-
Annick Alperovitch and Robert G Will.
Predicting the size of the vCJD epidemic in France.
C R Acad Sci III,
325(1):33-6,
January 2002.
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{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.}
}
-
M Beekes and R Kurth.
[BSE and Creutzfeldt-Jakob disease. Implication on health politics in Germany and Europe].
Dtsch Med Wochenschr,
127(7):335-40,
February 2002.
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{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}
}
-
Lejla Begic,
Adaleta Mulaomerovic,
Selma Berbic,
and Zlata Zigic.
[New findings on the diagnosis and therapy of prion diseases].
Med Arh,
56(5-6):305-11,
2002.
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{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.}
}
-
Ermias D Belay and Lawrence B Schonberger.
Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.
Clin Lab Med,
22(4):849-62,
December 2002.
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{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.}
}
-
Patrick Bocquet,
Matthieu De Stampa,
Stampa M De,
Celine Foucher,
Marie-Pierre Delporte,
and Joelle Martigny.
[Sporadic Creutzfeldt-Jakob disease and isolated dementia. Contribution of brain MRI].
Ann Med Interne (Paris),
153(1):62-7,
February 2002.
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{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}
}
-
Ray Bradley.
Bovine spongiform encephalopathy. Update.
Acta Neurobiol Exp (Warsz),
62(3):183-95,
2002.
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{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.}
}
-
Luis Otavio Sales Ferreira Caboclo,
LO Caboclo,
Nancy Huang,
Guilherme Alves Lepski,
Jose Antonio Livramento,
Carlos Alberto Buchpiguel,
Claudia Sellitto Porto,
and Ricardo Nitrini.
Iatrogenic Creutzfeldt-Jakob disease following human growth hormone therapy: case report.
Arq Neuropsiquiatr,
60(2-B):458-61,
June 2002.
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{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.}
}
-
J D Cooper and S M Bird.
UK dietary exposure to BSE in head meat: by birth cohort and gender.
J Cancer Epidemiol Prev,
7(2):71-83,
2002.
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 0x1.58ed97fffe3e8p-895s 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 581002046450f 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 581002130560f 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{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 0x1.58ed97fffe3e8p-895s 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 581002046450f 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 581002035560f 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.}
}
-
Jerome Huillard D'Aignaux,
Simon N Cousens,
Nicole Delasnerie-Laupretre,
Jean-Philippe Brandel,
Dominique Salomon,
Jean-Louis Laplanche,
Jean-Jacques Hauw,
and Annick Alperovitch.
Analysis of the geographical distribution of sporadic Creutzfeldt-Jakob disease in France between 1992 and 1998.
Int J Epidemiol,
31(2):490-5,
April 2002.
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{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.}
}
-
Stephen J DeArmond and Essia Bouzamondo.
Fundamentals of prion biology and diseases.
Toxicology,
181-182:9-16,
December 2002.
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{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.}
}
-
Christl A Donnelly.
BSE in France: epidemiological analysis and predictions.
C R Biol,
325(7):793-806,
July 2002.
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: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.}
}
-
Christl A Donnelly,
Neil M Ferguson,
Azra C Ghani,
and Roy M Anderson.
Implications of BSE infection screening data for the scale of the British BSE epidemic and current European infection levels.
Proc R Soc Lond B Biol Sci,
269(1506):2179-90,
November 2002.
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{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.}
}
-
Dominique Dormont.
Prions, BSE and food.
Int J Food Microbiol,
78(1-2):181-9,
September 2002.
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{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.}
}
-
G W Eschweiler,
H Wormstall,
U Widmann,
T Naegele,
and M Bartels.
[Correlation of diffusion-weighted magnetic resonance imaging with neurological deficits in sporadic Creutzfeldt-Jakob Disease].
Nervenarzt,
73(9):883-6,
September 2002.
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{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.}
}
-
D Galanaud,
D Dormont,
D Grabli,
P Charles,
J J Hauw,
C Lubetzki,
J P Brandel,
C Marsault,
and P J Cozzone.
MR spectroscopic pulvinar sign in a case of variant Creutzfeldt-Jakob disease.
J Neuroradiol,
29(4):285-7,
December 2002.
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{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.}
}
-
Azra C Ghani,
Christl A Donnelly,
Neil M Ferguson,
and Roy M Anderson.
The transmission dynamics of BSE and vCJD.
C R Acad Sci III,
325(1):37-47,
January 2002.
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{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.}
}
-
Stephane Haik,
Didier Dormont,
Baptiste A Faucheux,
Claude Marsault,
and Jean-Jacques Hauw.
Prion protein deposits match magnetic resonance imaging signal abnormalities in Creutzfeldt-Jakob disease.
Ann Neurol,
51(6):797-9,
June 2002.
Keywords:
Brain,
pathology,
Brain Chemistry,
Creutzfeldt-Jakob Syndrome,
metabolism,
Human,
Magnetic Resonance Imaging,
Prions,
analysis,
Support Non-U.S. Gov't.
@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}
}
-
Karsten Henkel,
Inga Zerr,
Andreas Hertel,
Klaus-F Gratz,
Andreas Schroter,
Henriette J Tschampa,
Heiner Bihl,
Udalrich Bull,
Frank Grunwald,
Alexander Drzezga,
Jorg Spitz,
and Sigrid Poser.
Positron emission tomography with [(18)F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD).
J Neurol,
249(6):699-705,
June 2002.
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{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.}
}
-
Colm Henry and Richard Knight.
Clinical features of variant Creutzfeldt-Jakob disease.
Rev Med Virol,
12(3):143-50,
May-Jun 2002.
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{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.}
}
-
Kurt Krapfenbauer,
Byong Chul Yoo,
Michael Fountoulakis,
Eva Mitrova,
and Gert Lubec.
Expression patterns of antioxidant proteins in brains of patients with sporadic Creutzfeldt-Jacob disease.
Electrophoresis,
23(15):2541-7,
August 2002.
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{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.}
}
-
Marek Kubicki,
Carl-Fredrik Westin,
Stephan E Maier,
Hatsuho Mamata,
Melissa Frumin,
Hal Ersner-Hershfield,
Ron Kikinis,
Ferenc A Jolesz,
Robert McCarley,
and Martha E Shenton.
Diffusion tensor imaging and its application to neuropsychiatric disorders.
Harv Rev Psychiatry,
10(6):324-36,
Nov-Dec 2002.
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{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.}
}
-
Martin Kulldorff.
Geographical distribution of sporadic Creutzfeldt-Jakob Disease in France.
Int J Epidemiol,
31(2):495-6,
April 2002.
Keywords:
Creutzfeldt-Jakob Syndrome,
epidemiology,
France,
epidemiology,
Human.
@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}
}
-
Sylvain Lehmann.
[The prion protein].
J Soc Biol,
196(4):309-12,
2002.
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{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.}
}
-
M-A Macleod,
G E Stewart,
M Zeidler,
R Will,
and R Knight.
Sensory features of variant Creutzfeldt-Jakob disease.
J Neurol,
249(6):706-11,
June 2002.
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 0x1.58ed97fffe3e8p-895d persistent sensory symptoms, 16 0x1.8b17008084d28p-895d no sensory symptoms and 18 % were uncertain. In 2 % here 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 (ases. 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 0x8065377atients), dysaesthesia (28 0x812e980atients), paraesthesia (31 0x7fffea2catients) and numbness (25 0x7ffff538atients). The symptoms were lateralised in 31 1001312672f 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{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 0x1.58ed97fffe3e8p-895d persistent sensory symptoms, 16 0x1.83b7008084d28p-895d no sensory symptoms and 18 % were uncertain. In 2 % here 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 (ases. 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 0x8065377atients), dysaesthesia (28 0x812e980atients), paraesthesia (31 0x7fffeb4catients) and numbness (25 0x7ffff538atients). The symptoms were lateralised in 31 1001312672f 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.}
}
-
J-F Mangin,
C Poupon,
C Clark,
D Le Bihan,
Bihan D Le,
and I Bloch.
Distortion correction and robust tensor estimation for MR diffusion imaging.
Med Image Anal,
6(3):191-8,
September 2002.
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{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.}
}
-
N Molko,
L Cohen,
J F Mangin,
F Chochon,
S Lehericy,
D Le Bihan,
Bihan D Le,
and S Dehaene.
Visualizing the neural bases of a disconnection syndrome with diffusion tensor imaging.
J Cogn Neurosci,
14(4):629-36,
May 2002.
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{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.}
}
-
Takaki Murata,
Yusei Shiga,
Shuichi Higano,
Shoki Takahashi,
and Shunji Mugikura.
Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusion-weighted imaging.
AJNR Am J Neuroradiol,
23(7):1164-72,
August 2002.
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{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.}
}
-
Tsutomu Nakada.
[Diffusion tensor analysis: principles and applications].
No To Shinkei,
54(4):290-7,
April 2002.
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{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.}
}
-
Dennis G Jr Pappas and Joel K Cure.
Diagnostic imaging.
Otolaryngol Clin North Am,
35(2):239-53,
April 2002.
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{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.}
}
-
Edgard Pereira.
Diffusion-weighted sequence on MRI for the diagnosis of Creutzfeldt-Jakob disease.
Arq Neuropsiquiatr,
60(4):906-8,
December 2002.
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{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.}
}
-
Alejandro A Rabinstein,
Michelle L Whiteman,
and Robert T Shebert.
Abnormal diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease following corneal transplantations.
Arch Neurol,
59(4):637-9,
April 2002.
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{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.}
}
-
J Ruiz-Alzola,
C-F Westin,
S K Warfield,
C Alberola,
S Maier,
and R Kikinis.
Nonrigid registration of 3D tensor medical data.
Med Image Anal,
6(2):143-61,
June 2002.
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{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.}
}
-
Katherine H Taber,
Pietro Cortelli,
Wolfgang Staffen,
and Robin A Hurley.
The expanding role of imaging in prion disease.
J Neuropsychiatry Clin Neurosci,
14(4):371-6,
Fall 2002.
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{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}
}
-
Rajeev Thakur,
Yasmeen Marbaniang Vincent,
and Sujata Chaturvedi.
Human prion diseases.
Natl Med J India,
15(6):339-45,
Nov-Dec 2002.
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{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.}
}
-
G G Tribl,
G Strasser,
J Zeitlhofer,
S Asenbaum,
C Jarius,
P Wessely,
and D Prayer.
Sequential MRI in a case of Creutzfeldt-Jakob disease.
Neuroradiology,
44(3):223-6,
March 2002.
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{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.}
}
-
H J T Ward,
HJ Ward,
D Everington,
E A Croes,
A Alperovitch,
N Delasnerie-Laupretre,
I Zerr,
S Poser,
C M van Duijn,
and Duijn CM van.
Sporadic Creutzfeldt-Jakob disease and surgery: a case-control study using community controls.
Neurology,
59(4):543-8,
August 2002.
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 °*Q*(M8(MQ* Q* *Q*H
M
Pǰ**(MQ*(M b<% #
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SO"*
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@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 °*Q*(M8(MQ* Q* *Q*H
M
Pǰ**(MQ*(M b<% #(MQ*(M,4yS % DU`ǰ*`*(MQ*P% S U
SO"*
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-
C-F Westin,
S E Maier,
H Mamata,
A Nabavi,
F A Jolesz,
and R Kikinis.
Processing and visualization for diffusion tensor MRI.
Med Image Anal,
6(2):93-108,
June 2002.
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{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.}
}
-
Byong Chul Yoo,
Kurt Krapfenbauer,
Nigel Cairns,
Girma Belay,
Michal Bajo,
and Gert Lubec.
Overexpressed protein disulfide isomerase in brains of patients with sporadic Creutzfeldt-Jakob disease.
Neurosci Lett,
334(3):196-200,
December 2002.
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{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.}
}
-
I Zerr,
B Mollenhauer,
Ch Werner,
and S Poser.
[Early and differential diagnosis of Creutzfeldt-Jakob disease].
Dtsch Med Wochenschr,
127(7):323-7,
February 2002.
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{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}
}