Choroba creutzfeldta-jakoba
Epidemiologia

Choroba Creutzfeldta-Jakoba (CJD) to rzadka, śmiertelna encefalopatia prionowa o częstości występowania około 1-2 przypadków na milion osób rocznie, z dominującą sporadyczną postacią (85-90% przypadków). Średni wiek zachorowania wynosi około 62 lata, a średni czas przeżycia w sporadycznej formie to 4-8 miesięcy, przy czym 90% pacjentów umiera w ciągu roku od wystąpienia objawów. Wariant CJD (vCJD), powiązany z gąbczastą encefalopatią bydła (BSE), jest znacznie rzadszy i dotyczy głównie młodszych dorosłych (średni wiek 29 lat). Epidemiologia CJD nie wykazuje sezonowości ani wyraźnych różnic geograficznych, choć obserwuje się wyższe wskaźniki zachorowań w populacjach białych. W ostatnich dekadach w USA odnotowano wzrost standaryzowanego względem wieku wskaźnika zgonów z powodu CJD, co wiąże się m.in. z intensyfikacją nadzoru i postępem diagnostycznym, takim jak test RT-QuIC w płynie mózgowo-rdzeniowym.

  1. Epidemiologia choroby Creutzfeldta-Jakoba
    1. Rodzaje CJD i ich epidemiologia
    2. Rozkład geograficzny i demograficzny
  2. Nadzór nad chorobą Creutzfeldta-Jakoba
    1. Cel nadzoru epidemiologicznego
    2. Międzynarodowe systemy nadzoru
    3. Amerykańskie stanowe systemy nadzoru
  3. Trendy epidemiologiczne w chorobie Creutzfeldta-Jakoba
    1. Zmienność zachorowalności w czasie
    2. Czynniki wpływające na zgłaszalność przypadków
    3. Wariant CJD (vCJD) i jego nadzór
  4. Wyzwania w nadzorze nad CJD i przyszłe kierunki
    1. Znaczenie ciągłości nadzoru
    2. Kolejne rozdziały

Epidemiologia choroby Creutzfeldta-Jakoba

Choroba Creutzfeldta-Jakoba (CJD) to rzadka, śmiertelna choroba neurodegeneracyjna wywoływana przez nieprawidłowo pofałdowane białka zwane prionami, które gromadzą się w ośrodkowym układzie nerwowym. Należy do grupy pasażowalnych encefalopatii gąbczastych występujących u ludzi na całym świecie, z szacowaną częstością występowania wynoszącą około 1-2 przypadki na milion osób rocznie.12 W ostatnich latach w Stanach Zjednoczonych zgłaszano około 500-600 przypadków rocznie.3 Choroba ta jest zawsze śmiertelna, przy czym prawie 70% pacjentów umiera w ciągu roku od wystąpienia objawów.4 Średni czas przeżycia w sporadycznej postaci CJD wynosi 4-8 miesięcy, a 90% pacjentów umiera w ciągu roku.5 W niektórych badaniach średni czas przeżycia określano na 4-5 miesięcy.6

Rodzaje CJD i ich epidemiologia

Choroba Creutzfeldta-Jakoba występuje w kilku formach, które różnią się etiologią i charakterystyką epidemiologiczną:

  • Sporadyczna CJD (sCJD) – najczęstsza forma choroby prionowej u ludzi, stanowiąca około 85-90% wszystkich przypadków.78 Średni wiek zachorowania wynosi 62 lata, chociaż opisywano również przypadki u młodszych i starszych grup wiekowych. Sporadyczna CJD charakteryzuje się proporcją zachorowań mężczyzn do kobiet wynoszącą 1:1.9
  • Genetyczna/rodzinna CJD (gCJD/fCJD) – druga co do częstości postać choroby, stanowiąca około 5-15% przypadków.1011 Pacjenci często mają obciążony wywiad rodzinny i autosomalnie dominujące mutacje genu PRNP.12
  • Nabyta CJD – występuje w mniej niż 1-5% przypadków, zwykle u młodych dorosłych ze średnim wiekiem 29 lat.1314 Do tej kategorii należą przypadki jatrogenne (związane z procedurami medycznymi) oraz wariant CJD (vCJD).

Rozkład geograficzny i demograficzny

Choroba Creutzfeldta-Jakoba występuje na całym świecie bez wyraźnych zmian sezonowych czy geograficznych.15 Najczęściej dotyka osoby w wieku 55-65 lat, chociaż przypadki mogą wystąpić u osób powyżej 90. roku życia i poniżej 55. roku życia.16 Warto zauważyć, że niektóre badania sugerują, że wskaźniki zachorowalności na CJD w grupach nie-białych są niższe niż u osób białych, chociaż mechanizm tego zjawiska nie jest w pełni wyjaśniony.17

W Stanach Zjednoczonych, na podstawie danych z nadzoru umieralności z lat 1979-1994, roczna zapadalność na CJD pozostawała stabilna na poziomie około 1 przypadku na milion osób.18 Jednakże w latach 1979-2017 zapadalność wzrosła do 3,5 przypadku na milion wśród osób powyżej 50. roku życia.19 Zgony z powodu CJD wśród osób poniżej 30. roku życia są niezwykle rzadkie (mniej niż pięć zgonów na miliard rocznie).20

Nadzór nad chorobą Creutzfeldta-Jakoba

Międzynarodowe programy nadzoru nad CJD działają aktywnie od czasu pojawienia się w połowie lat 90. XX wieku wariantu CJD (vCJD), choroby powiązanej z gąbczastą encefalopatią bydła (BSE).21 Nadzór epidemiologiczny jest kluczowy dla monitorowania trendów w epidemiologii chorób prionowych, identyfikacji nowych form choroby i minimalizacji zagrożeń dla zdrowia publicznego.22

Cel nadzoru epidemiologicznego

Główne cele systemów nadzoru nad CJD obejmują:

  • Monitorowanie trendów w epidemiologii chorób prionowych u ludzi23
  • Wykrywanie pojawienia się vCJD lub nowych chorób prionowych u ludzi2425
  • Ocenę skuteczności środków zapobiegawczych26
  • Zapobieganie potencjalnej jatrogennej transmisji27
  • Maksymalizację laboratoryjnego potwierdzenia podejrzanych przypadków we współpracy z ośrodkami referencyjnymi28

Międzynarodowe systemy nadzoru

Na całym świecie istnieje wiele systemów nadzoru nad CJD, z których najważniejsze to:

  • EuroCJD (Europejska Sieć Nadzoru nad Chorobą Creutzfeldta-Jakoba) – utworzona w 1993 roku przez siedem krajów w celu prowadzenia nadzoru epidemiologicznego nad CJD. Obecnie sieć obejmuje krajowe punkty kontaktowe, które są głównymi punktami kontaktowymi dla pasażowalnych encefalopatii gąbczastych w każdym państwie członkowskim UE.29 Nadzór nad vCJD regulowany jest w UE od 2000 roku, a odpowiedzialność za sieć została przekazana Europejskiemu Centrum ds. Zapobiegania i Kontroli Chorób (ECDC) w 2007 roku.30
  • National CJD Research & Surveillance Unit (NCJDRSU) w Wielkiej Brytanii – założona w 1990 roku, zbiera dane o liczbie przypadków i typach CJD w Wielkiej Brytanii.31 Według raportu rocznego NCJDSU z 2021 roku, w okresie od 1 stycznia 1970 r. do 31 grudnia 2021 r. zidentyfikowano w Wielkiej Brytanii 2909 przypadków sCJD, a roczny wskaźnik śmiertelności z powodu sporadycznej CJD wynosił 1,97 przypadku na milion w 2021 roku.32
  • National Prion Disease Pathology Surveillance Center (NPDPSC) w USA – utworzone w 1997 roku przy Wydziale Neuropatologii Uniwersytetu Case Western Reserve. Centrum to monitoruje występowanie chorób prionowych w Stanach Zjednoczonych oraz identyfikuje przypadki, które mogą wynikać z nabytej etiologii.3334
  • Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS) – prowadzony przez Agencję Zdrowia Publicznego Kanady, prowadzi prospektywny krajowy nadzór nad wszystkimi rodzajami chorób prionowych u ludzi w Kanadzie. Głównym celem CJDSS jest lepsze zrozumienie epidemiologii chorób prionowych u ludzi, poprawa opcji dostępnych dla ich szybkiej i dokładnej diagnostyki oraz ochrona zdrowia Kanadyjczyków poprzez zmniejszenie ryzyka przenoszenia chorób prionowych.35 Nadzór nad CJD w Kanadzie rozpoczął się w 1998 roku.36
  • Australian National CJD Registry – z siedzibą na Uniwersytecie w Melbourne, prowadzi nadzór nad vCJD. Od 1990 roku australijski Departament Rolnictwa prowadzi aktywny nadzór nad BSE u bydła.37
  • China CJD Surveillance – formalnie rozpoczęty w 2006 roku, w ramach krajowej sieci nadzoru nad chorobami zakaźnymi Chińskiego Centrum Kontroli i Zapobiegania Chorobom (CCDC). Początkowo składał się z 10 prowincjonalnych CDC i 11 szpitali.38 Do końca 2010 roku zidentyfikowano i zdiagnozowano 284 różne przypadki CJD i ludzkich TSE.39
  • New Zealand Creutzfeldt-Jakob Disease Surveillance Registry – CJD jest chorobą podlegającą obowiązkowi zgłaszania w Nowej Zelandii. Podejrzane przypadki CJD powinny być zgłaszane zarówno do Rejestru Nadzoru nad CJD w Nowej Zelandii przy Dunedin School of Medicine, jak i do lokalnego urzędnika ds. zdrowia.40

Amerykańskie stanowe systemy nadzoru

W Stanach Zjednoczonych oprócz krajowego nadzoru prowadzonego przez NPDPSC, wiele stanów prowadzi własne systemy nadzoru:

  • California Creutzfeldt-Jakob Disease Surveillance Project – we współpracy z CDC i Kalifornijskim Departamentem Zdrowia Publicznego (CDPH), prowadzi wzmocniony nadzór nad CJD, vCJD i innymi pasażowalnymi encefalopatiami gąbczastymi w stanie Kalifornia. Od czerwca 2007 r. CDPH dodał TSE, w tym CJD, do listy chorób podlegających obowiązkowi zgłaszania.41
  • Texas CJD Surveillance – CJD jest chorobą podlegającą obowiązkowi zgłaszania w Teksasie od 1998 roku. Od 15 lat Teksas prowadzi wzmocniony nadzór (nadzór bierny i aktywny) nad CJD, w tym sporadyczną, genetyczną/rodzinną i nabytą (jatrogenną i wariantową) CJD.42 W Teksasie średni wskaźnik zgonów na milion mieszkańców z powodu CJD w ciągu ostatnich 10 lat (2013-2022) wynosi 1,2 przypadku na milion mieszkańców rocznie.43
  • Florida CJD Surveillance – choroba stała się podlegającą zgłoszeniu 9 czerwca 2003 r. CJD jest monitorowana w celu zapobiegania i kontroli ognisk choroby.44
  • Delaware CJD Surveillance – CJD stała się chorobą podlegającą zgłoszeniu w Delaware w 2006 roku.45
  • Wyoming Prion Disease Surveillance – podejrzane przypadki CJD i innych ludzkich chorób prionowych podlegają zgłoszeniu do instytucji zdrowia publicznego w Wyoming.46

Trendy epidemiologiczne w chorobie Creutzfeldta-Jakoba

Dane z nadzoru na całym świecie wykazały pewne interesujące trendy w epidemiologii CJD, które pomogły pogłębić nasze zrozumienie tej choroby.

Zmienność zachorowalności w czasie

Chociaż ogólna roczna zapadalność na CJD pozostaje na poziomie około 1-2 przypadków na milion osób, w ostatnich latach odnotowano pewne zmiany w trendach:

  • W Stanach Zjednoczonych dane dotyczące zgonów z powodu CJD i standaryzowanego względem wieku wskaźnika zgonów wskazują na rosnący trend w liczbie zgonów w latach 1979-2017.47
  • W Teksasie odnotowano wzrost liczby przypadków CJD w ciągu ostatnich pięciu lat, co można przypisać kilku czynnikom, w tym intensyfikacji metod nadzoru.48
  • Roczne wskaźniki zachorowalności na CJD znacznie się różniły w 25-letnim okresie badania na Florydzie, potencjalnie odzwierciedlając fluktuacje w nieznanych narażeniach środowiskowych.49

Czynniki wpływające na zgłaszalność przypadków

Kilka czynników może wpływać na zgłaszaną częstość występowania CJD:

  • Intensywność nadzoru – zwiększa liczbę zgłaszanych przypadków, często w krajach, w których w przeszłości występowały epidemie CJD i gdzie zasoby do prowadzenia nadzoru są największe.50 Na przykład w Teksasie zwiększony nadzór przyczynił się do wyższych wskaźników wykrywania przypadków.51
  • Udoskonalone metody diagnostyczne – metody takie jak RT-QuIC w płynie mózgowo-rdzeniowym pozwalają na zmniejszenie zależności od obecności specyficznych objawów neurologicznych w klasyfikacji przypadku CJD. Umożliwiło to klasyfikację przypadków CJD, które wcześniej mogły nie być liczone.52
  • Starzenie się populacji – ponieważ zapadalność na sporadyczną CJD jest wyższa wśród starszych pacjentów, starzenie się populacji na całym świecie prowadzi do ewolucji epidemiologii CJD.53
  • Świadomość klinicystów – zwiększona świadomość wśród pracowników służby zdrowia przyczynia się do lepszego rozpoznawania i zgłaszania przypadków.54
  • Wskaźniki autopsji – potwierdzenie CJD wymaga neuropatologicznego i/lub immunodiagnostycznego badania tkanki mózgowej uzyskanej w biopsji lub autopsji.55 Jednakże, nie u każdego, kto prawdopodobnie zmarł z powodu choroby prionowej, analizowano tkankę mózgową.56 W Kalifornii autopsje przeprowadza się tylko w około 22% przypadków CJD.57

Wariant CJD (vCJD) i jego nadzór

Wariant CJD (vCJD) pojawił się w połowie lat 90. XX wieku i został powiązany z gąbczastą encefalopatią bydła (BSE). Chociaż środki kontroli skutecznie opanowały BSE, a częstość występowania vCJD spadła, istnieją obawy, że dalsze przypadki vCJD mogą pojawić się w wyniku przedłużonej inkubacji i/lub wtórnego przenoszenia.58

Według danych Światowej Organizacji Zdrowia, do marca 2011 r. na całym świecie zgłoszono łącznie 224 przypadki vCJD: 175 przypadków w Wielkiej Brytanii i 49 przypadków w innych krajach (w tym 25 we Francji, 5 w Hiszpanii, 4 w Irlandii, 3 w Holandii, 3 w Stanach Zjednoczonych).59 Do 31 grudnia 2021 r. w Wielkiej Brytanii zidentyfikowano 178 przypadków potwierdzonego lub prawdopodobnego vCJD.60

Wariant CJD jest jeszcze rzadszy niż sporadyczna CJD, z około 220 znanymi przypadkami na całym świecie. W Stanach Zjednoczonych nie odnotowano żadnych przypadków vCJD nabytych w kraju.61 Trzy przypadki odnotowane w USA związane były z narażeniem podczas pobytu w Wielkiej Brytanii (dwa przypadki) i Arabii Saudyjskiej (jeden przypadek).62

Wyzwania w nadzorze nad CJD i przyszłe kierunki

Pomimo postępów w nadzorze nad CJD, nadal istnieją wyzwania i obszary wymagające dalszej uwagi:

  • Potrzeba zwiększenia wskaźników autopsji – ponieważ tylko badanie patologiczne tkanki może odróżnić klasyczne i wariantowe formy CJD, należy zwiększyć wskaźniki autopsji w podejrzanych przypadkach CJD.63
  • Opracowanie testów diagnostycznych – opracowanie testu, który mógłby być stosowany na łatwo dostępnej tkance w celu wykrycia zakażenia we wczesnym okresie inkubacji, nie tylko pogłębiłoby zrozumienie epidemiologii zakażenia czynnikiem, ale również pomogłoby we wdrażaniu środków kontroli zapobiegających potencjalnemu jatrogennemu rozprzestrzenianiu się.64
  • Monitorowanie nowych chorób prionowych – pojawiające się choroby, takie jak choroba wyniszczająca jeleniowatych i choroba prionowa wielbłądów, stwarzają dalsze zagrożenia dla zdrowia publicznego.65
  • Różnice kulturowe w podejściu do leczenia – w Japonii, na Tajwanie i w Chinach, w dobrze zorganizowanych systemach opieki zdrowotnej, większość pacjentów poddawana jest intensywnemu leczeniu podtrzymującemu życie, które przedłuża ich przeżycie. Dla kontrastu, w krajach zachodnich, ze względu na aspekty finansowe i etyczne, intensywne leczenie podtrzymujące życie, takie jak karmienie przez zgłębnik, nie jest powszechnie stosowane u pacjentów ze śmiertelnymi schorzeniami neurologicznymi, takimi jak choroba prionowa.66

Znaczenie ciągłości nadzoru

Pomimo spadku liczby przypadków vCJD, kontynuacja nadzoru nad CJD jest konieczna, aby sprostać potencjalnym przyszłym przypadkom vCJD lub pojawieniu się nowych chorób prionowych u ludzi.67 Nadzór zapewnia dokładne dane na temat liczby przypadków i typów CJD, co ma kluczowe znaczenie dla zrozumienia epidemiologii choroby i opracowania skutecznych strategii zapobiegania.

Jak podkreślono w przeglądzie opublikowanym w Nature Reviews Neurology, międzynarodowe programy nadzoru nad CJD odegrały kluczową rolę w monitorowaniu trendów w epidemiologii CJD i łagodzeniu zagrożeń dla zdrowia publicznego.68 Kontynuacja tych wysiłków będzie miała zasadnicze znaczenie dla ochrony zdrowia publicznego w przyszłości.

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  1. 10.04.2026
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Materiały źródłowe

  • #1 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #2 Classic Creutzfeldt-Jakob Disease | Classic CJD | CDC
    https://www.cdc.gov/creutzfeldt-jakob/about/index.html
    Classic CJD is a quick-moving, always fatal disease that occurs worldwide. […] CJD occurs worldwide with an estimated rate of about one to two illness per one million population members per year. In recent years in the United States, about 500-600 cases have been reported per year. […] Classic CJD is always fatal, usually within a few months. […] Classic Creutzfeldt-Jakob Disease is a fatal brain disease that most often occurs sporadically. There is no treatment and death usually occurs in 4-5 months.
  • #3 Classic Creutzfeldt-Jakob Disease | Classic CJD | CDC
    https://www.cdc.gov/creutzfeldt-jakob/about/index.html
    Classic CJD is a quick-moving, always fatal disease that occurs worldwide. […] CJD occurs worldwide with an estimated rate of about one to two illness per one million population members per year. In recent years in the United States, about 500-600 cases have been reported per year. […] Classic CJD is always fatal, usually within a few months. […] Classic Creutzfeldt-Jakob Disease is a fatal brain disease that most often occurs sporadically. There is no treatment and death usually occurs in 4-5 months.
  • #4 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #5 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #6 Classic Creutzfeldt-Jakob Disease | Classic CJD | CDC
    https://www.cdc.gov/creutzfeldt-jakob/about/index.html
    Classic CJD is a quick-moving, always fatal disease that occurs worldwide. […] CJD occurs worldwide with an estimated rate of about one to two illness per one million population members per year. In recent years in the United States, about 500-600 cases have been reported per year. […] Classic CJD is always fatal, usually within a few months. […] Classic Creutzfeldt-Jakob Disease is a fatal brain disease that most often occurs sporadically. There is no treatment and death usually occurs in 4-5 months.
  • #7 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #8 NC DPH: Creutzfeldt-Jakob Disease (CJD)
    https://epi.dph.ncdhhs.gov/cd/diseases/cjd.html
    Classic Creutzfeldt-Jakob Disease (CJD) is a rare human prion disease that affects one to two persons per million per year worldwide. […] The United States has reported fewer than 300 cases of CJD a year in recent years. […] Most (about 85%) of CJD cases develop spontaneously (no known cause) and are called Sporadic CJD (sCJD). Genetic or familial CJD, represents about 5-15 percent of cases and is associated with inherited mutations of the prion protein gene (examples are Gerstmann-Straussler-Scheinker Syndrome and fatal familial insomnia). […] Less than one percent of cases occur during invasive medical procedures involving the central nervous system and are due to exposure to contaminated brain tissue. […] An autopsy is very important in the diagnosis of CJD because it is the best way to confirm presence of the disease. […] Classic CJD is not related to „mad cow” disease, or Bovine Spongiform Encephalopathy (BSE).
  • #9 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #10 NC DPH: Creutzfeldt-Jakob Disease (CJD)
    https://epi.dph.ncdhhs.gov/cd/diseases/cjd.html
    Classic Creutzfeldt-Jakob Disease (CJD) is a rare human prion disease that affects one to two persons per million per year worldwide. […] The United States has reported fewer than 300 cases of CJD a year in recent years. […] Most (about 85%) of CJD cases develop spontaneously (no known cause) and are called Sporadic CJD (sCJD). Genetic or familial CJD, represents about 5-15 percent of cases and is associated with inherited mutations of the prion protein gene (examples are Gerstmann-Straussler-Scheinker Syndrome and fatal familial insomnia). […] Less than one percent of cases occur during invasive medical procedures involving the central nervous system and are due to exposure to contaminated brain tissue. […] An autopsy is very important in the diagnosis of CJD because it is the best way to confirm presence of the disease. […] Classic CJD is not related to „mad cow” disease, or Bovine Spongiform Encephalopathy (BSE).
  • #11 Creutzfeldt-Jakob Disease (CJD) – Epidemiology
    https://www.vdh.virginia.gov/epidemiology/epidemiology-fact-sheets/creutzfeldt-jakob-disease-cjd/
    CJD occurs worldwide. In the United States, about one person in every one million people develop CJD. […] A smaller percentage of patients (5%15%) inherit the disease. […] Although very rare (1%), people who received infected tissue during surgical procedures (corneal transplants, surgery on the brain or its coverings) have developed CJD. […] Hospitals are aware of the need to use disposable equipment or take extra precautions to sterilize surgical instruments so that patients are protected against infection with CJD. […] Properly sterilizing medical equipment may also prevent the spread of disease.
  • #12 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #13 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK507860/
    Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. This condition belongs to a group of transmissible spongiform encephalopathies affecting people worldwide, with an incidence of 1 case per million per year. Approximately 350 cases are diagnosed annually in the United States. Death occurs in nearly 70% of patients within a year. […] CJD affects about 1 individual per million per year worldwide. Approximately 350 cases are diagnosed annually in the United States. Sporadic CJD is the most common form of human prion disease. The condition has a mean onset age of 62, although it has also been reported in younger and older age groups. Sporadic CJD has a 1:1 male-to-female ratio. Approximately 1 to 2 new sporadic CJD cases appear per 1,000,000 individuals worldwide annually. Death occurs in nearly 70% of patients within a year of onset. The mean survival of sporadic CJD is 4 to 8 months, with 90% of patients dying within a year. […] Genetic CJD is the second most common type of this condition. Patients often have a family history and autosomal-dominant PRNP gene mutations. Acquired cases are seen in less than 1% of cases, usually in young adults with a mean age of 29.
  • #14 Creutzfeldt-Jakob Disease (CJD) – Epidemiology
    https://www.vdh.virginia.gov/epidemiology/epidemiology-fact-sheets/creutzfeldt-jakob-disease-cjd/
    CJD occurs worldwide. In the United States, about one person in every one million people develop CJD. […] A smaller percentage of patients (5%15%) inherit the disease. […] Although very rare (1%), people who received infected tissue during surgical procedures (corneal transplants, surgery on the brain or its coverings) have developed CJD. […] Hospitals are aware of the need to use disposable equipment or take extra precautions to sterilize surgical instruments so that patients are protected against infection with CJD. […] Properly sterilizing medical equipment may also prevent the spread of disease.
  • #15 Surveillance for Creutzfeldt-Jakob disease in China from 2006 to 2007 | BMC Public Health | Full Text
    https://bmcpublichealth.biomedcentral.com/articles/10.1186/1471-2458-8-360
    Chinese CJD patients possessed similar epidemiological and clinical characteristics as worldwide. […] In China, the CJD surveillance system was established under the framework of the surveillances for communicable diseases led by Chinese Center for Disease Control and Prevention (CCDC) since 2002 and became broader in the past two years. […] The surveillance and study was approved by the Ethical Review Committee of China CDC. […] The resident places of probable and possible sCJD cases were diffused in 23 different provinces in China, although Beijing and Shanghai reported more cases than other provinces. […] The occupations of probable and possible sCJD cases were also various, including workers, farmers, teachers, officials, self-employees and house-wives. […] The suspected CJD cases were reported around all year, without seasonal specificity. […] The results of the present study revealed the general epidemiology status of CJD in China from 2006 to 2007. […] The morbidity of CJD in China still remained unsettled for the short-term surveillance.
  • #16 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD occurs worldwide at a rate of about 1 case per million population per year. […] On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million people in the United States. […] In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per billion per year). […] The disease is found most frequently in people 55-65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age. […] In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms. […] CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017. […] Approximately 85% of CJD cases are sporadic and 10-15% of CJD cases are due to inherited mutations of the prion protein gene.
  • #17 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD deaths and age-adjusted death rate in the United States indicate an increasing trend in the number of deaths between 1979 and 2017. […] Although not fully understood, additional information suggests that CJD rates in nonwhite groups are lower than in whites. […] The intensity of CJD surveillance increases the number of reported cases, often in countries where CJD epidemics have occurred in the past and where surveillance resources are greatest. […] An increase in surveillance and reporting of CJD is most likely in response to BSE and vCJD. […] Possible factors contributing to an increase of CJD incidence are an aging population, population increase, clinician awareness, and more accurate diagnostic methods.
  • #18 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD occurs worldwide at a rate of about 1 case per million population per year. […] On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million people in the United States. […] In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per billion per year). […] The disease is found most frequently in people 55-65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age. […] In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms. […] CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017. […] Approximately 85% of CJD cases are sporadic and 10-15% of CJD cases are due to inherited mutations of the prion protein gene.
  • #19 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD occurs worldwide at a rate of about 1 case per million population per year. […] On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million people in the United States. […] In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per billion per year). […] The disease is found most frequently in people 55-65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age. […] In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms. […] CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017. […] Approximately 85% of CJD cases are sporadic and 10-15% of CJD cases are due to inherited mutations of the prion protein gene.
  • #20 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD occurs worldwide at a rate of about 1 case per million population per year. […] On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million people in the United States. […] In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per billion per year). […] The disease is found most frequently in people 55-65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age. […] In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms. […] CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017. […] Approximately 85% of CJD cases are sporadic and 10-15% of CJD cases are due to inherited mutations of the prion protein gene.
  • #21 The importance of ongoing international surveillance for Creutzfeldt–Jakob disease | Nature Reviews Neurology
    https://www.nature.com/articles/s41582-021-00488-7
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. […] However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. […] Continued CJD surveillance is a necessity to meet the potential for further cases of vCJD or the emergence of novel prion diseases in humans. […] Creutzfeldt-Jakob disease (CJD) is a transmissible and universally fatal human prion disease; surveillance programmes exist globally to monitor trends in CJD epidemiology and mitigate public health risks.
  • #22 The importance of ongoing international surveillance for Creutzfeldt–Jakob disease | Nature Reviews Neurology
    https://www.nature.com/articles/s41582-021-00488-7
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. […] However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. […] Continued CJD surveillance is a necessity to meet the potential for further cases of vCJD or the emergence of novel prion diseases in humans. […] Creutzfeldt-Jakob disease (CJD) is a transmissible and universally fatal human prion disease; surveillance programmes exist globally to monitor trends in CJD epidemiology and mitigate public health risks.
  • #23 Creutzfeldt-Jakob disease (CJD) – Delaware Health and Social Services – State of Delaware
    https://www.dhss.delaware.gov/dph/epi/cjd.html
    CJD became a reportable condition in Delaware in 2006: Regulations for the Control of Communicable and Other Disease Conditions. […] Purpose of Reporting and Surveillance: […] To monitor trends in the epidemiology of human prion diseases. […] To maximize laboratory confirmation of suspected cases in collaboration with the National Prion Disease Pathology Surveillance Center. […] To detect the emergence of vCJD or novel prion diseases in the United States. […] To prevent potential iatrogenic (acquired) transmission. […] Healthcare providers should contact the Delaware Division of Public Health (DPH), Bureau of Epidemiology to report any suspect case of CJD.
  • #24 Creutzfeldt-Jakob disease (CJD) – Delaware Health and Social Services – State of Delaware
    https://www.dhss.delaware.gov/dph/epi/cjd.html
    CJD became a reportable condition in Delaware in 2006: Regulations for the Control of Communicable and Other Disease Conditions. […] Purpose of Reporting and Surveillance: […] To monitor trends in the epidemiology of human prion diseases. […] To maximize laboratory confirmation of suspected cases in collaboration with the National Prion Disease Pathology Surveillance Center. […] To detect the emergence of vCJD or novel prion diseases in the United States. […] To prevent potential iatrogenic (acquired) transmission. […] Healthcare providers should contact the Delaware Division of Public Health (DPH), Bureau of Epidemiology to report any suspect case of CJD.
  • #25 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    Prion disease surveillance monitors the occurrence of prion diseases in the United States, but it also monitors for the emergence of vCJD and other potentially preventable new prion diseases, as well as for rare classic forms of prion diseases that are attributable to medical procedures. Prion disease surveillance also helps assess the efficacy of ongoing U.S. prevention measures. […] CJD has been a NOTIFIABLE CONDITION in Texas since 1998, and it was likely under-reported and misdiagnosed for many years. For 15 years now, Texas has carried out enhanced surveillance (passive and active surveillance) for CJD, including sporadic, genetic/familial, and acquired (iatrogenic and variant) CJD. The success of this program is demonstrated by the identification and confirmation of sporadic (sCJD), genetic/familial (gCJD or fCJD) and variant CJD (vCJD), as well as other cases of human prion diseases such as Variably Protease Sensitive Prionopathy (VPSPr), Fatal Familial Insomnia (FFI), sporadic Fatal Insomnia (sFI), and Gerstmann-Strussler-Scheinker (GSS) syndrome.
  • #26 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    Prion disease surveillance monitors the occurrence of prion diseases in the United States, but it also monitors for the emergence of vCJD and other potentially preventable new prion diseases, as well as for rare classic forms of prion diseases that are attributable to medical procedures. Prion disease surveillance also helps assess the efficacy of ongoing U.S. prevention measures. […] CJD has been a NOTIFIABLE CONDITION in Texas since 1998, and it was likely under-reported and misdiagnosed for many years. For 15 years now, Texas has carried out enhanced surveillance (passive and active surveillance) for CJD, including sporadic, genetic/familial, and acquired (iatrogenic and variant) CJD. The success of this program is demonstrated by the identification and confirmation of sporadic (sCJD), genetic/familial (gCJD or fCJD) and variant CJD (vCJD), as well as other cases of human prion diseases such as Variably Protease Sensitive Prionopathy (VPSPr), Fatal Familial Insomnia (FFI), sporadic Fatal Insomnia (sFI), and Gerstmann-Strussler-Scheinker (GSS) syndrome.
  • #27 Creutzfeldt-Jakob disease (CJD) – Delaware Health and Social Services – State of Delaware
    https://www.dhss.delaware.gov/dph/epi/cjd.html
    CJD became a reportable condition in Delaware in 2006: Regulations for the Control of Communicable and Other Disease Conditions. […] Purpose of Reporting and Surveillance: […] To monitor trends in the epidemiology of human prion diseases. […] To maximize laboratory confirmation of suspected cases in collaboration with the National Prion Disease Pathology Surveillance Center. […] To detect the emergence of vCJD or novel prion diseases in the United States. […] To prevent potential iatrogenic (acquired) transmission. […] Healthcare providers should contact the Delaware Division of Public Health (DPH), Bureau of Epidemiology to report any suspect case of CJD.
  • #28 Creutzfeldt-Jakob disease (CJD) – Delaware Health and Social Services – State of Delaware
    https://www.dhss.delaware.gov/dph/epi/cjd.html
    CJD became a reportable condition in Delaware in 2006: Regulations for the Control of Communicable and Other Disease Conditions. […] Purpose of Reporting and Surveillance: […] To monitor trends in the epidemiology of human prion diseases. […] To maximize laboratory confirmation of suspected cases in collaboration with the National Prion Disease Pathology Surveillance Center. […] To detect the emergence of vCJD or novel prion diseases in the United States. […] To prevent potential iatrogenic (acquired) transmission. […] Healthcare providers should contact the Delaware Division of Public Health (DPH), Bureau of Epidemiology to report any suspect case of CJD.
  • #29 European Creutzfeldt-Jakob Disease Surveillance Network (EuroCJD)
    https://www.ecdc.europa.eu/en/about-ecdc/what-we-do/partners-and-networks/disease-and-laboratory-networks/european-creutzfeldt
    The EuroCJD network was established in 1993 by seven countries to conduct epidemiological surveillance for Creutzfeldt Jakob disease and was subsequently enlarged to include more countries and a research component, funded by DG Research (now DG RI) and DG SANCO (now DG SANTE) within the European Commission. […] The surveillance of variant Creutzfeldt Jakob disease (vCJD) was regulated in the EU in 2000, and the network was evaluated and responsibility was transferred to the European Centre for Disease Prevention and Control (ECDC) in 2007. […] The legal basis for vCJD surveillance is now retained under Decision No 1082/2013/EU of the European Parliament and of the Council of 22 October 2013 on serious cross-border threats to health. […] The network is now comprised of National Focal Points (NFPs), who are the primary contact point for TSEs in each Member State, and Operational Contact Points (OCPs) who are nominated on the basis of their technical and operational expertise in vCJD and other TSEs.
  • #30 European Creutzfeldt-Jakob Disease Surveillance Network (EuroCJD)
    https://www.ecdc.europa.eu/en/about-ecdc/what-we-do/partners-and-networks/disease-and-laboratory-networks/european-creutzfeldt
    The EuroCJD network was established in 1993 by seven countries to conduct epidemiological surveillance for Creutzfeldt Jakob disease and was subsequently enlarged to include more countries and a research component, funded by DG Research (now DG RI) and DG SANCO (now DG SANTE) within the European Commission. […] The surveillance of variant Creutzfeldt Jakob disease (vCJD) was regulated in the EU in 2000, and the network was evaluated and responsibility was transferred to the European Centre for Disease Prevention and Control (ECDC) in 2007. […] The legal basis for vCJD surveillance is now retained under Decision No 1082/2013/EU of the European Parliament and of the Council of 22 October 2013 on serious cross-border threats to health. […] The network is now comprised of National Focal Points (NFPs), who are the primary contact point for TSEs in each Member State, and Operational Contact Points (OCPs) who are nominated on the basis of their technical and operational expertise in vCJD and other TSEs.
  • #31 THE NATIONAL CJD RESEARCH & SURVEILLANCE UNIT (NCJDRSU) | National CJD Research & Surveillance Unit
    https://cjd.ed.ac.uk/
    Following the NIHRs decision not to renew funding, the National CJD Research and Surveillance Unit (NCJDRSU, originally the NCJDSU), founded in 1990, will cease to function on 31.03.25. […] There is still importance in ensuring accurate data about the number of cases, and types, of CJD in the UK. […] The NCJDAS will continue the past NCJDRSUs collaboration/liaison with the NHS National Prion Clinic at UCLH (consultants Professors John Collinge FRS and Simon Mead and Dr Tzehow Mok).
  • #32 Creutzfeldt-Jakob Disease (CJD) | Doctor
    https://patient.info/doctor/creutzfeldt-jakob-disease
    This disease is notifiable in the UK – see NOIDs article for more detail. […] International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] There is a National CJD Surveillance Unit (NCJDSU) based at the Western General Infirmary in Edinburgh, which brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease. […] The annual NCJDSU report in 2021 reported: Between 1st January 1970 and 31st December 2021, 2909 cases of sCJD were identified in the UK. […] The annual mortality rate for sporadic CJD (sCJD) was 1.97 cases/million in 2021. […] Up to 31st December 2021, 178 cases of definite or probable vCJD had been identified in the UK.
  • #33 CJD Surveillance | Pathology | School of Medicine | Case Western Reserve University
    https://case.edu/medicine/pathology/research/national-prion-disease-pathology-surveillance-center/cjd-surveillance
    The National Prion Disease Pathology Surveillance Center (NPDPSC) was established in 1997 at the Division of Neuropathology of Case Western Reserve University. Several European countries also have established surveillance centers to monitor the occurrence of prion diseases or spongiform encephalopathies, in response to the epidemic of Bovine Spongiform Encephalopathy (BSE), also known as „mad cow disease,” which occurred in the United Kingdom during the 1980s. […] The center studies and tracks the following prion diseases. […] Creutzfeldt-Jakob Disease (CJD) […] Variant Creutzfeldt-Jakob Disease (vCJD) […] Gerstmann-Straussler-Scheinker Syndrome (GSS) […] Fatal Familial Insomnia (FFI)
  • #34 Creutzfeldt-Jakob Disease (CJD) Resources | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd/creutzfeldt-jakob-disease-cjd-resources
    Supported by CDC and sponsored by the American Association of Neuropathologists, NPDPSC was established to help monitor the incidence of human prion disease in the United States as well as decipher which cases may be due to an acquired etiology and monitor for novel prion diseases. […] The mission of the Creutzfeldt-Jakob Disease Foundation (CJD Foundation) is to support families affected by Prion Disease, raise awareness, and support medical education and research.
  • #35 Creutzfeldt-Jakob Disease Surveillance System – Canada.ca
    https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease.html
    The Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS) is operated by the Public Health Agency of Canada and conducts prospective national surveillance for all types of human prion disease in Canada. The main purposes of the CJDSS are to better understand the epidemiology of human prion diseases, to improve the options available for their rapid and accurate diagnosis, and ultimately to protect the health of Canadians by reducing risks of prion disease transmission. […] Please note that all human prion diseases are provincially reportable and nationally notifiable in Canada.
  • #36 Creutzfeldt-Jakob disease surveillance system report – Canada.ca
    https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html
    Referrals of suspected CJD reported by CJDSS […] CJD cases reported by CJDSS […] CJD cases reported by province/territory […] Incidence of CJD cases reported by CJDSS in Canada […] Canadian CJD surveillance began in 1998.
  • #37 Creutzfeldt-Jakob disease
    https://www.health.vic.gov.au/infectious-diseases/creutzfeldt-jakob-disease
    Creutzfeldt-Jakob disease (CJD) must be notified by medical practitioners and pathology services in writing within 5 days of diagnosis. […] CreutzfeldtJakob disease (CJD) is a routine notifiable condition and must be notified by medical practitioners and pathology services in writing within 5 days of diagnosis. […] The Australian National CJD Registry based at the University of Melbourne conducts surveillance for vCJD. […] Since 1990, the Australian Department of Agriculture and its predecessors have conducted active surveillance for BSE in cattle. […] Sporadic CJD continues to occur; about 20 cases are reported in Australia annually. Global annual incidence is approximately one per 1 million population.
  • #38 The Epidemiological, Clinical, and Laboratory Features of Sporadic Creutzfeldt-Jakob Disease Patients in China: Surveillance Data from 2006 to 2010 | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0024231
    In China, the surveillance for CJD started in 2006, covering 12 provincial Centers for Disease Control and Prevention (CDCs) and 15 hospitals. […] In 2006, a surveillance program for CJD was conducted in China, which was supported by Chinese Center for Disease Control and Prevention (CCDC). Up to the end of 2010, 284 various CJD and human TSE cases were identified and diagnosed. […] China CJD surveillance started formally in 2006, under the framework of the national communicable surveillance network of the CCDC. It consisted of 10 provincial CDCs and 11 hospitals, distributing in Beijing, Shanghai, Tianjin, Chongqing, Jilin, Shaanxi, Hubei, Guangdong, Guizhou and Anhui. […] The clinical and epidemiological data of suspected CJD patient were collected with the designed questionnaires.
  • #39 The Epidemiological, Clinical, and Laboratory Features of Sporadic Creutzfeldt-Jakob Disease Patients in China: Surveillance Data from 2006 to 2010 | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0024231
    In China, the surveillance for CJD started in 2006, covering 12 provincial Centers for Disease Control and Prevention (CDCs) and 15 hospitals. […] In 2006, a surveillance program for CJD was conducted in China, which was supported by Chinese Center for Disease Control and Prevention (CCDC). Up to the end of 2010, 284 various CJD and human TSE cases were identified and diagnosed. […] China CJD surveillance started formally in 2006, under the framework of the national communicable surveillance network of the CCDC. It consisted of 10 provincial CDCs and 11 hospitals, distributing in Beijing, Shanghai, Tianjin, Chongqing, Jilin, Shaanxi, Hubei, Guangdong, Guizhou and Anhui. […] The clinical and epidemiological data of suspected CJD patient were collected with the designed questionnaires.
  • #40 New Zealand Creutzfeldt-Jakob Disease Surveillance Registry, New Zealand Creutzfeldt-Jakob Disease Surveillance Registry | University of Otago
    https://www.otago.ac.nz/cjd-registry
    Creutzfeldt-Jakob Disease (CJD) is a fatal disease characterised by rapidly progressing dementia and associated clinical features such as visual symptoms, ataxia, and myoclonus. […] CJD is a notifiable disease in New Zealand. […] Suspected CJD should be notified both to the New Zealand CJD Surveillance Registry at the Dunedin School of Medicine [email protected] and to the local medical officer of health. […] A formal surveillance classification of definite CJD requires pathological confirmation, i.e. post-mortem examination of brain tissue for spongiform change and/or positive immunoreactivity to prion protein. […] Post-mortems in suspected cases of CJD are performed at the national referral sites for high-risk post-mortems in Christchurch or Auckland and samples may be sent overseas for further analysis. […] Professor Patricia Priest is our epidemiologist.
  • #41 Creutzfeldt-Jakob Disease (CJD) – CEIP
    https://ceip.us/projects/cjd/
    The California Creutzfeldt-Jakob Disease (CJD) Surveillance Project, in collaboration with CDC and the California Department of Public Health (CDPH), conducts enhanced surveillance for CJD, variant CJD (vCJD), and other Transmissible Spongiform Encephalopathies (TSE) in the state of California. […] As of June 2007 CDPH added TSEs, including CJD, to the list of reportable diseases. […] Review CJD epidemiologic data to learn more about these illnesses, monitor trends and detect novel forms of disease or transmission. […] Monitor ongoing CJD testing in collaboration with the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University and the CDC. […] Support CDPH reporting efforts. […] Improve the accuracy of diagnoses through increased autopsy and biopsy rates in cases of suspected TSE in California.
  • #42 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    Prion disease surveillance monitors the occurrence of prion diseases in the United States, but it also monitors for the emergence of vCJD and other potentially preventable new prion diseases, as well as for rare classic forms of prion diseases that are attributable to medical procedures. Prion disease surveillance also helps assess the efficacy of ongoing U.S. prevention measures. […] CJD has been a NOTIFIABLE CONDITION in Texas since 1998, and it was likely under-reported and misdiagnosed for many years. For 15 years now, Texas has carried out enhanced surveillance (passive and active surveillance) for CJD, including sporadic, genetic/familial, and acquired (iatrogenic and variant) CJD. The success of this program is demonstrated by the identification and confirmation of sporadic (sCJD), genetic/familial (gCJD or fCJD) and variant CJD (vCJD), as well as other cases of human prion diseases such as Variably Protease Sensitive Prionopathy (VPSPr), Fatal Familial Insomnia (FFI), sporadic Fatal Insomnia (sFI), and Gerstmann-Strussler-Scheinker (GSS) syndrome.
  • #43 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    The Centers for Disease Control and Prevention (CDC) reports a worldwide (including the United States) CJD incidence rate of approximately 1-2 cases per million population per year, and from 2016-2020 the average annual rate in the United States was approximately 5 cases per million population per year in persons 55 years of age or older. […] In Texas, the average rate of deaths per million population due to CJD over the past 10 years (2013-2022) is 1.2 cases per million population per year. The average rate over two consecutive 5-year periods, 2013-2017 2018-2022, are 0.86 1.46 (cases per million population per year), respectively. There has been an increase in CJD cases over the last five years, and this can be attributed to several factors. […] The intensity of surveillance methods can also influence the reported incidence of CJD, and other prion diseases, and Texas conducts enhanced human prion disease surveillance.
  • #44 Creutzfeldt-Jakob Disease (CJD) – Florida Health CHARTS – Florida Department of Health
    https://www.flhealthcharts.gov/ChartsReports/rdPage.aspx?rdReport=NonVitalIndNoGrpCounts.DataViewer&cid=8605
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. CJD occurs worldwide and the estimated annual incidence in many countries, including the United States, has been reported to be about one case per million population. […] CJD is monitored to prevent and control outbreaks. […] This disease became reportable on June 9, 2003. The first full year of data collection is 2004. Cases occurring in previous years may have been retrospectively identified after becoming reportable. Counts and rates include confirmed and probable cases of Creutzfeldt-Jakob Disease. […] Data presented here are from Merlin, Florida’s web-based reportable disease surveillance system. Note that any data prior to 1992 are not maintained in Merlin and should be interpreted with caution. […] Reports for reportable disease data are produced weekly, monthly, and annually. To access these reports, visit Surveillance Publications. More detailed information on interpreting data can be found in the introduction section of the annual reports.
  • #45 Creutzfeldt-Jakob disease (CJD) – Delaware Health and Social Services – State of Delaware
    https://www.dhss.delaware.gov/dph/epi/cjd.html
    CJD became a reportable condition in Delaware in 2006: Regulations for the Control of Communicable and Other Disease Conditions. […] Purpose of Reporting and Surveillance: […] To monitor trends in the epidemiology of human prion diseases. […] To maximize laboratory confirmation of suspected cases in collaboration with the National Prion Disease Pathology Surveillance Center. […] To detect the emergence of vCJD or novel prion diseases in the United States. […] To prevent potential iatrogenic (acquired) transmission. […] Healthcare providers should contact the Delaware Division of Public Health (DPH), Bureau of Epidemiology to report any suspect case of CJD.
  • #46 Prion Diseases – Wyoming Department of Health
    https://health.wyo.gov/publichealth/infectious-disease-epidemiology-unit/disease/prion-diseases/
    Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a family of rare disorders that affect the brains of both humans and animals. The most common prion disease in humans is Creutzfeldt-Jakob Disease (CJD), which affects approximately 1 in a million people worldwide each year. […] Suspected CJD and other human prion diseases are reportable to public health in Wyoming. If you would like to report a case of human prion disease please visit our Disease Reporting page or call the Infectious Disease Epidemiology unit at 877-996-9000.
  • #47 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD deaths and age-adjusted death rate in the United States indicate an increasing trend in the number of deaths between 1979 and 2017. […] Although not fully understood, additional information suggests that CJD rates in nonwhite groups are lower than in whites. […] The intensity of CJD surveillance increases the number of reported cases, often in countries where CJD epidemics have occurred in the past and where surveillance resources are greatest. […] An increase in surveillance and reporting of CJD is most likely in response to BSE and vCJD. […] Possible factors contributing to an increase of CJD incidence are an aging population, population increase, clinician awareness, and more accurate diagnostic methods.
  • #48 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    The Centers for Disease Control and Prevention (CDC) reports a worldwide (including the United States) CJD incidence rate of approximately 1-2 cases per million population per year, and from 2016-2020 the average annual rate in the United States was approximately 5 cases per million population per year in persons 55 years of age or older. […] In Texas, the average rate of deaths per million population due to CJD over the past 10 years (2013-2022) is 1.2 cases per million population per year. The average rate over two consecutive 5-year periods, 2013-2017 2018-2022, are 0.86 1.46 (cases per million population per year), respectively. There has been an increase in CJD cases over the last five years, and this can be attributed to several factors. […] The intensity of surveillance methods can also influence the reported incidence of CJD, and other prion diseases, and Texas conducts enhanced human prion disease surveillance.
  • #49
    https://biomedgrid.com/fulltext/volume6/epidemiology-of-creutzfeldt-jakob-disease-in-floridian-men-and-women-1988-2012.001050.php
    Creutzfeldt-Jakob disease (CJD) is a rare and fatal prion brain disorder with unknown etiology. […] We examined the epidemiology of CJD in the population of Florida from 1988 to 2012. […] The overall annual incidence was 0.91 per million (95% Confidence Interval: 0.8-1.0) and the overall rate was slightly higher for men than women (0.94 versus 0.87 per million, respectively). […] Annual incidence rates of CJD varied widely across the 25-year study period potentially reflecting fluctuation in unknown environmental exposures. […] Overall, the annual rate was 0.91 per million but rates fluctuated widely during the 25-year study period with divergent patterns for men and women from 2001 to 2008. […] Well-designed case control studies are needed to elucidate environmental triggers of this rare condition.
  • #50 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD deaths and age-adjusted death rate in the United States indicate an increasing trend in the number of deaths between 1979 and 2017. […] Although not fully understood, additional information suggests that CJD rates in nonwhite groups are lower than in whites. […] The intensity of CJD surveillance increases the number of reported cases, often in countries where CJD epidemics have occurred in the past and where surveillance resources are greatest. […] An increase in surveillance and reporting of CJD is most likely in response to BSE and vCJD. […] Possible factors contributing to an increase of CJD incidence are an aging population, population increase, clinician awareness, and more accurate diagnostic methods.
  • #51 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    The Centers for Disease Control and Prevention (CDC) reports a worldwide (including the United States) CJD incidence rate of approximately 1-2 cases per million population per year, and from 2016-2020 the average annual rate in the United States was approximately 5 cases per million population per year in persons 55 years of age or older. […] In Texas, the average rate of deaths per million population due to CJD over the past 10 years (2013-2022) is 1.2 cases per million population per year. The average rate over two consecutive 5-year periods, 2013-2017 2018-2022, are 0.86 1.46 (cases per million population per year), respectively. There has been an increase in CJD cases over the last five years, and this can be attributed to several factors. […] The intensity of surveillance methods can also influence the reported incidence of CJD, and other prion diseases, and Texas conducts enhanced human prion disease surveillance.
  • #52 Creutzfeldt-Jakob Disease (CJD) | Texas DSHS
    https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd
    The combination of a positive RT-QuIC CSF result with neuropsychiatric symptoms allows for decreased reliance on the presence of specific neurologic symptoms to classify a case of CJD. This has permitted the classification of CJD cases that previously may not have been counted. […] The rate of CJD decreased from 2019 to 2020 and then increased from 2020 to 2021 and again from 2021 to 2022. However, small changes in case counts can lead to larger changes in rates, and with increased surveillance in Texas, increased awareness, and ease of ante-mortem testing using a better test, an average rate closer to 1.5 cases per million population per year may be more representative of CJD cases in Texas, which still falls within the worldwide rate of 1-2 cases per million population per year.
  • #53 Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020 – December 12, 2023 – USC Schaeffer
    https://schaeffer.usc.edu/research/change-creutzfeldt-jakob-disease/
    The research examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD. […] Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease. Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described. The incidence of sporadic CJD, the most common type, is higher among older patients. Due to aging populations worldwide, the epidemiology of CJD is evolving. […] We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
  • #54 Creutzfeldt–Jakob disease – Wikipedia
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
    CJD deaths and age-adjusted death rate in the United States indicate an increasing trend in the number of deaths between 1979 and 2017. […] Although not fully understood, additional information suggests that CJD rates in nonwhite groups are lower than in whites. […] The intensity of CJD surveillance increases the number of reported cases, often in countries where CJD epidemics have occurred in the past and where surveillance resources are greatest. […] An increase in surveillance and reporting of CJD is most likely in response to BSE and vCJD. […] Possible factors contributing to an increase of CJD incidence are an aging population, population increase, clinician awareness, and more accurate diagnostic methods.
  • #55 Clinical Overview of Creutzfeldt-Jakob Disease (CJD) | Classic CJD | CDC
    https://www.cdc.gov/creutzfeldt-jakob/hcp/clinical-overview/index.html
    CJD occurs worldwide and the estimated annual incidence in many countries, including the United States, has been reported to be about one to two cases per million population. […] Confirming CJD in a patient requires neuropathologic and/or immunodiagnostic testing of brain tissue obtained either at biopsy or autopsy. […] Not everyone who is thought to have died from prion disease has had their brain tissue analyzed, which is required to confirm prion disease. […] Annual numbers of people with this positive test but no brain tissue analyses can be found on the National Prion Disease Pathology Surveillance Center website (NPDPSC Tables). […] CJD is not related to BSE. Instead, cases occur either sporadically, via healthcare associated infections or via genetic links. […] Surveillance for Creutzfeldt-Jakob Disease, MMWR. August 9, 1996;45(31):665-668.
  • #56 Clinical Overview of Creutzfeldt-Jakob Disease (CJD) | Classic CJD | CDC
    https://www.cdc.gov/creutzfeldt-jakob/hcp/clinical-overview/index.html
    CJD occurs worldwide and the estimated annual incidence in many countries, including the United States, has been reported to be about one to two cases per million population. […] Confirming CJD in a patient requires neuropathologic and/or immunodiagnostic testing of brain tissue obtained either at biopsy or autopsy. […] Not everyone who is thought to have died from prion disease has had their brain tissue analyzed, which is required to confirm prion disease. […] Annual numbers of people with this positive test but no brain tissue analyses can be found on the National Prion Disease Pathology Surveillance Center website (NPDPSC Tables). […] CJD is not related to BSE. Instead, cases occur either sporadically, via healthcare associated infections or via genetic links. […] Surveillance for Creutzfeldt-Jakob Disease, MMWR. August 9, 1996;45(31):665-668.
  • #57 Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: Background, Pathophysiology and Etiology, Epidemiology
    https://emedicine.medscape.com/article/1169688-overview
    Cases of iatrogenic CJD have been identified as resulting from medical treatment, typically from donor blood products obtained from individuals infected with variant CJD. […] The number of cases of variant CJD peaked in 2000 in the United Kingdom at 28 and then steadied at 20 cases in 2001, 17 in 2002, and 18 in 2003. […] Despite the optimism, uncertainty remains about the likely size of the total variant CJD epidemic, because such calculations depend on assumptions, including the mean incubation period in humans or the infectious dose of BSE for humans. […] In contrast, sporadic CJD occurs with a uniform incidence of 1 case per million population per year worldwide. […] In the United States and Europe, surveillance of patients with prion diseases is performed. Therefore, reporting any suspected prion disease, in particular suspected variant CJD, to surveillance agencies is necessary. […] Autopsies are performed in only an estimated 22% of cases of CJD in California. The autopsy rates of suspected cases of CJD should be increased because only a pathologic review of tissue can distinguish between classic and variant forms of CJD.
  • #58 The importance of ongoing international surveillance for Creutzfeldt–Jakob disease | Nature Reviews Neurology
    https://www.nature.com/articles/s41582-021-00488-7
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. […] However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. […] Continued CJD surveillance is a necessity to meet the potential for further cases of vCJD or the emergence of novel prion diseases in humans. […] Creutzfeldt-Jakob disease (CJD) is a transmissible and universally fatal human prion disease; surveillance programmes exist globally to monitor trends in CJD epidemiology and mitigate public health risks.
  • #59 Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: Background, Pathophysiology and Etiology, Epidemiology
    https://emedicine.medscape.com/article/1169688-overview
    A study of 32,441 appendices obtained from 41 hospitals in the United Kingdom, which had been archived over some 6 decades, detected 16 instances of abnormal PrP protein, indicating a prevalence of approximately 1 case in 2,000. […] According to the World Health Organization fact sheet on variant CJD (revised February 2012), 224 total cases of variant CJD were reported worldwide through March 2011: 175 cases in the United Kingdom and 49 cases in other countries (25 in France, 5 in Spain, 4 in Ireland, 3 in the Netherlands, 3 in the United States, 2 in Canada, 2 in Italy, 2 in Portugal, 1 in Japan, 1 in Saudi Arabia, and 1 in Taiwan). […] The vast majority of cases of variant CJD have included documented exposure to food products in countries where bovine spongiform encephalopathy (BSE) occurs, and 2 cases have occurred secondarily as a result of exposure to blood transfusion from individuals who then developed variant CJD.
  • #60 Creutzfeldt-Jakob Disease (CJD) | Doctor
    https://patient.info/doctor/creutzfeldt-jakob-disease
    This disease is notifiable in the UK – see NOIDs article for more detail. […] International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] There is a National CJD Surveillance Unit (NCJDSU) based at the Western General Infirmary in Edinburgh, which brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease. […] The annual NCJDSU report in 2021 reported: Between 1st January 1970 and 31st December 2021, 2909 cases of sCJD were identified in the UK. […] The annual mortality rate for sporadic CJD (sCJD) was 1.97 cases/million in 2021. […] Up to 31st December 2021, 178 cases of definite or probable vCJD had been identified in the UK.
  • #61 Background Information – CEIP
    https://ceip.us/projects/cjd/background-information/
    CJD occurs worldwide with an incidence ranging between 0.5 and 1.5 cases per million people per year. […] Sporadic CJD (sCJD) accounts for greater than 85% of all CJD cases, familial CJD accounts for 10-15% of all cases, and iatrogenic CJD accounts for less than 5% of all CJD cases. vCJD is extremely rare. To date, there have been three cases of vCJD in the United States two due to exposure in the United Kingdom and one due to exposure from Saudi Arabia. There have not been any domestically acquired vCJD cases in the United States. […] The mode of transmission for sporadic CJD is not known. […] There is no evidence of person-to-person transmission of either vCJD or sCJD by casual contact or by the airborne/respiratory route. […] The incubation period for sporadic CJD is unknown. For iatrogenic CJD it varies by route of exposure from 15 months to 30 years. For vCJD the incubation period is unknown but it is estimated to be 10 to 20+ years. […] The National Prion Disease Pathology Surveillance Center does genetic testing for fCJD. […] Creutzfeldt-Jakob Disease (CJD)
  • #62 Variant Creutzfeldt-Jakob Disease (vCJD) – MN Dept. of Health
    https://www.health.state.mn.us/diseases/cjd/vcjd/index.html
    In 1996, a newly recognized variant form of Creutzfeldt-Jakob disease (CJD), called „variant” Creutzfeldt-Jakob disease (vCJD) was reported in the United Kingdom. Epidemiological evidence suggests that vCJD is linked with the consumption of cattle infected with bovine spongiform encephalopathy (BSE or „mad cow” disease). […] Variant CJD has been reported in 11 countries, with the majority of cases reported in the United Kingdom. […] Evidence suggests that 2 were exposed while living in the United Kingdom, and the third was exposed while living in Saudi Arabia.
  • #63 Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: Background, Pathophysiology and Etiology, Epidemiology
    https://emedicine.medscape.com/article/1169688-overview
    Cases of iatrogenic CJD have been identified as resulting from medical treatment, typically from donor blood products obtained from individuals infected with variant CJD. […] The number of cases of variant CJD peaked in 2000 in the United Kingdom at 28 and then steadied at 20 cases in 2001, 17 in 2002, and 18 in 2003. […] Despite the optimism, uncertainty remains about the likely size of the total variant CJD epidemic, because such calculations depend on assumptions, including the mean incubation period in humans or the infectious dose of BSE for humans. […] In contrast, sporadic CJD occurs with a uniform incidence of 1 case per million population per year worldwide. […] In the United States and Europe, surveillance of patients with prion diseases is performed. Therefore, reporting any suspected prion disease, in particular suspected variant CJD, to surveillance agencies is necessary. […] Autopsies are performed in only an estimated 22% of cases of CJD in California. The autopsy rates of suspected cases of CJD should be increased because only a pathologic review of tissue can distinguish between classic and variant forms of CJD.
  • #64 The Epidemiology of Variant Creutzfeldt-Jakob Disease | SpringerLink
    https://link.springer.com/chapter/10.1007/978-3-662-08441-0_7
    The development of a test that could be used on easily accessible tissue to detect infection early in the incubation period would not only advance understanding of the epidemiology of infection with the agent but would also aid the implementation of control measures to prevent potential iatrogenic spread.
  • #65 The importance of ongoing international surveillance for Creutzfeldt-Jakob disease – PubMed
    https://pubmed.ncbi.nlm.nih.gov/33972773/
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. […] Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. […] In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments.
  • #66 Understanding the Best Nutritional Management for Creutzfeldt–Jakob Disease Patients: A Comparison Between East Asian and Western Experiences
    https://www.mdpi.com/2075-1729/14/11/1496
    However, in Japan’s, Taiwan’s, and China’s cultures, in well-organized healthcare systems that include a health insurance system, the majority of patients undergo intensive life-sustaining treatment that prolongs their survival, and some even continue the treatment even if the advanced neurological disorder is irreversible. […] Conversely, in Western countries, due to financial and ethical findings, intensive life-sustaining treatments like tube feeding are not commonly provided to patients with fatal neurologic conditions, such as prion disease.
  • #67 The importance of ongoing international surveillance for Creutzfeldt–Jakob disease | Nature Reviews Neurology
    https://www.nature.com/articles/s41582-021-00488-7
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. […] However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. […] Continued CJD surveillance is a necessity to meet the potential for further cases of vCJD or the emergence of novel prion diseases in humans. […] Creutzfeldt-Jakob disease (CJD) is a transmissible and universally fatal human prion disease; surveillance programmes exist globally to monitor trends in CJD epidemiology and mitigate public health risks.
  • #68 The importance of ongoing international surveillance for Creutzfeldt–Jakob disease | Nature Reviews Neurology
    https://www.nature.com/articles/s41582-021-00488-7
    Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. […] Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. […] However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. […] Continued CJD surveillance is a necessity to meet the potential for further cases of vCJD or the emergence of novel prion diseases in humans. […] Creutzfeldt-Jakob disease (CJD) is a transmissible and universally fatal human prion disease; surveillance programmes exist globally to monitor trends in CJD epidemiology and mitigate public health risks.

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