronal tract
tracing to neuronal density imaging, DTA is rapidly becoming an
indispensable tool not only in neuroscience but also in clinical
investigations of the brain.}
}
@ARTICLE{pappas:ocna:2002,
AUTHOR = {Dennis G Jr Pappas and Joel K Cure},
JOURNAL = {Otolaryngol Clin North Am},
TITLE = {Diagnostic imaging},
YEAR = {2002},
MONTH = {April},
OPTNOTE = {},
NUMBER = {2},
PAGES = {239-53},
VOLUME = {35},
KEYWORDS = {Bone Diseases, diagnosis, Cerebellopontine Angle,
pathology, Human, Labyrinth, pathology, Magnetic Resonance Imaging,
Petrous Bone, pathology, Temporal Bone, pathology,
Tomography X-Ray Computed},
ABSTRACT = {Imaging technology continues to advance and simplify the
diagnosis of neurotologic pathology. Namely, high resolution magnetic
resonance imaging has provided detailed evaluation of the internal
auditory canal and membranous labyrinth. Conversely, the role of high
resolution magnetic resonance imaging as a screening tool remains
controversial. Functional imaging studies such as functional magnetic
resonance imaging and single photon emission computed tomography are
beginning to find significant roles in the evaluation of cochlear
implant patients. General imaging principles and imaging strategies
for specific temporal bone pathologies are also discussed.}
}
@ARTICLE{pereira:an:2002,
AUTHOR = {Edgard Pereira},
JOURNAL = {Arq Neuropsiquiatr},
TITLE = {Diffusion-weighted sequence on MRI for the diagnosis of
Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {December},
OPTNOTE = {},
NUMBER = {4},
PAGES = {906-8},
VOLUME = {60},
KEYWORDS = {Basal Ganglia, pathology, Case Report, Cerebral Cortex,
pathology, Creutzfeldt-Jakob Syndrome, diagnosis,
Diagnosis Differential, Diffusion Magnetic Resonance Imaging,
methods, Human, Male, Middle Age, Thalamus, pathology},
ABSTRACT = {Creutzfeldt-Jakob disease (CJD) is a progressive and
fatal dementing illness caused by a virus like agent called prion.
Currently, the definitive diagnosis can only be made through brain
biopsy. Given its potential transmissibility, it is paramount to have
noninvasive and reliable means to detect the disease. The present
case reports on a 63 year-old man with biopsy proven CJD, and
evaluates the dependability of diffusion-weighted MRI in this
condition, stressing the importance of this particular sequence to
its diagnosis.}
}
@ARTICLE{rabinstein:an:2002,
AUTHOR = {Alejandro A Rabinstein and Michelle L Whiteman and
Robert T Shebert},
JOURNAL = {Arch Neurol},
TITLE = {Abnormal diffusion-weighted magnetic resonance imaging
in Creutzfeldt-Jakob disease following corneal transplantations},
YEAR = {2002},
MONTH = {April},
OPTNOTE = {},
NUMBER = {4},
PAGES = {637-9},
VOLUME = {59},
KEYWORDS = {Brain, pathology, Case Report, Corneal Transplantation,
Creutzfeldt-Jakob Syndrome, diagnosis, Human,
Magnetic Resonance Imaging, methods, Male, Middle Age,
Predictive Value of Tests},
ABSTRACT = {BACKGROUND: The value of magnetic resonance imaging of
the brain in the diagnosis of iatrogenic cases of Creutzfeldt-Jakob
disease has been questioned. OBJECTIVE: To illustrate the value of
magnetic resonance imaging of the brain in the diagnosis of
iatrogenic Creutzfeldt-Jakob disease. METHODS: Case report. RESULTS:
A patient with a history of 3 corneal transplantations exhibited the
alien hand sign on initial examination. Diffusion-weighted magnetic
resonance imaging of the brain revealed prominent cortical diffusion
abnormalities. During the following months, the patient developed
rapidly progressive dementia. The diagnosis of Creutzfeldt-Jakob
disease was proven by brain biopsy. CONCLUSION: Brain magnetic
resonance imaging, particularly diffusion-weighted magnetic resonance
imaging, can be very helpful in the diagnosis of Creutzfeldt-Jakob
disease, even in suspected iatrogenic cases.}
}
@ARTICLE{ruizalzola:mia:2002,
AUTHOR = {J Ruiz-Alzola and C-F Westin and S K Warfield and
C Alberola and S Maier and R Kikinis},
JOURNAL = {Med Image Anal},
TITLE = {Nonrigid registration of 3D tensor medical data},
YEAR = {2002},
MONTH = {June},
OPTNOTE = {},
NUMBER = {2},
PAGES = {143-61},
VOLUME = {6},
KEYWORDS = {Algorithms, Brain, anatomy & histology,
Comparative Study, Computer Simulation, Diffusion, Human,
Image Processing Computer-Assisted, methods,
Imaging Three-Dimensional, methods, Magnetic Resonance Imaging,
methods, Matched-Pair Analysis, Models Anatomic, Models Neurological,
Sensitivity and Specificity, Support Non-U.S. Gov't,
Support U.S. Gov't P.H.S.},
ABSTRACT = {New medical imaging modalities offering multi-valued
data, such as phase contrast MRA and diffusion tensor MRI, require
general representations for the development of automated algorithms.
In this paper we propose a unified framework for the registration of
medical volumetric multi-valued data using local matching. The paper
extends the usual concept of similarity between two pieces of data to
be matched, commonly used with scalar (intensity) data, to the
general tensor case. Our approach to registration is based on a
multiresolution scheme, where the deformation field estimated in a
coarser level is propagated to provide an initial deformation in the
next finer one. In each level, local matching of areas with a high
degree of local structure and subsequent interpolation are performed.
Consequently, we provide an algorithm to assess the amount of
structure in generic multi-valued data by means of gradient and
correlation computations. The interpolation step is carried out by
means of the Kriging estimator, which provides a novel framework for
the interpolation of sparse vector fields in medical applications.
The feasibility of the approach is illustrated by results on
synthetic and clinical data.}
}
@ARTICLE{taber:jncn:2002,
AUTHOR = {Katherine H Taber and Pietro Cortelli and
Wolfgang Staffen and Robin A Hurley},
JOURNAL = {J Neuropsychiatry Clin Neurosci},
TITLE = {The expanding role of imaging in prion disease},
YEAR = {2002},
MONTH = {Fall},
OPTNOTE = {},
NUMBER = {4},
PAGES = {371-6},
VOLUME = {14},
KEYWORDS = {Brain, blood supply, Cerebrovascular Circulation,
physiology, Creutzfeldt-Jakob Syndrome, diagnosis,
Fludeoxyglucose F 18, diagnostic use, Human,
Magnetic Resonance Imaging, Oximes, diagnostic use,
Radiopharmaceuticals, diagnostic use, Tomography Emission-Computed,
Tomography Emission-Computed Single-Photon,
Tomography X-Ray Computed}
}
@ARTICLE{thakur:nmji:2002,
AUTHOR = {Rajeev Thakur and Yasmeen Marbaniang Vincent and
Sujata Chaturvedi},
JOURNAL = {Natl Med J India},
TITLE = {Human prion diseases},
YEAR = {2002},
MONTH = {Nov-Dec},
OPTNOTE = {},
NUMBER = {6},
PAGES = {339-45},
VOLUME = {15},
KEYWORDS = {Animal, Human, Population Surveillance, Prion Diseases,
epidemiology},
ABSTRACT = {Prion diseases is another name for a group of
'transmissible spongiform encephalopathies'. Creutzfeldt-Jakob
disease, the first prion disease described in humans, occurs in
sporadic, familial or iatrogenic form. Other transmissible spongiform
encephalopathies in humans such as familial Creutzfeldt-]akob
disease, Gerstmann-Straussler-Scheinker disease and fatal familial
Insomnia have been shown to be associated with specific prion protein
gene mutations. In 1996, a new variant of Creutzfeldt-Jakob disease
was reported in the United Kingdom among young patients with unusual
clinical features and unique neuropathological findings. This new
form could be due to transmission to humans of the agent causing
bovine spongiform encephalopathy. While examination of brain tissue
is the key to making a diagnosis, it is not always possible
antemortem. Immunological tests such as ELISA or western blot assays
along with tests for 1 4-3-3 protein in the cerebrospinal fluid
remain the main tools of diagnosis. Conventional disinfection and
sterilization practices are Ineffective for these agents. The unusual
properties of prions pose a challenge for treatment, surveillance and
control of these diseases.}
}
@ARTICLE{tribl:neuroradiology:2002,
AUTHOR = {G G Tribl and G Strasser and J Zeitlhofer and S Asenbaum and
C Jarius and P Wessely and D Prayer},
JOURNAL = {Neuroradiology},
TITLE = {Sequential MRI in a case of Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {March},
OPTNOTE = {},
NUMBER = {3},
PAGES = {223-6},
VOLUME = {44},
KEYWORDS = {Brain, pathology, Case Report, Contrast Media,
Creutzfeldt-Jakob Syndrome, diagnosis, Electroencephalography,
Gadolinium DTPA, diagnostic use, Human, Magnetic Resonance Imaging,
Magnetic Resonance Spectroscopy, diagnostic use, Male, Middle Age,
Support Non-U.S. Gov't, Time Factors},
ABSTRACT = {A 48-year-old man suddenly developed clinically and
electroencephalographically nonspecific dementia. On MRI sequences,
only diffusion-weighted images (DWI) of the cortex were unequivocally
pathological. Obvious atrophy and basal ganglia signal changes
appeared only 9 months after the onset. Brain biopsy confirmed
Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we
recommend DWI for early diagnosis of CJD.}
}
@ARTICLE{ward:neurology:2002,
AUTHOR = {H J T Ward and HJ Ward and D Everington and E A Croes and
A Alperovitch and N Delasnerie-Laupretre and I Zerr and S Poser and
van Duijn, C M and Duijn CM van},
JOURNAL = {Neurology},
TITLE = {Sporadic Creutzfeldt-Jakob disease and surgery: a
case-control study using community controls},
YEAR = {2002},
MONTH = {August},
OPTNOTE = {},
NUMBER = {4},
PAGES = {543-8},
VOLUME = {59},
KEYWORDS = {Aged, Case-Control Studies, Creutzfeldt-Jakob Syndrome,
epidemiology, Female, France, epidemiology, Germany, epidemiology,
Great Britain, epidemiology, Human, Male, Middle Age, Netherlands,
epidemiology, Odds Ratio, Registries, statistics & numerical data,
Risk Assessment, Risk Factors, Sex Distribution, Sex Factors,
Support Non-U.S. Gov't, Surgical Procedures Operative,
adverse effects},
ABSTRACT = {BACKGROUND: The cause of sporadic Creutzfeldt-Jakob
disease (CJD) is unknown. Previous studies found a link with a
history of surgery but had methodologic problems. OBJECTIVE: To help
elucidate medical and associated risk factors for sporadic CJD as
part of the 1993 to 1995 European Union collaborative studies of CJD.
METHODS: Medical and associated risk factors from 326 patients with
sporadic CJD, taken from population-based studies performed between
1993 and 1995 in France, Germany, the Netherlands, and the UK, were
compared with 326 community controls recruited by telephone in 2000.
RESULTS: A history of surgery was significantly associated with the
risk of sporadic CJD (odds ratio [OR]: 1.8; 95% CI: 1.2 to 2.6),
which was not dependent on the number of surgical procedures, and was
stronger in females (OR: 2.5; 95% CI: 1.5 to 4.0). Gynecologic (OR:
1.5; 95% CI: 1.0 to 2.3) and "other" operations (any operation other
than neurologic, eye, ear, gallbladder, gastrointestinal, and
gynecologic operations, tonsillectomy, and appendectomy) (OR: 1.5;
95% CI: 1.1 to 2.1) were associated with risk of CJD. Tonsillectomy
(OR: 0.3; 95% CI: 0.2 to 0.5) and appendectomy (OR: 0.6; 95% CI: 0.4
to 0.8) were observed less frequently in cases. An increased risk was
also found with a history of ear piercing in females (OR: 1.6; 95%
CI: 1.1 to 2.5) and psychiatric visit(s) (OR: 2.6; 95% CI: 1.5 to
4.3). CONCLUSIONS: These results support the hypothesis that cases of
sporadic CJD may result from hitherto unrecognized surgical
contamination events. However, because of the limits of the study
design, the rarity of the disease, and the potential for bias, the
results should be interpreted with caution.}
}
@ARTICLE{westin:mia:2002,
AUTHOR = {C-F Westin and S E Maier and H Mamata and A Nabavi and
F A Jolesz and R Kikinis},
JOURNAL = {Med Image Anal},
TITLE = {Processing and visualization for diffusion tensor MRI},
YEAR = {2002},
MONTH = {June},
OPTNOTE = {},
NUMBER = {2},
PAGES = {93-108},
VOLUME = {6},
KEYWORDS = {Brain, anatomy & histology, Data Display, Human,
Image Processing Computer-Assisted, methods,
Magnetic Resonance Imaging, methods, Models Theoretical,
Sensitivity and Specificity, Support Non-U.S. Gov't,
Support U.S. Gov't P.H.S.},
ABSTRACT = {This paper presents processing and visualization
techniques for Diffusion Tensor Magnetic Resonance Imaging (DT-MRI).
In DT-MRI, each voxel is assigned a tensor that describes local water
diffusion. The geometric nature of diffusion tensors enables us to
quantitatively characterize the local structure in tissues such as
bone, muscle, and white matter of the brain. This makes DT-MRI an
interesting modality for image analysis. In this paper we present a
novel analytical solution to the Stejskal-Tanner diffusion equation
system whereby a dual tensor basis, derived from the diffusion
sensitizing gradient configuration, eliminates the need to solve this
equation for each voxel. We further describe decomposition of the
diffusion tensor based on its symmetrical properties, which in turn
describe the geometry of the diffusion ellipsoid. A simple anisotropy
measure follows naturally from this analysis. We describe how the
geometry or shape of the tensor can be visualized using a coloring
scheme based on the derived shape measures. In addition, we
demonstrate that human brain tensor data when filtered can
effectively describe macrostructural diffusion, which is important in
the assessment of fiber-tract organization. We also describe how
white matter pathways can be monitored with the methods introduced in
this paper. DT-MRI tractography is useful for demonstrating neural
connectivity (in vivo) in healthy and diseased brain tissue.}
}
@ARTICLE{yoo:nl:2002,
AUTHOR = {Byong Chul Yoo and Kurt Krapfenbauer and Nigel Cairns and
Girma Belay and Michal Bajo and Gert Lubec},
JOURNAL = {Neurosci Lett},
TITLE = {Overexpressed protein disulfide isomerase in brains of
patients with sporadic Creutzfeldt-Jakob disease},
YEAR = {2002},
MONTH = {December},
OPTNOTE = {},
NUMBER = {3},
PAGES = {196-200},
VOLUME = {334},
KEYWORDS = {Aged, Alzheimer Disease, enzymology, Bacterial Proteins,
analysis, Blotting Western, methods, Cerebellum, enzymology,
Comparative Study, Creutzfeldt-Jakob Syndrome, enzymology,
Down Syndrome, enzymology, Electrophoresis Gel Two-Dimensional,
methods, Female, Glial Fibrillary Acidic Protein, chemistry, Human,
Male, Middle Age, Peptide Mapping, methods,
Phosphoprotein Phosphatase, analysis, Phosphopyruvate Hydratase,
chemistry, PrPSc Proteins, analysis, Protein Conformation,
Protein Disulfide-Isomerase, metabolism,
Spectrometry Mass Matrix-Assisted Laser Desorption-Ionization,
Statistics Nonparametric},
ABSTRACT = {Earlier studies have failed to detect covalent
modifications in beta-sheet-rich scrapie isoform prion protein
(PrP(Sc)) and have concluded that the conversion of alpha-helix-rich
cellular form prion protein (PrP(C)) to PrP(Sc) represents purely
conformational transition not involving chemical reactions. However,
recent studies have shown that the intradisulfide bond of PrP(C) can
play an important role for instability and conformational change to
PrP(Sc). Interestingly, we found overexpressed protein disufide
isomerase (PDI) in brains of sporadic Creutzfeldt-Jakob disease
(sCJD, human prion disease) patients using two dimensional
electrophoresis and Western blot analysis but not in other
neurodegenerative disorders as Down Syndrome and Alzheimer's disease.
However, proteinase K digestion and plasminogen binding assay of
brain homogenates incubated with PDI suggest that PDI has no effect
on either proteinase resistance or conformational change of PrP.
Overexpression of PDI protein in sCJD brain may simply reflect a
cellular defense response against the altered prion protein.}
}
@ARTICLE{zerr:dmw:2002,
AUTHOR = {I Zerr and B Mollenhauer and Ch Werner and S Poser},
JOURNAL = {Dtsch Med Wochenschr},
TITLE = {[Early and differential diagnosis of Creutzfeldt-Jakob
disease]},
YEAR = {2002},
MONTH = {February},
OPTNOTE = {},
NUMBER = {7},
PAGES = {323-7},
VOLUME = {127},
KEYWORDS = {Adult, Comparative Study, Creutzfeldt-Jakob Syndrome,
cerebrospinal fluid, Diagnosis Differential, Electroencephalography,
Human, Magnetic Resonance Imaging, Male, Middle Age,
Sensitivity and Specificity, Time Factors, Tomography X-Ray Computed}
}
@ARTICLE{collie:jbrbtr:2001,
AUTHOR = {D A Collie},
JOURNAL = {JBR-BTR},
TITLE = {The role of MRI in the diagnosis of sporadic and variant
Creutzfeldt-Jakob disease},
YEAR = {2001},
MONTH = {August},
OPTNOTE = {},
NUMBER = {4},
PAGES = {143-6},
VOLUME = {84},
KEYWORDS = {Creutzfeldt-Jakob Syndrome, pathology, Human,
Magnetic Resonance Imaging},
ABSTRACT = {Creutzfeldt-Jakob disease (CJD) is a rare but important
fatal, dementing illness. A number of types of CJD are identified,
each with distinct clinical features. Characteristic MRI changes have
been described recently. Sporadic CJD, the commonest type, is found
worldwide, and causes hyperintensity of the putamen and caudate
nuclei. In the recently described variant CJD, which affects younger
patients and has been linked to Bovine Spongiform Encephalopathy
(BSE) in cattle, a highly characteristic finding of bilateral
hyperintensity of the pulvinar nuclei is seen. The MRI features of
CJD are reviewed in this article.}
}
@ARTICLE{collie:cr:2001,
AUTHOR = {D A Collie and R J Sellar and M Zeidler and
A C Colchester and R Knight and R G Will},
JOURNAL = {Clin Radiol},
TITLE = {MRI of Creutzfeldt-Jakob disease: imaging features and
recommended MRI protocol},
YEAR = {2001},
MONTH = {September},
OPTNOTE = {},
NUMBER = {9},
PAGES = {726-39},
VOLUME = {56},
KEYWORDS = {Clinical Protocols, Creutzfeldt-Jakob Syndrome,
classification, Diagnosis Differential, Human,
Magnetic Resonance Imaging, methods, Pulvinar, pathology,
Support Non-U.S. Gov't, Thalamus, pathology},
ABSTRACT = {Creutzfeldt-Jakob Disease (CJD) is a rare, progressive
and invariably fatal neurodegenerative disease characterized by
specific histopathological features. Of the four subtypes of CJD
described, the commonest is sporadic CJD (sCJD). More recently, a new
clinically distinct form of the disease affecting younger patients,
known as variant CJD (vCJD), has been identified, and this has been
causally linked to the bovine spongiform encephalopathy (BSE) agent
in cattle. Characteristic appearances on magnetic resonance imaging
(MRI) have been identified in several forms of CJD; sCJD may be
associated with high signal changes in the putamen and caudate head
and vCJD is usually associated with hyperintensity of the pulvinar
(posterior nuclei) of the thalamus. These appearances and other
imaging features are described in this article. Using appropriate
clinical and radiological criteria and tailored imaging protocols,
MRI plays an important part in the in vivodiagnosis of this disease.}
}
@ARTICLE{dervaux:bjp:2001,
AUTHOR = {A Dervaux and S Vicart and F Lopes and Le Borgne, M H and
Borgne MH Le},
JOURNAL = {Br J Psychiatry},
TITLE = {Psychiatric features of vCJD similar in France and UK},
YEAR = {2001},
MONTH = {March},
OPTNOTE = {},
OPTNUMBER = {},
PAGES = {276},
VOLUME = {178},
KEYWORDS = {Adult, Case Report, Creutzfeldt-Jakob Syndrome,
diagnosis, Depressive Disorder, etiology, Female, France,
Great Britain, Human}
}
@ARTICLE{kim:kjr:2001,
AUTHOR = {H C Kim and K H Chang and I C Song and S H Lee and
B J Kwon and M H Han and S Y Kim},
JOURNAL = {Korean J Radiol},
TITLE = {Diffusion-weighted MR imaging in biopsy-proven
Creutzfeldt-Jakob disease},
YEAR = {2001},
MONTH = {Oct-Dec},
OPTNOTE = {},
NUMBER = {4},
PAGES = {192-6},
VOLUME = {2},
KEYWORDS = {Adult, Aged, Biopsy, Comparative Study,
Creutzfeldt-Jakob Syndrome, diagnosis, Female, Human,
Magnetic Resonance Imaging, methods, Male, Middle Age,
Retrospective Studies, Sensitivity and Specificity,
Support Non-U.S. Gov't},
ABSTRACT = {OBJECTIVE: To compare conventional and
diffusion-weighted MR imaging in terms of their depiction of the
abnormalities occurring in Creutzfeldt-Jakob disease. MATERIALS AND
METHODS: We retrospectively analyzed the findings of conventional
(T2-weighted and fluid-attenuated inversion recovery) and
diffusion-weighted MR imaging in four patients with biopsy-proven
Creutzfeldt-Jakob disease. The signal intensity of the lesion was
classified by visual assessment as markedly high, slightly high, or
isointense, relative to normal brain parenchyma. RESULTS: Both
conventional and diffusion-weighted MR images demonstrated bilateral
high signal intensity in the basal ganglia in all four patients.
Cortical lesions were observed on diffusion-weighted MR images in all
four, and on fluid-attenuated inversion recovery MR images in one,
but in no patient on T2-weighted images. Conventional MR images
showed slightly high signal intensity in all lesions, while
diffusion-weighted images showed markedly high signal intensity in
most. CONCLUSION: Diffusion-weighted MR imaging is more sensitive
than its conventional counterpart in the depiction of
Creutzfeldt-Jakob disease, and permits better detection of the lesion
in both the cerebral cortices and basal ganglia.}
}
@ARTICLE{konagaya:nts:2001,
AUTHOR = {M Konagaya and M Sakai and K Asakura and Y Matsuoka and
Y Hashizume},
JOURNAL = {No To Shinkei},
TITLE = {[T2-weighted MRI and pathological findings in the
cerebral hemisphere of panencephalitic Creutzfeldt-Jakob disease]},
YEAR = {2001},
MONTH = {April},
OPTNOTE = {},
NUMBER = {4},
PAGES = {398-9},
VOLUME = {53},
KEYWORDS = {Brain, pathology, Case Report,
Creutzfeldt-Jakob Syndrome, diagnosis, Encephalitis, diagnosis,
Female, Human, Magnetic Resonance Imaging, Middle Age}
}
@ARTICLE{koppinen:avj:2001,
AUTHOR = {J Koppinen},
JOURNAL = {Aust Vet J},
TITLE = {The French reflect on BSE},
YEAR = {2001},
MONTH = {July},
OPTNOTE = {},
NUMBER = {7},
PAGES = {452},
VOLUME = {79},
KEYWORDS = {Animal, Animal Feed, Cattle, Creutzfeldt-Jakob Syndrome,
prevention & control, Encephalopathy Bovine Spongiform,
prevention & control, France, Human}
}
@ARTICLE{kraft:vcnaep:2001,
AUTHOR = {S L Kraft and P Gavin},
JOURNAL = {Vet Clin North Am Equine Pract},
TITLE = {Physical principles and technical considerations for
equine computed tomography and magnetic resonance imaging},
YEAR = {2001},
MONTH = {April},
OPTNOTE = {},
NUMBER = {1},
PAGES = {115-30},
VOLUME = {17},
KEYWORDS = {Animal, Horse Diseases, diagnosis, Horses,
Magnetic Resonance Imaging, methods, Tomography X-Ray Computed,
methods},
ABSTRACT = {This article discusses how cross-sectional imaging
methods such as computed tomography and magnetic resonance imaging
can provide unique and diagnostically important information in
situations where radiography or diagnostic ultrasound have been
unrewarding.}
}
@ARTICLE{lebihan:jmri:2001,
AUTHOR = {Le Bihan, D and Bihan D Le and J F Mangin and C Poupon and
C A Clark and S Pappata and N Molko and H Chabriat},
JOURNAL = {J Magn Reson Imaging},
TITLE = {Diffusion tensor imaging: concepts and applications},
YEAR = {2001},
MONTH = {April},
OPTNOTE = {},
NUMBER = {4},
PAGES = {534-46},
VOLUME = {13},
KEYWORDS = {Brain Diseases, diagnosis, Diffusion, Human,
Image Processing Computer-Assisted, Magnetic Resonance Imaging,
methods},
ABSTRACT = {The success of diffusion magnetic resonance imaging
(MRI) is deeply rooted in the powerful concept that during their
random, diffusion-driven displacements molecules probe tissue
structure at a microscopic scale well beyond the usual image
resolution. As diffusion is truly a three-dimensional process,
molecular mobility in tissues may be anisotropic, as in brain white
matter. With diffusion tensor imaging (DTI), diffusion anisotropy
effects can be fully extracted, characterized, and exploited,
providing even more exquisite details on tissue microstructure. The
most advanced application is certainly that of fiber tracking in the
brain, which, in combination with functional MRI, might open a window
on the important issue of connectivity. DTI has also been used to
demonstrate subtle abnormalities in a variety of diseases (including
stroke, multiple sclerosis, dyslexia, and schizophrenia) and is
currently becoming part of many routine clinical protocols. The aim
of this article is to review the concepts behind DTI and to present
potential applications.}
}
@ARTICLE{mathews:cnm:2001,
AUTHOR = {D Mathews and D H Unwin},
JOURNAL = {Clin Nucl Med},
TITLE = {Quantitative cerebral blood flow imaging in a patient
with the Heidenhain variant of Creutzfeldt-Jakob disease},
YEAR = {2001},
MONTH = {September},
OPTNOTE = {},
NUMBER = {9},
PAGES = {770-3},
VOLUME = {26},
KEYWORDS = {Acetazolamide, pharmacology, Brain, pathology,
Case Report, Cerebrovascular Circulation, drug effects,
Creutzfeldt-Jakob Syndrome, diagnosis, Human,
Magnetic Resonance Imaging, Male, Middle Age,
Tomography Emission-Computed Single-Photon, Xenon Radioisotopes,
diagnostic use},
ABSTRACT = {PURPOSE: This report describes quantitative SPECT
cerebral blood flow (CBF) changes in a patient with the confirmed
Heidenhain variant of Creutzfeldt-Jakob disease. MATERIALS AND
METHODS: A 60-year-old man reported visual disturbances, including
left hemianopsia. An electroencephalogram, magnetic resonance
imaging, cerebral arteriogram, and quantitative SPECT CBF imaging
with Xe-133, with and without acetazolamide, were performed. After
the patient's death, an autopsy was performed. RESULTS: The
electroencephalographic findings were nonspecific, those of magnetic
resonance imaging were normal, and the cerebral arteriogram showed
minimal atherosclerosis. However, the SPECT scan revealed marked
depression of perfusion in the parietal and occipital cortices that
did not change with the administration of acetazolamide. Autopsy
results were consistent with CJD. CONCLUSIONS: Although there is no
consistent pattern of CBF changes that is characteristic of
Creutzfeldt-Jakob disease, the Heidenhain variant of that disease may
be an exception. Clinical symptoms include profound changes in
vision, and the pattern of CBF changes in the patient described here
included depression of perfusion in areas of primary and associated
visual cortex. Also of interest is the finding that although other
imaging did not reveal an abnormality, the CBF changes were marked.
In addition, vasoreactivity is poor in response to acetazolamide, a
finding that occurs only late in other neuronal degenerative
conditions such as Alzheimer's disease.}
}
@ARTICLE{matoba:jcat:2001,
AUTHOR = {M Matoba and H Tonami and H Miyaji and H Yokota and
I Yamamoto},
JOURNAL = {J Comput Assist Tomogr},
TITLE = {Creutzfeldt-Jakob disease: serial changes on
diffusion-weighted MRI},
YEAR = {2001},
MONTH = {Mar-Apr},
OPTNOTE = {},
NUMBER = {2},
PAGES = {274-7},
VOLUME = {25},
KEYWORDS = {Aged, Brain, pathology, Case Report,
Creutzfeldt-Jakob Syndrome, diagnosis, Female, Human,
Magnetic Resonance Imaging},
ABSTRACT = {We present serial changes on diffusion-weighted MRI
(DWI) in a patient with Creutzfeldt-Jakob disease (CJD). DWI revealed
serial changes of abnormal hyperintense lesions that had become more
extensive and conspicuous with progression of neurologic findings,
more sensitively than conventional MRI. In the late stage,
disappearance of abnormal hyperintense lesions on DWI was observed.
DWI proved to be particularly useful for monitoring the progression
of CJD.}
}
@ARTICLE{poon:neuroradiology:2001,
AUTHOR = {M A Poon and S Stuckey and E Storey},
JOURNAL = {Neuroradiology},
TITLE = {MRI evidence of cerebellar and hippocampal involvement
in Creutzfeldt-Jakob disease},
YEAR = {2001},
MONTH = {September},
OPTNOTE = {},
NUMBER = {9},
PAGES = {746-9},
VOLUME = {43},
KEYWORDS = {Brain, pathology, Case Report, Cerebellum, pathology,
Creutzfeldt-Jakob Syndrome, diagnosis, Diagnosis Differential,
Female, Hippocampus, pathology, Human, Magnetic Resonance Imaging,
Magnetic Resonance Spectroscopy, diagnostic use, Middle Age},
ABSTRACT = {We report a 51-year-old woman with the
Brownell-Oppenheimer (cerebellar) variant of Creutzfeldt-Jakob
disease (CJD). She had the typical findings of bilateral basal
ganglion changes on MRI, as well as changes in the cerebellum and
hippocampus. This case adds further information to the known imaging
characteristics of CJD.}
}
@ARTICLE{poupon:mia:2001,
AUTHOR = {C Poupon and J Mangin and C A Clark and V Frouin and
J Regis and Le Bihan, D and Bihan D Le and I Bloch},
JOURNAL = {Med Image Anal},
TITLE = {Towards inference of human brain connectivity from MR
diffusion tensor data},
YEAR = {2001},
MONTH = {March},
OPTNOTE = {},
NUMBER = {1},
PAGES = {1-15},
VOLUME = {5},
KEYWORDS = {Axons, physiology, Brain Mapping, Diffusion, Human,
Image Processing Computer-Assisted, Magnetic Resonance Imaging,
methods, Mathematics, Nerve Fibers, physiology},
ABSTRACT = {This paper describes a method to infer the connectivity
induced by white matter fibers in the living human brain. This method
stems from magnetic resonance tensor imaging (DTI), a technique which
gives access to fiber orientations. Given typical DTI spatial
resolution, connectivity is addressed at the level of fascicles made
up by a bunch of parallel fibers. We propose first an algorithm
dedicated to fascicle tracking in a direction map inferred from
diffusion data. This algorithm takes into account fan-shaped fascicle
forks usual in actual white matter organization. Then, we propose a
method of inferring a regularized direction map from diffusion data
in order to improve the robustness of the tracking. The
regularization stems from an analogy between white matter
organization and spaghetti plates. Finally, we propose a study of the
tracking behavior according to the weight given to the regularization
and some examples of the tracking results with in vivo human brain
data.}
}
@ARTICLE{smart:aajr:2001,
AUTHOR = {J M Smart and A Wood},
JOURNAL = {AJR Am J Roentgenol},
TITLE = {Value of fluid-attenuated inversion recovery MR imaging
in an unusual case of sporadic Creutzfeldt-Jakob disease},
YEAR = {2001},
MONTH = {October},
OPTNOTE = {},
NUMBER = {4},
PAGES = {948-9},
VOLUME = {177},
KEYWORDS = {Case Report, Creutzfeldt-Jakob Syndrome, pathology,
Human, Magnetic Resonance Imaging, methods, Male, Middle Age}
}
@ARTICLE{thompson:bba:2001,
AUTHOR = {A J Thompson and K J Barnham and R S Norton and
C J Barrow},
JOURNAL = {Biochim Biophys Acta},
TITLE = {The Val-210-Ile pathogenic Creutzfeldt-Jakob disease
mutation increases both the helical and aggregation propensities of a
sequence corresponding to helix-3 of PrP(C)},
YEAR = {2001},
MONTH = {January},
OPTNOTE = {},
NUMBER = {1-2},
PAGES = {242-54},
VOLUME = {1544},
KEYWORDS = {Amino Acid Sequence, Circular Dichroism,
Creutzfeldt-Jakob Syndrome, genetics, Isoleucine, genetics,
Molecular Sequence Data, Mutation,
Nuclear Magnetic Resonance Biomolecular, Phosphoprotein Phosphatase,
chemistry, Protein Structure Secondary, Support Non-U.S. Gov't,
Valine, genetics},
ABSTRACT = {A peptide corresponding to the third helical region
within the PrP(C) protein, from residues 198 to 218 (helix-3), was
synthesised with and without the familial 210-Val to Ile
Creutzfeldt-Jakob disease mutation. The NMR structure of PrP(C)
predicts no global variation in stability for this mutation,
indicating that local sequence rather than global structural factors
are involved in the pathological effects of this mutation. 1H NMR
analysis of peptides with and without this mutation indicated that it
had no significant effect on local helical structure. Temperature
denaturation studies monitored by CD showed that the mutation
increased the helical content within this region (helical
propensity), but did not stabilise the helix toward denaturation
(helical stability). Aggregation data indicated that, in addition to
increasing helical propensity, this mutation increased the
aggregation propensity of this sequence. CD and NMR data indicate
that helical interactions, stabilised by the Val-210-Ile mutation,
may precede the formation of beta-sheet aggregates in this peptide
sequence. Therefore, this pathological mutation probably does not
facilitate PrP(C) to PrP(Sc) conversion by directly destabilising the
helical structure of PrP(C), but may preferentially stabilise PrP(Sc)
by facilitating beta-sheet formation within this sequence region of
PrP. In addition, helical interactions between helix-3 in two or more
PrP(C) molecules may promote conversion to PrP(Sc).}
}
@ARTICLE{zeidler:neurology:2001,
AUTHOR = {M Zeidler and D A Collie and M A Macleod and R J Sellar and
R Knight},
JOURNAL = {Neurology},
TITLE = {FLAIR MRI in sporadic Creutzfeldt-Jakob disease},
YEAR = {2001},
MONTH = {January},
OPTNOTE = {},
NUMBER = {2},
PAGES = {282},
VOLUME = {56},
KEYWORDS = {Brain, pathology, Creutzfeldt-Jakob Syndrome, pathology,
Human, Magnetic Resonance Imaging}
}
@ARTICLE{collins:jcn:2000,
AUTHOR = {S Collins and A Boyd and A Fletcher and M F Gonzales and
C A McLean and C L Masters},
JOURNAL = {J Clin Neurosci},
TITLE = {Recent advances in the pre-mortem diagnosis of
Creutzfeldt-Jakob disease},
YEAR = {2000},
MONTH = {May},
OPTNOTE = {},
NUMBER = {3},
PAGES = {195-202},
VOLUME = {7},
KEYWORDS = {Biological Markers, cerebrospinal fluid, Biopsy, Brain,
pathology, Creutzfeldt-Jakob, Diagnosis Differential,
Electroencephalography, Human, Magnetic Resonance Imaging, Prions,
cerebrospinal fluid, Support Non-U.S. Gov't,
Tomography Emission-Computed,
Tomography Emission-Computed Single-Photon, Tonsil, pathology},
ABSTRACT = {Included in the spectrum of human transmissible
spongiform encephalopathies are Creutzfeldt-Jakob disease (CJD) and
the new variant form (vCJD), Gerstmann-Straussler-Scheinker syndrome,
fatal familial insomnia, kuru and various less distinct
neuropsychiatric disorders. Progress in our understanding of this
group of disorders continues at a prodigious rate, although important
vexing practical issues persist. The definitive confirmation of
symptomatic prion disease still requires pathological examination,
most reliably performed post-mortem. However, paralleling the recent
advances in the molecular biological understanding of normal prion
protein (PrP(c)) function and the pathophysiology of prion diseases,
there have been worthwhile developments in the pre-mortem diagnosis
of CJD. Efforts to develop less invasive but very reliable
ante-mortem diagnostic tests have received an additional impetus
because of the potential epidemic of vCJD. Historically, the
ancillary investigation of most merit has been the EEG, whereas the
recent advances have encompassed a broader range of technologies,
including both magnetic resonance and radioisotopic neuroimaging, and
immunoassays for a range of non-specific marker proteins in both CSF,
and less commonly, blood. However, given the recent refinement of
sophisticated immunoassays, it is envisaged that the pathognomonic,
protease-resistant, disease-associated isoforms of the prion protein
(PrPres) may soon be directly detectable in the blood and tissues of
patients manifesting or incubating a spongiform encephalopathy.}
}
@ARTICLE{dormont:es:2000,
AUTHOR = {D Dormont},
JOURNAL = {Euro Surveill},
TITLE = {New variant of Creutzfeldt Jakob disease},
YEAR = {2000},
MONTH = {September},
OPTNOTE = {},
NUMBER = {9},
PAGES = {95-97},
VOLUME = {5},
ABSTRACT = {Work on experimental pathology carried out for over 10
years indicates that the biological properties of the BSE agent,
responsible for the new variant of Creutzfeldt-Jakob disease (vCJD),
are particular. In contrast to the situation observed in the other}
}
@ARTICLE{molloy:aajr:2000,
AUTHOR = {S Molloy and R O'Laoide and F Brett and M Farrell},
JOURNAL = {AJR Am J Roentgenol},
TITLE = {The Pulvinar sign in variant Creutzfeldt-Jakob disease},
YEAR = {2000},
MONTH = {August},
OPTNOTE = {},
NUMBER = {2},
PAGES = {555-6},
VOLUME = {175},
KEYWORDS = {Adult, Case Report, Creutzfeldt-Jakob Syndrome,
pathology, Female, Human, Magnetic Resonance Imaging, Pulvinar,
pathology}
}
@ARTICLE{oppenheim:lancet:2000,
AUTHOR = {C Oppenheim and J P Brandel and J J Hauw and J P Deslys and
B Fontaine},
JOURNAL = {Lancet},
TITLE = {MRI and the second French case of vCJD},
YEAR = {2000},
MONTH = {July},
OPTNOTE = {},
NUMBER = {9225},
PAGES = {253-4},
VOLUME = {356},
KEYWORDS = {Adult, Brain Diseases, diagnosis, Case Report,
Caudate Nucleus, pathology, Corpus Striatum, pathology,
Creutzfeldt-Jakob Syndrome, diagnosis, Fatal Outcome, Female, France,
Human, Image Processing Computer-Assisted, methods,
Magnetic Resonance Imaging, methods, Pulvinar, pathology, Putamen,
pathology}
}
@ARTICLE{poupon:neuroimage:2000,
AUTHOR = {C Poupon and C A Clark and V Frouin and J Regis and
I Bloch and Le Bihan, D and Bihan D Le and J Mangin},
JOURNAL = {Neuroimage},
TITLE = {Regularization of diffusion-based direction maps for the
tracking of brain white matter fascicles},
YEAR = {2000},
MONTH = {August},
OPTNOTE = {},
NUMBER = {2},
PAGES = {184-95},
VOLUME = {12},
KEYWORDS = {Algorithms, Bayes Theorem, Brain, anatomy & histology,
Brain Mapping, methods, Diffusion, Human,
Image Processing Computer-Assisted, Magnetic Resonance Imaging,
statistics & numerical data, Models Statistical},
ABSTRACT = {Magnetic resonance diffusion tensor imaging (DTI)
provides information about fiber local directions in brain white
matter. This paper addresses inference of the connectivity induced by
fascicles made up of numerous fibers from such diffusion data. The
usual fascicle tracking idea, which consists of following locally the
direction of highest diffusion, is prone to erroneous forks because
of problems induced by fiber crossing. In this paper, this difficulty
is partly overcomed by the use of a priori knowledge of the low
curvature of most of the fascicles. This knowledge is embedded in a
model of the bending energy of a spaghetti plate representation of
the white matter used to compute a regularized fascicle direction
map. A new tracking algorithm is then proposed to highlight putative
fascicle trajectories from this direction map. This algorithm takes
into account potential fan shaped junctions between fascicles. A
study of the tracking behavior according to the influence given to
the a priori knowledge is proposed and concrete tracking results
obtained with in vivo human brain data are illustrated. These results
include putative trajectories of some pyramidal, commissural, and
various association fibers.}
}
@ARTICLE{urbach:neuroreport:2000,
AUTHOR = {H Urbach},
JOURNAL = {Neuroreport},
TITLE = {Creutzfeldt-Jakob disease: analysis of the MRI signal},
YEAR = {2000},
MONTH = {November},
OPTNOTE = {},
NUMBER = {17},
OPTPAGES = {},
VOLUME = {11},
KEYWORDS = {Brain, pathology, Creutzfeldt-Jakob Syndrome, pathology,
Gliosis, pathology, Human, Magnetic Resonance Imaging}
}
@ARTICLE{cecil:ncna:1998,
AUTHOR = {K M Cecil and R E Lenkinski},
JOURNAL = {Neuroimaging Clin N Am},
TITLE = {Proton MR spectroscopy in inflammatory and infectious
brain disorders},
YEAR = {1998},
MONTH = {November},
OPTNOTE = {},
NUMBER = {4},
PAGES = {863-80},
VOLUME = {8},
KEYWORDS = {AIDS Dementia Complex, diagnosis, Brain Abscess,
diagnosis, Brain Diseases, diagnosis, Creutzfeldt-Jakob Syndrome,
diagnosis, Herpes Simplex, diagnosis, Human,
Magnetic Resonance Spectroscopy, Support U.S. Gov't P.H.S.,
Tuberculoma, diagnosis},
ABSTRACT = {This article reviews the proton MR spectroscopy
literature regarding brain infarction and inflammatory diseases. We
examine the salient findings reported for bacterial abscesses,
intracranial tuberculomas, Creutzfeldt-Jakob disease, herpes simplex
encephalitis and HIV. These processes demonstrate specific metabolic
profiles which may be useful in differential diagnosis. The results
reported in the literature support the view that MR spectroscopy can
be employed in longitudinal studies to monitor the response to
therapy and therefore may lead to individual optimized treatment
effectiveness.}
}
@ARTICLE{peled:br:1998,
AUTHOR = {S Peled and H Gudbjartsson and C F Westin and R Kikinis and
F A Jolesz},
JOURNAL = {Brain Res},
TITLE = {Magnetic resonance imaging shows orientation and
asymmetry of white matter fiber tracts},
YEAR = {1998},
MONTH = {January},
OPTNOTE = {},
NUMBER = {1},
PAGES = {27-33},
VOLUME = {780},
KEYWORDS = {Anisotropy, Brain Mapping, methods, Diffusion, Female,
Human, In Vitro, Laterality, physiology, Linear Models,
Magnetic Resonance Imaging, Male, Nerve Fibers, pathology,
Neural Pathways, physiology, Reference Values,
Support Non-U.S. Gov't, Support U.S. Gov't P.H.S.},
ABSTRACT = {Apparent diffusion tensor maps of the human brain were
acquired with a magnetic resonance imaging sequence (Gudbjartsson,
H., Maier, S.E., Mulkern, R.V., M6rocz, I.A., Patz, S., Jolesz, F.A.,
Magn. Reson. Med. 36 (1996) 509-519). It was shown that the geometric
nature of the apparent diffusion tensors can quantitatively
characterize the tissue structure. Display of the orientation and
directional uniformity of the water diffusion in the brain
demonstrated most of the known major anatomical constituents of human
white matter. A comparison of corresponding anatomic regions in the
white matter of both hemispheres in 24 healthy volunteers revealed
that fiber tracts within the anterior limb of the internal capsule
have a significantly higher (P < 0.01) measure of alignment in the
right hemisphere. This method offers a unique tool for the in vivo
demonstration of neural connectivity in healthy and diseased brain.}
}
@ARTICLE{mooyaart:ntt:1993,
AUTHOR = {E L Mooyaart},
JOURNAL = {Ned Tijdschr Tandheelkd},
TITLE = {[Magnetic resonance imaging. Principles and applications
in the head and neck area]},
YEAR = {1993},
MONTH = {June},
OPTNOTE = {},
NUMBER = {6},
PAGES = {291-3},
VOLUME = {100},
KEYWORDS = {English Abstract, Head, pathology, Human,
Magnetic Resonance Imaging, methods, Neck, pathology},
ABSTRACT = {Magnetic Resonance Imaging is one of the most recently
developed imaging techniques of the human body. In this review the
physical principles and applications for oral surgeons are discussed.}
}
@MISC{dorozynski:2000,
AUTHOR = {A Dorozynski},
OPTHOWPUBLISHED = {},
MONTH = {November},
OPTNOTE = {},
TITLE = {France prepares for more cases of vCJD},
YEAR = {0},
JOURNAL = {BMJ},
KEYWORDS = {Creutzfeldt-Jakob Syndrome, epidemiology,
Disease Outbreaks, France, epidemiology, Human},
NUMBER = {7271},
PAGES = {1241},
VOLUME = {321 YEAR = 2000}
}
@MISC{sellars:2002,
AUTHOR = {Robin J Sellars and Donald A Collie and Robert J Will},
OPTHOWPUBLISHED = {},
MONTH = {August},
OPTNOTE = {},
TITLE = {Progress in understanding Creutzfeldt-Jakob disease},
YEAR = {0},
JOURNAL = {AJNR Am J Neuroradiol},
KEYWORDS = {Brain, blood supply, Creutzfeldt-Jakob Syndrome,
diagnosis, Disease Progression, Human, Magnetic Resonance Imaging,
Sensitivity and Specificity, Severity of Illness Index},
NUMBER = {7},
PAGES = {1070-2},
VOLUME = {23 YEAR = 2002}
}