Post by Terry S. Singeltary Sr. on Dec 7, 2023 14:34:08 GMT -6
Prion 2023
Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study
Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5
Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center
Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.
Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.
Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.
Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.
Funded by: Ionis Pharmaceuticals
Grant number: N/A
Acknowledgment: XXX
prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."
22 years ago;
2001 Singeltary on CJD
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
jamanetwork.com/journals/jama/article-abstract/1031186
creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
FRIDAY, JANUARY 15, 2021
CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire?
if not, why not?
cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html
CJD TSE Prion Questionnaire USA, UK, Singeltary
CJD FOUNDATION Questionnaire
cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf
UK CJD Questionnaire
web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
cjd questionnaire 1979
webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf
CJD Questionnaire
F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)
web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
NOW we know that CWD will transmit to cattle, pigs and sheep, by oral routes, seems some of the old documents pertaining to Pigs and BSE might come to light...terry
NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry
THE MAKING OF THE USA CJD QUESTIONNAIRE
cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html
creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html
cjdquestionnaire.blogspot.com/2009/
cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html
cjdquestionnaire.blogspot.com/2007/
cjdquestionnaire.blogspot.com/
MONDAY, SEPTEMBER 11, 2023
Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases
Singeltary sCJD
creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
wasted days and wasted nights...Freddy Fender
terry
Terry S. Singeltary Sr., Bacliff, Texas, 77518, Galveston Bay, flounder9@verizon.net, on the bottom!
Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study
Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5
Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center
Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.
Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.
Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.
Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.
Funded by: Ionis Pharmaceuticals
Grant number: N/A
Acknowledgment: XXX
prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."
22 years ago;
2001 Singeltary on CJD
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
jamanetwork.com/journals/jama/article-abstract/1031186
creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
FRIDAY, JANUARY 15, 2021
CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire?
if not, why not?
cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html
CJD TSE Prion Questionnaire USA, UK, Singeltary
CJD FOUNDATION Questionnaire
cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf
UK CJD Questionnaire
web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
cjd questionnaire 1979
webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf
CJD Questionnaire
F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)
web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
NOW we know that CWD will transmit to cattle, pigs and sheep, by oral routes, seems some of the old documents pertaining to Pigs and BSE might come to light...terry
NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry
THE MAKING OF THE USA CJD QUESTIONNAIRE
cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html
creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html
cjdquestionnaire.blogspot.com/2009/
cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html
cjdquestionnaire.blogspot.com/2007/
cjdquestionnaire.blogspot.com/
MONDAY, SEPTEMBER 11, 2023
Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases
Singeltary sCJD
creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
wasted days and wasted nights...Freddy Fender
terry
Terry S. Singeltary Sr., Bacliff, Texas, 77518, Galveston Bay, flounder9@verizon.net, on the bottom!