Postępujące porażenie nadrdzeniowe
Rokowania, prognozy i postęp choroby

Postępujące porażenie nadjądrowe (PSP) to rzadkie, neurodegeneracyjne schorzenie o niekorzystnym rokowaniu, charakteryzujące się medianą przeżycia od 6 do 7,4 lat od pojawienia się pierwszych objawów w typowej postaci Richarsona. Czas przeżycia może się wahać od 2 do 17 lat, a średni czas od diagnozy wynosi 3-4 lata. Kluczowe czynniki prognostyczne obejmują wiek zachorowania (późniejszy wiek wiąże się z gorszym rokowaniem), płeć (kobiety mają szybszą progresję), wskaźnik obszaru śródmózgowie/most (M/P area ratio), wynik w skali PSPRS oraz tempo progresji choroby. Przebieg choroby cechuje się szybkim pogorszeniem sprawności ruchowej, z koniecznością używania pomocy przy chodzeniu średnio po 3,1 roku oraz unieruchomieniem po około 8,2 latach od wystąpienia objawów.

  1. Rokowanie w postępującym porażeniu nadjądrowym
    1. Czas przeżycia
    2. Główne czynniki rokownicze
    3. Kluczowe etapy progresji choroby
    4. Prognozowanie przeżycia
    5. Przyczyny zgonu
    6. Możliwości poprawy rokowania
    7. Obciążenie chorobą
    8. Kolejne rozdziały

Rokowanie w postępującym porażeniu nadjądrowym

Postępujące porażenie nadjądrowe (Progressive Supranuclear Palsy, PSP) jest rzadkim schorzeniem neurodegeneracyjnym o niekorzystnym rokowaniu. Charakteryzuje się stopniowym pogorszeniem stanu pacjenta, prowadząc do poważnej niepełnosprawności i skrócenia długości życia. Obecnie nie istnieją terapie modyfikujące przebieg choroby, a dostępne leczenie objawowe ma ograniczoną skuteczność.12

Czas przeżycia

Mediana przeżycia pacjentów z PSP typu Richardson (najbardziej typowa postać) wynosi od 6 do 7,4 lat od wystąpienia pierwszych objawów choroby.3 Badania kohortowe pacjentów umierających pod obserwacją kliniczną sugerują, że PSP prowadzi zazwyczaj do zgonu w ciągu około 6 lat od początku choroby, choć obserwowano zakres od 2 do 17 lat. Analiza tablic trwania życia w badaniu kohorty Golbe i współpracowników wykazała medianę czasu trwania choroby wynoszącą 9,7 lat.4 Inne źródła podają, że pacjenci z PSP zazwyczaj umierają w ciągu 6-9 lat od postawienia diagnozy, choć czas ten może być zróżnicowany.5 Średni czas przeżycia wynosi tylko 3-4 lata po diagnozie i 5,3-13,0 lat od początku choroby.6

Główne czynniki rokownicze

Zidentyfikowano kilka kluczowych czynników wpływających na rokowanie w PSP:

  • Wiek zachorowania – późniejszy początek choroby wiąże się z gorszym rokowaniem. Badanie Golbe sugerowało, że młodsi pacjenci mają tendencję do dłuższego przeżycia, choć nie wszystkie badania potwierdzają tę zależność.78
  • Płeć – wykazano, że kobiety progresują do śmierci szybciej niż mężczyźni.9
  • Wskaźnik obszaru śródmózgowie/most (M/P area ratio) – zmniejszony stosunek powierzchni śródmózgowia do mostu w badaniach neuroobrazowych jest predyktorem gorszego rokowania i wcześniejszej instytucjonalizacji.10
  • Wynik w skali PSPRS (Progressive Supranuclear Palsy Rating Scale) na początku obserwacji – wyższy wynik wiąże się z krótszym czasem przeżycia.11
  • Tempo progresji choroby od wystąpienia objawów do pierwszej wizyty – szybsza progresja koreluje z gorszym rokowaniem.12

Kluczowe etapy progresji choroby

Przebieg PSP charakteryzuje się stopniowym pogarszaniem sprawności i osiąganiem określonych kamieni milowych w progresji choroby:

  • Wczesna niepełnosprawność ruchowa – około 50% pacjentów wymaga pomocy przy chodzeniu w ciągu 3 lat od pojawienia się pierwszych objawów.13
  • Konieczność używania pomocy przy chodzeniu – średni czas od wystąpienia pierwszych objawów do konieczności używania laski lub chodzika wynosi 3,1 roku.14
  • Wózek inwalidzki – większość pacjentów z PSP ostatecznie wymaga wózka inwalidzkiego.15
  • Opieka częściowa lub całodobowa – może być wymagana już po 3-4 latach od diagnozy PSP, choć czas ten jest zróżnicowany między pacjentami.16
  • Unieruchomienie – średni czas od wystąpienia objawów do konieczności pozostawania w łóżku lub na krześle wynosi 8,2 roku.17

Prognozowanie przeżycia

Badania dostarczyły narzędzi prognostycznych pozwalających na oszacowanie mediany czasu do śmierci na podstawie czynników klinicznych. Mediana czasu do zgonu waha się od 4,8 lat dla mężczyzny z wynikiem PSPRS 25 i wolną prędkością progresji od wystąpienia objawów do pierwszej wizyty (0,51 punktu PSPRS/miesiąc) do 1,8 roku dla kobiety z wynikiem PSPRS 65 i szybką początkową prędkością (2,25 punktu/miesiąc).18

Opracowano również wygodne, niedrogie i nieinwazyjne narzędzie referencyjne do doradztwa pacjentom z zespołem Richardsona w zakresie przybliżonego czasu do osiągnięcia 13 kamieni milowych zmieniających życie w przebiegu choroby.19 Jest to istotne, ponieważ pacjenci i ich rodziny często pytają nie tylko o przewidywaną długość życia, ale również o czas do osiągnięcia etapów choroby, które mogą wpłynąć na decyzje dotyczące przejścia na emeryturę, zatrudnienia opiekunów, modyfikacji środowiska domowego, przeniesienia do placówki opiekuńczej, decyzji o gastrostomii odżywczej czy przygotowania psychicznego do zaawansowanej niepełnosprawności i śmierci.20

Przyczyny zgonu

Głównymi przyczynami zgonu u pacjentów z PSP są infekcje i powikłania płucne, często związane z unieruchomieniem.21 Najczęstszą przyczyną śmierci jest zachłystowe zapalenie płuc (aspiracyjne zapalenie płuc) spowodowane osłabieniem i brakiem koordynacji mięśni gardła, co prowadzi do przypadkowego przedostawania się pokarmu i płynów do tchawicy i płuc.2223

Pacjenci z PSP mają również zwiększone ryzyko upadków, które mogą skutkować złamaniami kości i urazami głowy. Upadki powodujące poważne obrażenia są częstą przyczyną zgonów wśród osób z PSP.24

Możliwości poprawy rokowania

Mimo że obecnie nie ma możliwości zatrzymania postępu PSP, odpowiednia opieka i wsparcie mogą pomóc pacjentom zachować większą niezależność i poprawić jakość życia:25

  • Wczesna diagnostyka i rozpoczęcie leczenia objawowego mogą poprawić jakość życia.26
  • Pomoc logopedy we wczesnym stadium może zmniejszyć ryzyko zaburzeń połykania i związanych z nimi powikłań.27
  • Interwencje zapobiegające upadkom mogą zmniejszyć ryzyko urazów i złamań.
  • Odpowiednia opieka i asystowanie umożliwiają dłuższe zachowanie niezależności.

Obciążenie chorobą

Główne obciążenie chorobą związane jest z zaburzeniami równowagi prowadzącymi do unieruchomienia, choć otępienie, objawy wzrokowe i dysfagia stanowią również istotne problemy.28 Rokowanie w PSP jest niekorzystne, z podwyższonym ryzykiem upadków, zaburzeń połykania, zachłystowego zapalenia płuc i odleżyn, co prowadzi do instytucjonalizacji i krótkiego czasu przeżycia.29

Badania nad nowymi metodami leczenia dają nadzieję, że w przyszłości możliwe będzie zatrzymanie postępu choroby, jednak obecnie najważniejszym elementem opieki jest wczesna diagnostyka, leczenie objawowe i odpowiednie wsparcie interdyscyplinarne.30

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.

  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #2
    https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/
    There’s currently nothing that can be done to stop PSP gradually worsening, although research into new treatments gives hope that this may be possible in the future. […] Good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications. […] Difficulty swallowing can cause choking or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life threatening. […] Help from a speech and language therapist at an early stage can lower this risk for as long as possible.
  • #3 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Median survival in progressive supranuclear palsy (PSP)-Richardson’s syndrome is reported by various studies as 6 to 7.4 years from symptom onset. […] Experience suggests that from the time they first understand that PSP is a progressive and poorly treatable condition, patients and families are interested in specific prognostic information. […] Many patients and families request not only an estimate of survival duration but also of time to disease milestones that may influence decisions to retire from work, hire caregivers, alter the home environment, move to a seniors-oriented or institutional living arrangement, decide on a feeding gastrostomy and not least, prepare psychologically for advanced disability and death. […] For death as the outcome milestone, we found a predictive relationship not only for baseline PSPRS but also for both onset-to-baseline progression velocity and sex, with women progressing to death more quickly than men.
  • #4 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #5 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #6 Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy | BMC Neurology | Full Text
    https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-01692-6
    Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. […] The prognosis of PSP is poor with increased risks of falls, dysphagia, aspiration pneumonia and pressure ulcer, leading to institutionalization and short survival time. […] The mean survival is only 3 to 4 years after the diagnosis and 5.3 to 13.0 years at the onset of the disease. […] Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. […] Older AAO and decreased M/P area ratio were predictors for earlier death and institutionalization in PSP. […] The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.
  • #7 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #8 Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy | BMC Neurology | Full Text
    https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-01692-6
    Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. […] The prognosis of PSP is poor with increased risks of falls, dysphagia, aspiration pneumonia and pressure ulcer, leading to institutionalization and short survival time. […] The mean survival is only 3 to 4 years after the diagnosis and 5.3 to 13.0 years at the onset of the disease. […] Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. […] Older AAO and decreased M/P area ratio were predictors for earlier death and institutionalization in PSP. […] The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.
  • #9 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Median survival in progressive supranuclear palsy (PSP)-Richardson’s syndrome is reported by various studies as 6 to 7.4 years from symptom onset. […] Experience suggests that from the time they first understand that PSP is a progressive and poorly treatable condition, patients and families are interested in specific prognostic information. […] Many patients and families request not only an estimate of survival duration but also of time to disease milestones that may influence decisions to retire from work, hire caregivers, alter the home environment, move to a seniors-oriented or institutional living arrangement, decide on a feeding gastrostomy and not least, prepare psychologically for advanced disability and death. […] For death as the outcome milestone, we found a predictive relationship not only for baseline PSPRS but also for both onset-to-baseline progression velocity and sex, with women progressing to death more quickly than men.
  • #10 Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy | BMC Neurology | Full Text
    https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-01692-6
    Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. […] The prognosis of PSP is poor with increased risks of falls, dysphagia, aspiration pneumonia and pressure ulcer, leading to institutionalization and short survival time. […] The mean survival is only 3 to 4 years after the diagnosis and 5.3 to 13.0 years at the onset of the disease. […] Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. […] Older AAO and decreased M/P area ratio were predictors for earlier death and institutionalization in PSP. […] The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.
  • #11 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Progressive supranuclear palsy (PSP) causes major disability, shortens life, and as yet has no disease-modifying and little symptomatic treatment. A convenient prognostic tool is needed to assist patients, families, and clinicians in planning care. […] We derived median times to milestones, with 25th and 75th percentiles and 95% confidence intervals of the median for baseline PSPRS scores from 25 to 65 (scale range, 0-100). […] Median time to death ranged from 4.8 years for a man with PSPRS score of 25 and a slow progression velocity from onset to initial visit of 0.51 PSPRS points/month to 1.8 years for a woman with PSPRS 65 and rapid initial velocity of 2.25 points/month. […] We have created a convenient, inexpensive, noninvasive reference for counseling patients with PSP-Richardson syndrome on approximate time to encountering 13 life-altering disease milestones.
  • #12 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Progressive supranuclear palsy (PSP) causes major disability, shortens life, and as yet has no disease-modifying and little symptomatic treatment. A convenient prognostic tool is needed to assist patients, families, and clinicians in planning care. […] We derived median times to milestones, with 25th and 75th percentiles and 95% confidence intervals of the median for baseline PSPRS scores from 25 to 65 (scale range, 0-100). […] Median time to death ranged from 4.8 years for a man with PSPRS score of 25 and a slow progression velocity from onset to initial visit of 0.51 PSPRS points/month to 1.8 years for a woman with PSPRS 65 and rapid initial velocity of 2.25 points/month. […] We have created a convenient, inexpensive, noninvasive reference for counseling patients with PSP-Richardson syndrome on approximate time to encountering 13 life-altering disease milestones.
  • #13 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #14 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #15 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #16 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #17 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #18 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Progressive supranuclear palsy (PSP) causes major disability, shortens life, and as yet has no disease-modifying and little symptomatic treatment. A convenient prognostic tool is needed to assist patients, families, and clinicians in planning care. […] We derived median times to milestones, with 25th and 75th percentiles and 95% confidence intervals of the median for baseline PSPRS scores from 25 to 65 (scale range, 0-100). […] Median time to death ranged from 4.8 years for a man with PSPRS score of 25 and a slow progression velocity from onset to initial visit of 0.51 PSPRS points/month to 1.8 years for a woman with PSPRS 65 and rapid initial velocity of 2.25 points/month. […] We have created a convenient, inexpensive, noninvasive reference for counseling patients with PSP-Richardson syndrome on approximate time to encountering 13 life-altering disease milestones.
  • #19 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Progressive supranuclear palsy (PSP) causes major disability, shortens life, and as yet has no disease-modifying and little symptomatic treatment. A convenient prognostic tool is needed to assist patients, families, and clinicians in planning care. […] We derived median times to milestones, with 25th and 75th percentiles and 95% confidence intervals of the median for baseline PSPRS scores from 25 to 65 (scale range, 0-100). […] Median time to death ranged from 4.8 years for a man with PSPRS score of 25 and a slow progression velocity from onset to initial visit of 0.51 PSPRS points/month to 1.8 years for a woman with PSPRS 65 and rapid initial velocity of 2.25 points/month. […] We have created a convenient, inexpensive, noninvasive reference for counseling patients with PSP-Richardson syndrome on approximate time to encountering 13 life-altering disease milestones.
  • #20 A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7396865/
    Median survival in progressive supranuclear palsy (PSP)-Richardson’s syndrome is reported by various studies as 6 to 7.4 years from symptom onset. […] Experience suggests that from the time they first understand that PSP is a progressive and poorly treatable condition, patients and families are interested in specific prognostic information. […] Many patients and families request not only an estimate of survival duration but also of time to disease milestones that may influence decisions to retire from work, hire caregivers, alter the home environment, move to a seniors-oriented or institutional living arrangement, decide on a feeding gastrostomy and not least, prepare psychologically for advanced disability and death. […] For death as the outcome milestone, we found a predictive relationship not only for baseline PSPRS but also for both onset-to-baseline progression velocity and sex, with women progressing to death more quickly than men.
  • #21 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #22 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #23
    https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/
    There’s currently nothing that can be done to stop PSP gradually worsening, although research into new treatments gives hope that this may be possible in the future. […] Good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications. […] Difficulty swallowing can cause choking or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life threatening. […] Help from a speech and language therapist at an early stage can lower this risk for as long as possible.
  • #24 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #25
    https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/
    There’s currently nothing that can be done to stop PSP gradually worsening, although research into new treatments gives hope that this may be possible in the future. […] Good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications. […] Difficulty swallowing can cause choking or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life threatening. […] Help from a speech and language therapist at an early stage can lower this risk for as long as possible.
  • #26 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy
    The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isnt treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be. […] Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person. […] Over time, PSP complications can be fatal. […] People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary. […] PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs. […] People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
  • #27
    https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/
    There’s currently nothing that can be done to stop PSP gradually worsening, although research into new treatments gives hope that this may be possible in the future. […] Good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications. […] Difficulty swallowing can cause choking or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life threatening. […] Help from a speech and language therapist at an early stage can lower this risk for as long as possible.
  • #28 Progressive Supranuclear Palsy: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1151430-overview
    Studies of cohort patients dying under surveillance suggest that PSP is usually fatal within approximately 6 years of onset (range, 2-17 years); life table analysis among the entire cohort of Golbe et al revealed a median disease duration of 9.7 years. […] Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies. […] The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. […] Often, the primary morbidity relates to imbalance leading to immobility, though dementia, visual symptoms, and dysphagia are major concerns. […] About 50% of patients require some aid to walk within 3 years of the initial onset of symptoms. […] The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.
  • #29 Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy | BMC Neurology | Full Text
    https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-01692-6
    Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. […] The prognosis of PSP is poor with increased risks of falls, dysphagia, aspiration pneumonia and pressure ulcer, leading to institutionalization and short survival time. […] The mean survival is only 3 to 4 years after the diagnosis and 5.3 to 13.0 years at the onset of the disease. […] Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. […] Older AAO and decreased M/P area ratio were predictors for earlier death and institutionalization in PSP. […] The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.
  • #30
    https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/
    There’s currently nothing that can be done to stop PSP gradually worsening, although research into new treatments gives hope that this may be possible in the future. […] Good care and assistance can help someone with PSP to be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications. […] Difficulty swallowing can cause choking or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life threatening. […] Help from a speech and language therapist at an early stage can lower this risk for as long as possible.

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