Zwyrodnienie korowo-podstawne (zespół korowo-podstawny) – Rokowania, prognozy i postęp choroby

Zwyrodnienie korowo-podstawne (zespół korowo-podstawny)
Rokowania, prognozy i postęp choroby

Zwyrodnienie korowo-podstawne (CBD/CBS) to rzadkie schorzenie neurodegeneracyjne charakteryzujące się postępującym pogorszeniem funkcji ruchowych, poznawczych i behawioralnych, z medianą przeżycia od wystąpienia objawów wynoszącą około 7,9 lat (SD 0,7), a od momentu diagnozy około 4,9 lat (SD 0,7). Średni czas trwania choroby w badaniach wynosi 6-8 lat, z dużą zmiennością (3-15 lat). Rokowanie pogarszają czynniki takie jak wczesna obustronna bradykinezja, obecność zespołu czołowego, współwystępowanie drżenia, sztywności i bradykinezji oraz apatia, która jest istotnym predyktorem zgonu w ciągu 2,5 roku. Wiek, płeć i globalne zaburzenia poznawcze nie wykazują istotnego wpływu na przeżycie. Choroba rozpoczyna się zwykle między 50. a 70. rokiem życia i prowadzi do utraty zdolności chodzenia, dysfagii wymagającej karmienia przez zgłębnik oraz nietrzymania moczu.

Rokowanie w zwyrodnieniu korowo-podstawnym (zespole korowo-podstawnym)

Średni czas przeżycia
Czynniki wpływające na rokowanie
Naturalny przebieg choroby
Powikłania wpływające na rokowanie
Wyzwania diagnostyczne a rokowanie
Interwencje wpływające na rokowanie
Biomarkery a prognozowanie przebiegu choroby
Kolejne rozdziały

Rokowanie w zwyrodnieniu korowo-podstawnym (zespole korowo-podstawnym)

Zwyrodnienie korowo-podstawne (CBD, zespół korowo-podstawny, CBS) to rzadkie schorzenie neurodegeneracyjne, które wpływa na funkcje ruchowe, poznawcze i behawioralne. Rokowanie w tym schorzeniu jest ściśle związane z naturalnym przebiegiem choroby, jej progresją oraz występowaniem powikłań.¹²³

Średni czas przeżycia

Badania naukowe dostarczają spójnych danych dotyczących średniego czasu przeżycia pacjentów z CBD/CBS. Według analiz klinicznych mediana przeżycia od momentu wystąpienia objawów wynosi około 7,9 lat (SD 0,7) z zakresem od 2,5 do 12,5 lat. Od momentu pierwszej wizyty lekarskiej średni czas przeżycia szacuje się na około 4,9 lat (SD 0,7) z zakresem od 0,8 do 10 lat.⁴

Inne badania wskazują podobne wyniki przeżywalności:

Średni czas przeżycia od wystąpienia objawów: 6-8 lat⁵⁶
Średni czas przeżycia od momentu diagnozy: 4,6 lat (badanie brytyjskie)⁷
W metaanalizie Kansal i wsp. oszacowano utratę lat życia na 11,33 lat (95% przedział ufności: 9,60-13,06)⁸

W dużym przeglądzie przypadków CBD przeprowadzonym przez Armstrong i wsp. średni czas trwania choroby wynosił 6,6 lat (SD 2,4) z zakresem od 2,0 do 12,5 lat.⁹ Podsumowując dostępne dane, przeciętny czas przeżycia w zwyrodnieniu korowo-podstawnym wynosi 7-8 lat, ale obserwuje się znaczną zmienność w zakresie 3-15 lat.¹⁰¹¹¹²

Czynniki wpływające na rokowanie

Zidentyfikowano kilka czynników wpływających na rokowanie i czas przeżycia pacjentów z CBD/CBS:

Czynniki związane z krótszym przeżyciem:

Wczesne bradykinezja/” title=”bradykinezja” class=”to-tag” data-termid=”31142″>obustronne spowolnienie ruchowe (bradykinezja)¹³
Obecność zespołu czołowego¹⁴
Współwystępowanie co najmniej dwóch z trzech objawów: drżenie, sztywność, bradykinezja¹⁵
Obecność apatii – w brytyjskim badaniu kohortowym apatia była istotnym predyktorem zgonu w ciągu 2,5 roku od oceny i była najwyższa właśnie w zespole korowo-podstawnym w porównaniu z innymi zespołami neurodegeneracyjnymi¹⁶

Czynniki bez istotnego wpływu na przeżycie:

Wiek w momencie badania¹⁷
Płeć¹⁸
Globalne zaburzenia poznawcze¹⁹

Naturalny przebieg choroby

CBD/CBS charakteryzuje się stopniowym pogorszeniem stanu neurologicznego pacjenta. Choroba zazwyczaj rozpoczyna się u osób w wieku 50-70 lat i wykazuje powolną, ale konsekwentną progresję.²⁰²¹

W miarę postępu choroby pacjenci doświadczają nasilenia objawów ruchowych i poznawczych. Ostatecznie większość osób z CBD traci zdolność chodzenia.²² Problemem staje się również dysfagia (trudności w połykaniu), prowadząca do konieczności karmienia przez zgłębnik, oraz nietrzymanie moczu.²³

Powikłania wpływające na rokowanie

Osoby z CBD/CBS mogą rozwijać poważne powikłania, które często przyczyniają się do śmierci. Do najczęstszych należą:

Zapalenie płuc – szczególnie zachłystowe, będące główną przyczyną zgonów w CBD²⁴²⁵
Zatory płucne²⁶
Sepsa – niebezpieczna odpowiedź organizmu na infekcję²⁷
Infekcje dróg moczowych – związane z nietrzymaniem moczu, wymagające właściwej higieny dla profilaktyki²⁸

Wyzwania diagnostyczne a rokowanie

Warto podkreślić, że rokowanie w przypadku pacjentów z klinicznym rozpoznaniem zespołu korowo-podstawnego (CBS) może być trudne do precyzyjnego określenia, ponieważ pod tym samym obrazem klinicznym mogą kryć się różne patologie neurodegeneracyjne. Badania pokazują, że zaledwie 50% pacjentów z klinicznym CBS ma faktycznie CBD w badaniu neuropatologicznym.²⁹

Najczęstsze patologie leżące u podstaw CBS to:³⁰

Zwyrodnienie korowo-podstawne (CBD) – 32% przypadków
Postępujące porażenie nadjądrowe (PSP) – 31% przypadków
Choroba Alzheimera (AD) – 20% przypadków
Inne przyczyny (m.in. otępienie z ciałami Lewy’ego, FTLD-TDP, choroba neuronu ruchowego, choroba Picka, FTLD-FUS, choroba Creutzfeldta-Jakoba, choroby naczyniowe mózgu) – 17% przypadków

Badania genetyczne wskazują na pewne wspólne czynniki ryzyka dla CBD i PSP, w tym warianty genów MAPT i MOBP na chromosomie 3p22, co może częściowo wyjaśniać podobieństwa w rokowaniu obu tych chorób.³¹

Interwencje wpływające na rokowanie

Obecnie nie istnieją terapie lekowe mogące zmodyfikować przebieg zwyrodnienia korowo-podstawnego. Leczenie koncentruje się głównie na łagodzeniu objawów.³² Niemniej jednak, odpowiednio wczesne interwencje mogą poprawić jakość życia i potencjalnie wpłynąć na rokowanie:

Stosowanie zgłębnika do karmienia – może zapobiec aspiracyjnemu zapaleniu płuc, będącemu główną przyczyną zgonów³³
Odpowiednia higiena – niezbędna w profilaktyce zakażeń układu moczowego³⁴
Terapia mowy i terapia zajęciowa – usługi wspomagające, które nie powinny być pomijane w kompleksowej opiece³⁵
Wczesne planowanie opieki – współpraca z lekarzami (POZ i specjalistami) w planowaniu postępowania w późniejszych stadiach choroby³⁶

Biomarkery a prognozowanie przebiegu choroby

Obecnie trudno jest przewidzieć patologię leżącą u podstaw CBS wyłącznie na podstawie cech klinicznych, co utrudnia dokładne prognozowanie przebiegu choroby. Jednak rozwijające się biomarkery molekularne mogą pomóc w różnicowaniu choroby Alzheimera od innych etiologii CBS.³⁷

Aktualne kryteria diagnostyczne dla CBS mają suboptymalne czułość i swoistość, ale nowo powstające biomarkery dają nadzieję na przyszłe udoskonalenia w diagnostyce patologii leżącej u podstaw u pacjentów z CBS.³⁸ Dokładniejsza identyfikacja patologicznego podłoża może przyczynić się do bardziej precyzyjnego określenia rokowania i potencjalnie, w przyszłości, zastosowania bardziej ukierunkowanych metod terapeutycznych, zwłaszcza jeśli pojawią się leczenie ukierunkowane na określone białka.³⁹

Obecnie definitywne rozpoznanie wymaga oceny patologicznej, ale w przyszłości być może będzie możliwe przewidywanie podstawowej patologii za życia w oparciu o kombinację objawów klinicznych i informacji z różnych biomarkerów.⁴⁰

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

Materiały źródłowe

#1 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#2 Corticobasal syndrome: a practical guide | Practical Neurology
https://pn.bmj.com/content/21/4/276
Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. […] Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. […] The prognosis for a patient diagnosed with corticobasal syndrome depends mainly on the underlying neuropathology (i.e. cause), the difficulty being that that cause is not easily determined during life. […] In summary, on present knowledge, average survival in corticobasal syndrome is 7-8 years but with a considerable range of some 3-15 years.
#3 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#4 Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination – PubMed
https://pubmed.ncbi.nlm.nih.gov/9489528/
Objective: To analyse the natural history and survival of corticobasal degeneration by investigating the clinical features of 14 cases confirmed by postmortem examination. […] Median survival time after onset of symptoms was 7.9 (SD 0.7) (range, 2.5-12.5) years, and, after the first clinic visit, 4.9 (SD 0.7) (range, 0.8-10) years. Early bilateral bradykinesia, frontal syndrome, or two out of tremor, rigidity, and bradykinesia, predicted a shorter survival. […] The study also suggests that a focal cognitive and extrapyramidal motor syndrome is indicative of corticobasal degeneration. Survival in corticobasal degeneration was shortened by the early presence of (more) widespread parkinsonian features or frontal lobe syndrome.
#5 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#6 Corticobasal degeneration (corticobasal syndrome) – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767
Corticobasal degeneration (corticobasal syndrome) gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk. […] People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.
#7 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#8 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#9 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#10 Corticobasal syndrome: a practical guide | Practical Neurology
https://pn.bmj.com/content/21/4/276
Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. […] Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. […] The prognosis for a patient diagnosed with corticobasal syndrome depends mainly on the underlying neuropathology (i.e. cause), the difficulty being that that cause is not easily determined during life. […] In summary, on present knowledge, average survival in corticobasal syndrome is 7-8 years but with a considerable range of some 3-15 years.
#11 Corticobasal degeneration | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/corticobasal-degeneration?embed_domain=hackmd.io%25252525252F%252525252540yipuafecsl2jsu8smr5njq%25252525252Fbnjhjgjghjghjghradiopaedia-icon-144.pnglang%3Dus&lang=gb
Corticobasal degeneration (CBD) is an uncommon neurodegenerative disease and is one of the subset of tauopathies. […] Unfortunately, the overall prognosis is poor, with patients demonstrating gradual neurological decline. Death occurs typically 5 to 10 years after the diagnosis is first made. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief. Supportive services such as speech and occupational therapy should not be overlooked.
#12
https://www.nhs.uk/conditions/corticobasal-degeneration/
Corticobasal degeneration (CBD) is a rare condition that can cause gradually worsening problems with movement, speech, memory and swallowing. […] CBD usually changes very slowly. Many people find it helpful to plan ahead with their doctors (GP and specialist) to make decisions about what to do in later stages of the illness. […] As a result of these complications, the average life expectancy for someone with CBD is around 6 to 8 years from when their symptoms start. However, this is only an average and CBD is very variable.
#13 Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination – PubMed
https://pubmed.ncbi.nlm.nih.gov/9489528/
Objective: To analyse the natural history and survival of corticobasal degeneration by investigating the clinical features of 14 cases confirmed by postmortem examination. […] Median survival time after onset of symptoms was 7.9 (SD 0.7) (range, 2.5-12.5) years, and, after the first clinic visit, 4.9 (SD 0.7) (range, 0.8-10) years. Early bilateral bradykinesia, frontal syndrome, or two out of tremor, rigidity, and bradykinesia, predicted a shorter survival. […] The study also suggests that a focal cognitive and extrapyramidal motor syndrome is indicative of corticobasal degeneration. Survival in corticobasal degeneration was shortened by the early presence of (more) widespread parkinsonian features or frontal lobe syndrome.
#14 Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination – PubMed
https://pubmed.ncbi.nlm.nih.gov/9489528/
Objective: To analyse the natural history and survival of corticobasal degeneration by investigating the clinical features of 14 cases confirmed by postmortem examination. […] Median survival time after onset of symptoms was 7.9 (SD 0.7) (range, 2.5-12.5) years, and, after the first clinic visit, 4.9 (SD 0.7) (range, 0.8-10) years. Early bilateral bradykinesia, frontal syndrome, or two out of tremor, rigidity, and bradykinesia, predicted a shorter survival. […] The study also suggests that a focal cognitive and extrapyramidal motor syndrome is indicative of corticobasal degeneration. Survival in corticobasal degeneration was shortened by the early presence of (more) widespread parkinsonian features or frontal lobe syndrome.
#15 Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination – PubMed
https://pubmed.ncbi.nlm.nih.gov/9489528/
Objective: To analyse the natural history and survival of corticobasal degeneration by investigating the clinical features of 14 cases confirmed by postmortem examination. […] Median survival time after onset of symptoms was 7.9 (SD 0.7) (range, 2.5-12.5) years, and, after the first clinic visit, 4.9 (SD 0.7) (range, 0.8-10) years. Early bilateral bradykinesia, frontal syndrome, or two out of tremor, rigidity, and bradykinesia, predicted a shorter survival. […] The study also suggests that a focal cognitive and extrapyramidal motor syndrome is indicative of corticobasal degeneration. Survival in corticobasal degeneration was shortened by the early presence of (more) widespread parkinsonian features or frontal lobe syndrome.
#16 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#17 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#18 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#19 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1150039-overview
Prognosis is variable, with several studies indicating mean survival of 6-8 years from symptom onset; multiple cases with survival of 10 years have been reported. […] One UK study found mean survival of 4.6 years from time of diagnosis. […] In the large number of CBD cases reviewed for the development of diagnostic criteria by Armstrong et al., mean (standard deviation) disease duration was 6.6 (2.4) years with a range of 2.0 to 12.5 years. […] In their meta-analysis, Kansal et al. estimated the number of years of life lost based on mean survival from three studies at 11.33 years, with 95% confidence interval of 9.60 to 13.06. […] In a UK cohort comprised of 29 CBS, 35 PSP, 33 primary progressive aphasia, and 27 behavioral variant Frontotemporal degeneration patients, presence of apathy (as assessed from carer reports using the Apathy Evaluation Scale, Neuropsychiatric Inventory, and Cambridge Behavioral Inventory) predicted death at 2.5 years post-assessment and on average was highest for corticobasal syndrome (CBS). […] Age at assessment, sex, and global cognitive impairment were not significant predictors of survival in this study.
#20 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#21 Corticobasal degeneration | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/corticobasal-degeneration?embed_domain=hackmd.io%25252525252F%252525252540yipuafecsl2jsu8smr5njq%25252525252Fbnjhjgjghjghjghradiopaedia-icon-144.pnglang%3Dus&lang=gb
Corticobasal degeneration (CBD) is an uncommon neurodegenerative disease and is one of the subset of tauopathies. […] Unfortunately, the overall prognosis is poor, with patients demonstrating gradual neurological decline. Death occurs typically 5 to 10 years after the diagnosis is first made. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief. Supportive services such as speech and occupational therapy should not be overlooked.
#22 Corticobasal degeneration (corticobasal syndrome) – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767
Corticobasal degeneration (corticobasal syndrome) gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk. […] People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.
#23 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#24 Corticobasal degeneration (corticobasal syndrome) – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767
Corticobasal degeneration (corticobasal syndrome) gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk. […] People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.
#25 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#26 Corticobasal degeneration (corticobasal syndrome) – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767
Corticobasal degeneration (corticobasal syndrome) gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk. […] People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.
#27 Corticobasal degeneration (corticobasal syndrome) – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767
Corticobasal degeneration (corticobasal syndrome) gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk. […] People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.
#28 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#29 Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy | Nature Communications
https://www.nature.com/articles/ncomms8247
Corticobasal degeneration (CBD) is a neurodegenerative disorder affecting movement and cognition, definitively diagnosed only at autopsy. […] Patients who present with the archetypal corticobasal syndrome (CBS), however, do not necessarily have CBD pathology upon neuropathologic examination. […] This phenotypic variability results in 50% of patients with CBS having CBD at autopsy. […] Recognition of the lack of specificity of the corticobasal syndrome for CBD has promoted increasing interest among clinicians to have autopsy confirmation of patients thought to have CBD. […] Importantly, we show that CBD and PSP share a genetic risk factor other than MAPT, at 3p22 MOBP. […] The results of the current study and those of the PSP GWAS indicate an overlap in genetic risk factors for PSP and CBD, but it appears that there is additional genetic variation that differs between the two disorders. […] In conclusion, this first CBD GWAS identified MAPT and MOBP as shared genetic risk factors between CBD and PSP.
#30 Neuropathology and emerging biomarkers in corticobasal syndrome
https://pmc.ncbi.nlm.nih.gov/articles/PMC9380481/
Corticobasal syndrome (CBS) is a clinical syndrome characterised by progressive asymmetric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomenon. […] Clinical features that can predict the underlying pathology of CBS remain unclear. […] Although the current clinical diagnostic criteria for CBS have suboptimal sensitivity and specificity, emerging biomarkers hold promise for future improvements in the diagnosis of underlying pathology in patients with CBS. […] The most common pathological diagnoses were CBD (32%), followed by PSP (31%), AD (20%) and others (17%), including LBD, FTLD-TDP, motor neuron disease, Picks disease, FTLD-FUS, CJD and cerebrovascular disease. […] It is challenging to predict the underlying pathology of CBS based solely on clinical features, but molecular biomarkers may help differentiate AD from other aetiologies. […] At present, pathological assessment is required for definitive diagnosis, but in the future, it may be possible to predict the underlying pathology during life based on a combination of clinical symptoms and signs with supporting information from various biomarkers.
#31 Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy | Nature Communications
https://www.nature.com/articles/ncomms8247
Corticobasal degeneration (CBD) is a neurodegenerative disorder affecting movement and cognition, definitively diagnosed only at autopsy. […] Patients who present with the archetypal corticobasal syndrome (CBS), however, do not necessarily have CBD pathology upon neuropathologic examination. […] This phenotypic variability results in 50% of patients with CBS having CBD at autopsy. […] Recognition of the lack of specificity of the corticobasal syndrome for CBD has promoted increasing interest among clinicians to have autopsy confirmation of patients thought to have CBD. […] Importantly, we show that CBD and PSP share a genetic risk factor other than MAPT, at 3p22 MOBP. […] The results of the current study and those of the PSP GWAS indicate an overlap in genetic risk factors for PSP and CBD, but it appears that there is additional genetic variation that differs between the two disorders. […] In conclusion, this first CBD GWAS identified MAPT and MOBP as shared genetic risk factors between CBD and PSP.
#32 Corticobasal degeneration | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/corticobasal-degeneration?embed_domain=hackmd.io%25252525252F%252525252540yipuafecsl2jsu8smr5njq%25252525252Fbnjhjgjghjghjghradiopaedia-icon-144.pnglang%3Dus&lang=gb
Corticobasal degeneration (CBD) is an uncommon neurodegenerative disease and is one of the subset of tauopathies. […] Unfortunately, the overall prognosis is poor, with patients demonstrating gradual neurological decline. Death occurs typically 5 to 10 years after the diagnosis is first made. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief. Supportive services such as speech and occupational therapy should not be overlooked.
#33 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#34 Corticobasal degeneration – Wikipedia
https://en.wikipedia.org/wiki/Corticobasal_degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can’t express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
#35 Corticobasal degeneration | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/corticobasal-degeneration?embed_domain=hackmd.io%25252525252F%252525252540yipuafecsl2jsu8smr5njq%25252525252Fbnjhjgjghjghjghradiopaedia-icon-144.pnglang%3Dus&lang=gb
Corticobasal degeneration (CBD) is an uncommon neurodegenerative disease and is one of the subset of tauopathies. […] Unfortunately, the overall prognosis is poor, with patients demonstrating gradual neurological decline. Death occurs typically 5 to 10 years after the diagnosis is first made. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief. Supportive services such as speech and occupational therapy should not be overlooked.
#36
https://www.nhs.uk/conditions/corticobasal-degeneration/
Corticobasal degeneration (CBD) is a rare condition that can cause gradually worsening problems with movement, speech, memory and swallowing. […] CBD usually changes very slowly. Many people find it helpful to plan ahead with their doctors (GP and specialist) to make decisions about what to do in later stages of the illness. […] As a result of these complications, the average life expectancy for someone with CBD is around 6 to 8 years from when their symptoms start. However, this is only an average and CBD is very variable.
#37 Neuropathology and emerging biomarkers in corticobasal syndrome
https://pmc.ncbi.nlm.nih.gov/articles/PMC9380481/
Corticobasal syndrome (CBS) is a clinical syndrome characterised by progressive asymmetric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomenon. […] Clinical features that can predict the underlying pathology of CBS remain unclear. […] Although the current clinical diagnostic criteria for CBS have suboptimal sensitivity and specificity, emerging biomarkers hold promise for future improvements in the diagnosis of underlying pathology in patients with CBS. […] The most common pathological diagnoses were CBD (32%), followed by PSP (31%), AD (20%) and others (17%), including LBD, FTLD-TDP, motor neuron disease, Picks disease, FTLD-FUS, CJD and cerebrovascular disease. […] It is challenging to predict the underlying pathology of CBS based solely on clinical features, but molecular biomarkers may help differentiate AD from other aetiologies. […] At present, pathological assessment is required for definitive diagnosis, but in the future, it may be possible to predict the underlying pathology during life based on a combination of clinical symptoms and signs with supporting information from various biomarkers.
#38 Neuropathology and emerging biomarkers in corticobasal syndrome
https://pmc.ncbi.nlm.nih.gov/articles/PMC9380481/
Corticobasal syndrome (CBS) is a clinical syndrome characterised by progressive asymmetric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomenon. […] Clinical features that can predict the underlying pathology of CBS remain unclear. […] Although the current clinical diagnostic criteria for CBS have suboptimal sensitivity and specificity, emerging biomarkers hold promise for future improvements in the diagnosis of underlying pathology in patients with CBS. […] The most common pathological diagnoses were CBD (32%), followed by PSP (31%), AD (20%) and others (17%), including LBD, FTLD-TDP, motor neuron disease, Picks disease, FTLD-FUS, CJD and cerebrovascular disease. […] It is challenging to predict the underlying pathology of CBS based solely on clinical features, but molecular biomarkers may help differentiate AD from other aetiologies. […] At present, pathological assessment is required for definitive diagnosis, but in the future, it may be possible to predict the underlying pathology during life based on a combination of clinical symptoms and signs with supporting information from various biomarkers.
#39 Corticobasal syndrome: a practical guide | Practical Neurology
https://pn.bmj.com/content/21/4/276
Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. […] Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. […] The prognosis for a patient diagnosed with corticobasal syndrome depends mainly on the underlying neuropathology (i.e. cause), the difficulty being that that cause is not easily determined during life. […] In summary, on present knowledge, average survival in corticobasal syndrome is 7-8 years but with a considerable range of some 3-15 years.
#40 Neuropathology and emerging biomarkers in corticobasal syndrome
https://pmc.ncbi.nlm.nih.gov/articles/PMC9380481/
Corticobasal syndrome (CBS) is a clinical syndrome characterised by progressive asymmetric limb rigidity and apraxia with dystonia, myoclonus, cortical sensory loss and alien limb phenomenon. […] Clinical features that can predict the underlying pathology of CBS remain unclear. […] Although the current clinical diagnostic criteria for CBS have suboptimal sensitivity and specificity, emerging biomarkers hold promise for future improvements in the diagnosis of underlying pathology in patients with CBS. […] The most common pathological diagnoses were CBD (32%), followed by PSP (31%), AD (20%) and others (17%), including LBD, FTLD-TDP, motor neuron disease, Picks disease, FTLD-FUS, CJD and cerebrovascular disease. […] It is challenging to predict the underlying pathology of CBS based solely on clinical features, but molecular biomarkers may help differentiate AD from other aetiologies. […] At present, pathological assessment is required for definitive diagnosis, but in the future, it may be possible to predict the underlying pathology during life based on a combination of clinical symptoms and signs with supporting information from various biomarkers.