Atrofia wieloukładowa
Rokowania, prognozy i postęp choroby

Atrofia wieloukładowa (MSA) to nieuleczalna, neurodegeneracyjna choroba charakteryzująca się postępującym pogorszeniem funkcji neurologicznych i autonomicznych, z medianą przeżycia wynoszącą od 6 do 10 lat (np. 8,6 lat z 95% CI: 6,7-10,2). Kluczowymi czynnikami prognostycznymi są wczesne wystąpienie ciężkiej niewydolności autonomicznej (CASS ≥6), zwłaszcza konieczność cewnikowania pęcherza moczowego, które zwiększa ryzyko zgonu (HR=7,9; P=0,004) i skraca medianę przeżycia do około 7,3 lat. Dodatkowo, istotne są objawy ruchowe (UMSARS), objawy osiowe (upadki, dysfagia), zaburzenia poznawcze oraz starszy wiek przy wystąpieniu pierwszych objawów (HR=1,04; P=0,03). Hipotensja ortostatyczna z obniżeniem ciśnienia skurczowego ≥30 mmHg również koreluje z wyższą śmiertelnością.

Prognozy przeżycia w atrofii wieloukładowej

Atrofia wieloukładowa (MSA) jest nieuleczalną, neurodegeneracyjną chorobą prowadzącą do postępującego pogorszenia funkcji neurologicznych i autonomicznych, która ostatecznie prowadzi do śmierci. Zrozumienie czynników prognostycznych dla tej choroby ma kluczowe znaczenie dla optymalizacji monitorowania przebiegu schorzenia oraz opracowania nowych strategii neuroprotekcyjnych.1

Średni czas przeżycia

Dane dotyczące średniego czasu przeżycia pacjentów z MSA różnią się w zależności od badanej populacji, jednak większość badań wskazuje na okres od 6 do 10 lat od momentu wystąpienia pierwszych objawów.23 Dokładne wartości uzyskane w poszczególnych badaniach to:

  • 8,6 [6,7-10,2] lat (mediana z 95% przedziałem ufności) według jednego z badań4
  • 6,31 lat (mediana) w dużej populacji chińskich pacjentów z MSA56
  • 8,0 lat (mediana) w innym badaniu, bez istotnej statystycznie różnicy między podtypami MSA-P (9,0 lat) i MSA-C (8,0 lat)7

W mniej nasilonych przypadkach pacjenci mogą przeżyć nawet do 15 lat, natomiast w bardzo ciężkich przypadkach czas przeżycia może być znacznie krótszy.8 Należy podkreślić, że MSA charakteryzuje się gorszą prognozą niż choroba Parkinsona, z którą niekiedy jest mylona we wczesnych stadiach.9

Kliniczne czynniki prognostyczne

Badania identyfikują kilka kluczowych czynników klinicznych, które są związane z krótszym czasem przeżycia pacjentów z MSA:

Zaburzenia autonomiczne

Wczesne wystąpienie ciężkiej, uogólnionej niewydolności autonomicznej jest jednym z najważniejszych czynników prognostycznych. Pacjenci z wczesnym i znacznym uszkodzeniem układu autonomicznego (ocena CASS ≥6) mają ponad trzykrotnie zwiększone ryzyko krótszego przeżycia.10 Mediana przeżycia pacjentów z wczesną, uogólnioną niewydolnością autonomiczną wynosi około 7,0 [3,9-9,8] lat w porównaniu do 9,8 [4,6-13,8] lat u pacjentów bez tej cechy klinicznej (P=0,036).11

Szczególnie istotnym czynnikiem jest wczesna konieczność cewnikowania pęcherza moczowego, która wiąże się z medianą przeżycia 7,3 [3,1-10,2] lat, w porównaniu do 13,7 [8,5-14,9] lat u pacjentów bez tej konieczności (P=0,003).12 W analizie proporcjonalnego hazardu Coxa, wczesna konieczność cewnikowania pęcherza moczowego zwiększała ryzyko zgonu prawie ośmiokrotnie (HR=7,9 [1,88-38,63]; P=0,004).13

Również znaczący spadek ciśnienia skurczowego (≥30 mmHg) w hipotensji ortostatycznej istotnie prognozuje wyższą śmiertelność u pacjentów z MSA.14

Objawy ruchowe i osiowe

Bardziej nasilone objawy ruchowe oceniane w skali UMSARS oraz objawy osiowe, takie jak upadki i dysfagia, są związane z gorszym przeżyciem.15 Analiza składowych głównych (PCA) wykazała, że składowa główna związana z funkcjami motorycznymi (PC1) była istotnym predyktorem krótszego czasu przeżycia (HR=1,71, 95% CI: 1,26-2,30, p<0,001).16

Zaburzenia poznawcze

Upośledzenie funkcji poznawczych również koreluje z gorszą prognozą przeżycia w MSA.17 Składowa główna związana z funkcjami niemotorycznymi (PC3) była istotnym czynnikiem predykcyjnym krótszego czasu przeżycia (HR=1,68, 95% CI: 1,31-2,10, p<0,001).18

Wiek zachorowania

Starszy wiek w momencie pojawienia się pierwszych objawów jest niezależnym czynnikiem prognostycznym krótszego przeżycia (HR=1,04 [1,01-1,08]; P=0,03).1920

Biomarkery laboratoryjne jako wskaźniki prognostyczne

Niedawne badania wykazały, że pewne parametry laboratoryjne mogą służyć jako biomarkery prognostyczne w MSA. Można je podzielić na dwie główne kategorie:

Markery stanu zapalnego

Markery wskazujące na zwiększony stan zapalny w organizmie są związane z wyższą śmiertelnością w MSA:2122

Markery stanu odżywienia

Parametry wskazujące na gorszy stan odżywienia również korelują z wyższą śmiertelnością:2324

Obserwacje te sugerują, że dokładne monitorowanie stanu zapalnego i stanu odżywienia od wczesnego etapu choroby może mieć istotne znaczenie dla prognozy pacjentów z MSA.25

Analiza grupowania i identyfikacja podgrup pacjentów

Analiza grupowania (cluster analysis) pozwoliła na identyfikację podgrup pacjentów z MSA o różnym rokowaniu. Badania wykazały, że określone grupy (Klaster 3, HR=4,15, 95% CI: 1,73-9,90, p=0,001 oraz Klaster 4, HR=4,18, 95% CI: 1,73-10,1, p=0,002) były niezależnie związane z krótszym czasem przeżycia.26 Grupy te charakteryzowały się bardziej nasilonymi objawami ruchowymi, objawami osiowymi, hipotensją ortostatyczną i zaburzeniami poznawczymi.27

Potencjalne mechanizmy wpływające na prognozę

Istnieje kilka potencjalnych mechanizmów wyjaśniających wpływ zidentyfikowanych czynników na przeżycie w MSA:

  • Wczesna dysfunkcja pęcherza moczowego może prowadzić do nawracających infekcji układu moczowego z możliwym uszkodzeniem nerek wskutek refluksu pęcherzowo-moczowodowego28
  • Wczesna dysfunkcja pęcherza i nasilona niewydolność autonomiczna mogą odzwierciedlać poważniejszą utratę neuronów serotoninergicznych, które uczestniczą w regulacji autonomicznej i chemowrażliwości oddechowej29
  • Stan zapalny organizmu może przyspieszać neurodegenerację i pogarszać ogólny stan zdrowia30
  • Niedożywienie może zmniejszać odporność na infekcje i ogólną zdolność organizmu do radzenia sobie z postępującą chorobą31

Znaczenie wczesnej diagnostyki różnicowej

Ze względu na podobieństwo wczesnych objawów MSA do choroby Parkinsona (PD), szczególnie we wczesnych stadiach, opracowanie biomarkerów neuroobrazowych, które mogą rozróżnić te dwie choroby z wysoką dokładnością na poziomie indywidualnego pacjenta, ma duże znaczenie kliniczne.32 Badania łączące metryki pochodne tensora dyfuzji i uczenie maszynowe wykazały obiecujące wyniki w rozróżnianiu pacjentów z MSA i PD.33

Analizy siły połączeń strukturalnych między strukturami podkorowymi, mierzone jako liczba strumieni (NOS) wyprowadzonych z traktografii, wykazały potencjał do prawidłowego rozróżniania pacjentów z MSA i PD z zadowalającą dokładnością (0,78-0,84).34 Te metody mogą okazać się bardziej przydatne niż metryki pochodne tensora dyfuzji do wykrywania MSA.35

Ogólna prognoza i przebieg choroby

Prognoza dla pacjentów z MSA jest niepomyślna. Objawy choroby ulegają postępującemu pogorszeniu i zawsze prowadzą do zaburzenia funkcji organizmu, co skutkuje śmiertelnymi powikłaniami.36 Około połowa pacjentów z MSA wymaga pomocy przy chodzeniu w ciągu kilku lat od początku choroby.37

Przebieg choroby jest szybki i prowadzi do całkowitej niepełnosprawności, z zazwyczaj brakiem odpowiedzi na leczenie dopaminergiczne, co odróżnia MSA od choroby Parkinsona.38 Ten agresywny przebieg podkreśla konieczność badań nad nowymi metodami terapeutycznymi, które mogłyby wydłużyć przeżycie i poprawić jakość życia pacjentów z MSA.39

Znaczenie identyfikacji czynników prognostycznych

Identyfikacja czynników prognostycznych MSA ma istotne znaczenie z kilku powodów:

  • Pozwala na wcześniejsze rozpoznanie pacjentów o potencjalnie gorszym rokowaniu40
  • Umożliwia lepsze projektowanie interwencji terapeutycznych w celu wydłużenia przeżycia i poprawy jakości życia41
  • Może pomóc w optymalizacji monitorowania choroby i opracowaniu nowych strategii neuroprotekcyjnych42
  • Wspiera właściwe poradnictwo dla pacjentów i ich rodzin odnośnie prognozowanego przebiegu choroby43

Podsumowując, atrofia wieloukładowa jest chorobą o niepomyślnej prognozie, z medianą przeżycia wynoszącą około 6-10 lat. Wczesne wystąpienie ciężkiej niewydolności autonomicznej, zwłaszcza zaburzeń pęcherza moczowego, objawy osiowe, zaburzenia poznawcze oraz biomarkery stanu zapalnego i odżywienia są istotnymi czynnikami prognostycznymi. Monitorowanie tych parametrów od wczesnego etapu choroby może mieć kluczowe znaczenie dla optymalizacji opieki nad pacjentami z MSA.44

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  1. 10.04.2026
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Materiały źródłowe

  • #1 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    To elucidate the biomarkers related to survival in multiple system atrophy(MSA), we analyzed the predictability of retrospectively collected blood markers for survival in 650 probable MSA. High absolute neutrophil count, red-cell distribution width, C-reactive protein, erythrocyte sedimentation rate, and low hemoglobin, protein, albumin, and creatinine were correlated with higher mortality in MSA. Systemic alteration in inflammation and nutritional status in the early stage are associated with higher mortality in MSA. […] Thus, it is important to establish a biomarker that predicts the prognosis in the early phase of the disease to optimize disease monitoring and to develop novel neuroprotective strategies. […] Among clinical biomarkers, early autonomic failure, older age of onset, and absence of response to levodopa have been associated with shorter survival.
  • #2 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. […] Survival (median [95% confidence interval]) was 8.6 [6.7-10.2] years. […] Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score 6) autonomic failure (7.0 [3.9-9.8] vs. 9.8 [4.6-13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1-10.2] vs. 13.7 [8.5-14.9] years; P=0.003) compared to those without these clinical features. […] On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01-1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88-38.63]; P=0.004) and early generalized (composite autonomic severity score 6) autonomic failure (2.8 [1.01-9.26]; P=0.047).
  • #3 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #4 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. […] Survival (median [95% confidence interval]) was 8.6 [6.7-10.2] years. […] Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score 6) autonomic failure (7.0 [3.9-9.8] vs. 9.8 [4.6-13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1-10.2] vs. 13.7 [8.5-14.9] years; P=0.003) compared to those without these clinical features. […] On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01-1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88-38.63]; P=0.004) and early generalized (composite autonomic severity score 6) autonomic failure (2.8 [1.01-9.26]; P=0.047).
  • #5 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #6 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    More serious motor symptoms, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment were associated with poor survival in MSA via PCA and cluster analysis. […] Our study on a large Chinese MSA patient population confirmed the median survival time of Chinese MSA patients was 6.31 years. […] We identified several independent survival factors of MSA including more serious motor symptoms based on UMSARS scores, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment via PCA and cluster analysis.
  • #7 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In laboratory biomarkers, there is growing evidence that biomarkers reflecting high systemic inflammation are associated with disease severity and progression of MSA. […] Furthermore, recent studies revealed that biomarkers associated with neuronal damage and malnutrition are associated with disease severity, low quality of life, and high mortality in MSA. […] The median survival duration from the onset was 8.0 years. […] The median survival of MSA-P patients and MSA-C patients were 9.0 and 8.0 years, respectively, with no statistical difference between the two subtypes. […] With a cut-off of mean value of laboratory markers within MSA patients, high WBC, ANC, RDW, CRP, and ESR values were significantly related to higher mortality. […] Low (mean) lymphocyte, Hb, total protein, albumin, creatinine, calcium, and GPT were related to higher mortality.
  • #8 Multiple System Atrophy (MSA): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17250-multiple-system-atrophy
    Multiple system atrophy is a degenerative brain disease, meaning it causes parts of your brain to deteriorate. This disease is usually fatal within 10 years, but may have a shorter or longer life expectancy depending on severity. […] MSA is a rare neurological disease that causes certain brain areas to deteriorate. This disease is ultimately fatal. […] People who have MSA will usually develop movement-related symptoms first. This condition gets progressively worse over time. About half of people with this condition need help walking within this time frame. […] The average survival time for this condition is six to 10 years. In less severe cases, people can survive up to 15 years. However, in very severe cases, survival time may be much lower. […] The outlook for MSA is poor. The symptoms of this condition get progressively worse and always disrupt body function, leading to deadly complications.
  • #9 Differentiation of multiple system atrophy from Parkinson’s disease by structural connectivity derived from probabilistic tractography | Scientific Reports
    https://www.nature.com/articles/s41598-019-52829-8
    Recent studies combining diffusion tensor-derived metrics and machine learning have shown promising results in the discrimination of multiple system atrophy (MSA) and Parkinsons disease (PD) patients. […] The aim of this work is assessing whether the strength of structural connectivity between subcortical structures, measured as the number of streamlines (NOS) derived from tractography, can be used to classify MSA and PD patients at the single-patient level. […] Our findings suggest that structural connectivity derived from tractography has the potential to correctly distinguish between MSA and PD patients. Furthermore, NOS measures obtained from tractography might be more useful than diffusion tensor-derived metrics for the detection of MSA. […] Although MSA may resemble Parkinsons disease (PD) in its early stages, brain damage is more aggressive, with usually no response to dopaminergic medication, and leading to a rapidly progressive disease course with a fatal prognosis.
  • #10 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Our data suggests that early development of severe generalized autonomic failure more than triples the risk of shorter survival in patients with MSA. […] Factors predicting survival in patients with MSA are not fully established and the role of early autonomic failure as a prognostic indicator remains controversial. […] Our hypothesis was that early generalized autonomic failure is an independent risk factor of shorter survival in patients with MSA. […] The most relevant findings are that: 1) autonomic involvement starts early in a substantial proportion of patients with MSA; and 2) both development of severe and generalized autonomic failure at early stages of disease and older age at onset predict shorter survival. […] Early moderate-to-severe generalized autonomic failure as defined by CASS 6 was a risk factor for mortality.
  • #11 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. […] Survival (median [95% confidence interval]) was 8.6 [6.7-10.2] years. […] Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score 6) autonomic failure (7.0 [3.9-9.8] vs. 9.8 [4.6-13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1-10.2] vs. 13.7 [8.5-14.9] years; P=0.003) compared to those without these clinical features. […] On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01-1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88-38.63]; P=0.004) and early generalized (composite autonomic severity score 6) autonomic failure (2.8 [1.01-9.26]; P=0.047).
  • #12 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. […] Survival (median [95% confidence interval]) was 8.6 [6.7-10.2] years. […] Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score 6) autonomic failure (7.0 [3.9-9.8] vs. 9.8 [4.6-13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1-10.2] vs. 13.7 [8.5-14.9] years; P=0.003) compared to those without these clinical features. […] On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01-1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88-38.63]; P=0.004) and early generalized (composite autonomic severity score 6) autonomic failure (2.8 [1.01-9.26]; P=0.047).
  • #13 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. […] Survival (median [95% confidence interval]) was 8.6 [6.7-10.2] years. […] Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score 6) autonomic failure (7.0 [3.9-9.8] vs. 9.8 [4.6-13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1-10.2] vs. 13.7 [8.5-14.9] years; P=0.003) compared to those without these clinical features. […] On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01-1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88-38.63]; P=0.004) and early generalized (composite autonomic severity score 6) autonomic failure (2.8 [1.01-9.26]; P=0.047).
  • #14 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In orthostatic hypotension, sBP drop 30 significantly predicted higher mortality in MSA patients. […] The current study presents novel findings regarding high inflammation and poor nutritional status associated with higher mortality in MSA. […] In this study, we showed that low albumin, protein, and hemoglobin, which reflect poor nutritional status, were associated with higher mortality in MSA. […] These observations suggest that close observation of the nutritional state from an early stage of the disease would be important in the prognosis of MSA. […] In conclusion, a systemic alteration in inflammation and nutritional status was associated with a poor prognosis in MSA. Thus, close observation of inflammation and nutritional state from an early stage of the disease would be important in the prognosis of MSA.
  • #15 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    More serious motor symptoms, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment were associated with poor survival in MSA via PCA and cluster analysis. […] Our study on a large Chinese MSA patient population confirmed the median survival time of Chinese MSA patients was 6.31 years. […] We identified several independent survival factors of MSA including more serious motor symptoms based on UMSARS scores, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment via PCA and cluster analysis.
  • #16 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #17 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    More serious motor symptoms, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment were associated with poor survival in MSA via PCA and cluster analysis. […] Our study on a large Chinese MSA patient population confirmed the median survival time of Chinese MSA patients was 6.31 years. […] We identified several independent survival factors of MSA including more serious motor symptoms based on UMSARS scores, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment via PCA and cluster analysis.
  • #18 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #19 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    Neurologic and autonomic presentation in multiple system atrophy (MSA) may predict early mortality. […] Survival (median [95% confidence interval]) was 8.6 [6.7-10.2] years. […] Survival was shorter in patients with early laboratory evidence of generalized (composite autonomic severity score 6) autonomic failure (7.0 [3.9-9.8] vs. 9.8 [4.6-13.8] years; P=0.036), and early requirement of bladder catheterization (7.3 [3.1-10.2] vs. 13.7 [8.5-14.9] years; P=0.003) compared to those without these clinical features. […] On Cox proportional analysis, prognostic indicators of shorter survival were older age at onset (hazard ratio [95% confidence interval], 1.04 [1.01-1.08]; P=0.03), early requirement of bladder catheterization (7.9 [1.88-38.63]; P=0.004) and early generalized (composite autonomic severity score 6) autonomic failure (2.8 [1.01-9.26]; P=0.047).
  • #20 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    To elucidate the biomarkers related to survival in multiple system atrophy(MSA), we analyzed the predictability of retrospectively collected blood markers for survival in 650 probable MSA. High absolute neutrophil count, red-cell distribution width, C-reactive protein, erythrocyte sedimentation rate, and low hemoglobin, protein, albumin, and creatinine were correlated with higher mortality in MSA. Systemic alteration in inflammation and nutritional status in the early stage are associated with higher mortality in MSA. […] Thus, it is important to establish a biomarker that predicts the prognosis in the early phase of the disease to optimize disease monitoring and to develop novel neuroprotective strategies. […] Among clinical biomarkers, early autonomic failure, older age of onset, and absence of response to levodopa have been associated with shorter survival.
  • #21 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In laboratory biomarkers, there is growing evidence that biomarkers reflecting high systemic inflammation are associated with disease severity and progression of MSA. […] Furthermore, recent studies revealed that biomarkers associated with neuronal damage and malnutrition are associated with disease severity, low quality of life, and high mortality in MSA. […] The median survival duration from the onset was 8.0 years. […] The median survival of MSA-P patients and MSA-C patients were 9.0 and 8.0 years, respectively, with no statistical difference between the two subtypes. […] With a cut-off of mean value of laboratory markers within MSA patients, high WBC, ANC, RDW, CRP, and ESR values were significantly related to higher mortality. […] Low (mean) lymphocyte, Hb, total protein, albumin, creatinine, calcium, and GPT were related to higher mortality.
  • #22 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In orthostatic hypotension, sBP drop 30 significantly predicted higher mortality in MSA patients. […] The current study presents novel findings regarding high inflammation and poor nutritional status associated with higher mortality in MSA. […] In this study, we showed that low albumin, protein, and hemoglobin, which reflect poor nutritional status, were associated with higher mortality in MSA. […] These observations suggest that close observation of the nutritional state from an early stage of the disease would be important in the prognosis of MSA. […] In conclusion, a systemic alteration in inflammation and nutritional status was associated with a poor prognosis in MSA. Thus, close observation of inflammation and nutritional state from an early stage of the disease would be important in the prognosis of MSA.
  • #23 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In laboratory biomarkers, there is growing evidence that biomarkers reflecting high systemic inflammation are associated with disease severity and progression of MSA. […] Furthermore, recent studies revealed that biomarkers associated with neuronal damage and malnutrition are associated with disease severity, low quality of life, and high mortality in MSA. […] The median survival duration from the onset was 8.0 years. […] The median survival of MSA-P patients and MSA-C patients were 9.0 and 8.0 years, respectively, with no statistical difference between the two subtypes. […] With a cut-off of mean value of laboratory markers within MSA patients, high WBC, ANC, RDW, CRP, and ESR values were significantly related to higher mortality. […] Low (mean) lymphocyte, Hb, total protein, albumin, creatinine, calcium, and GPT were related to higher mortality.
  • #24 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In orthostatic hypotension, sBP drop 30 significantly predicted higher mortality in MSA patients. […] The current study presents novel findings regarding high inflammation and poor nutritional status associated with higher mortality in MSA. […] In this study, we showed that low albumin, protein, and hemoglobin, which reflect poor nutritional status, were associated with higher mortality in MSA. […] These observations suggest that close observation of the nutritional state from an early stage of the disease would be important in the prognosis of MSA. […] In conclusion, a systemic alteration in inflammation and nutritional status was associated with a poor prognosis in MSA. Thus, close observation of inflammation and nutritional state from an early stage of the disease would be important in the prognosis of MSA.
  • #25 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In orthostatic hypotension, sBP drop 30 significantly predicted higher mortality in MSA patients. […] The current study presents novel findings regarding high inflammation and poor nutritional status associated with higher mortality in MSA. […] In this study, we showed that low albumin, protein, and hemoglobin, which reflect poor nutritional status, were associated with higher mortality in MSA. […] These observations suggest that close observation of the nutritional state from an early stage of the disease would be important in the prognosis of MSA. […] In conclusion, a systemic alteration in inflammation and nutritional status was associated with a poor prognosis in MSA. Thus, close observation of inflammation and nutritional state from an early stage of the disease would be important in the prognosis of MSA.
  • #26 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #27 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    More serious motor symptoms, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment were associated with poor survival in MSA via PCA and cluster analysis. […] Our study on a large Chinese MSA patient population confirmed the median survival time of Chinese MSA patients was 6.31 years. […] We identified several independent survival factors of MSA including more serious motor symptoms based on UMSARS scores, axial symptoms such as falls and dysphagia, orthostatic hypotension, and cognitive impairment via PCA and cluster analysis.
  • #28 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    An association between early development of severe urinary retention and shorter survival was observed in our patients. […] Early development of recurrent urinary infections with or without kidney damage due to vesicoureteral reflux could be one potential explanation for the increased mortality observed in our study. […] Early bladder dysfunction as well as early severity of autonomic failure may reflect a more severe loss of serotonergic neurons that participate in autonomic regulation and respiratory chemosensitivity.
  • #29 Multiple System Atrophy: Prognostic Indicators of Survival
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4139446/
    An association between early development of severe urinary retention and shorter survival was observed in our patients. […] Early development of recurrent urinary infections with or without kidney damage due to vesicoureteral reflux could be one potential explanation for the increased mortality observed in our study. […] Early bladder dysfunction as well as early severity of autonomic failure may reflect a more severe loss of serotonergic neurons that participate in autonomic regulation and respiratory chemosensitivity.
  • #30 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In laboratory biomarkers, there is growing evidence that biomarkers reflecting high systemic inflammation are associated with disease severity and progression of MSA. […] Furthermore, recent studies revealed that biomarkers associated with neuronal damage and malnutrition are associated with disease severity, low quality of life, and high mortality in MSA. […] The median survival duration from the onset was 8.0 years. […] The median survival of MSA-P patients and MSA-C patients were 9.0 and 8.0 years, respectively, with no statistical difference between the two subtypes. […] With a cut-off of mean value of laboratory markers within MSA patients, high WBC, ANC, RDW, CRP, and ESR values were significantly related to higher mortality. […] Low (mean) lymphocyte, Hb, total protein, albumin, creatinine, calcium, and GPT were related to higher mortality.
  • #31 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In orthostatic hypotension, sBP drop 30 significantly predicted higher mortality in MSA patients. […] The current study presents novel findings regarding high inflammation and poor nutritional status associated with higher mortality in MSA. […] In this study, we showed that low albumin, protein, and hemoglobin, which reflect poor nutritional status, were associated with higher mortality in MSA. […] These observations suggest that close observation of the nutritional state from an early stage of the disease would be important in the prognosis of MSA. […] In conclusion, a systemic alteration in inflammation and nutritional status was associated with a poor prognosis in MSA. Thus, close observation of inflammation and nutritional state from an early stage of the disease would be important in the prognosis of MSA.
  • #32 Differentiation of multiple system atrophy from Parkinson’s disease by structural connectivity derived from probabilistic tractography | Scientific Reports
    https://www.nature.com/articles/s41598-019-52829-8
    For this reason, improving our ability to diagnose and to predict MSA progression after diagnosis is a major objective in clinical practice. […] Given that MSA patients can be incorrectly diagnosed as having PD, especially in early stages of the disease, the development of neuroimaging biomarkers that can distinguish between these two illnesses with a high accuracy at the individual patient level would be of great clinical interest. […] We found that the streamline count in the affected connections detected using edge-wise connectivity analysis was informative enough to distinguish MSA patients from PD patients with satisfactory accuracy, concretely of 0.78 and 0.84, when using the whole dataset and a paired subsample, respectively. […] Taken together, our results demonstrate the usefulness of NOS for correctly distinguishing MSA from PD and HC, which outperformed the classification obtained with diffusion tensor-derived metrics.
  • #33 Differentiation of multiple system atrophy from Parkinson’s disease by structural connectivity derived from probabilistic tractography | Scientific Reports
    https://www.nature.com/articles/s41598-019-52829-8
    Recent studies combining diffusion tensor-derived metrics and machine learning have shown promising results in the discrimination of multiple system atrophy (MSA) and Parkinsons disease (PD) patients. […] The aim of this work is assessing whether the strength of structural connectivity between subcortical structures, measured as the number of streamlines (NOS) derived from tractography, can be used to classify MSA and PD patients at the single-patient level. […] Our findings suggest that structural connectivity derived from tractography has the potential to correctly distinguish between MSA and PD patients. Furthermore, NOS measures obtained from tractography might be more useful than diffusion tensor-derived metrics for the detection of MSA. […] Although MSA may resemble Parkinsons disease (PD) in its early stages, brain damage is more aggressive, with usually no response to dopaminergic medication, and leading to a rapidly progressive disease course with a fatal prognosis.
  • #34 Differentiation of multiple system atrophy from Parkinson’s disease by structural connectivity derived from probabilistic tractography | Scientific Reports
    https://www.nature.com/articles/s41598-019-52829-8
    For this reason, improving our ability to diagnose and to predict MSA progression after diagnosis is a major objective in clinical practice. […] Given that MSA patients can be incorrectly diagnosed as having PD, especially in early stages of the disease, the development of neuroimaging biomarkers that can distinguish between these two illnesses with a high accuracy at the individual patient level would be of great clinical interest. […] We found that the streamline count in the affected connections detected using edge-wise connectivity analysis was informative enough to distinguish MSA patients from PD patients with satisfactory accuracy, concretely of 0.78 and 0.84, when using the whole dataset and a paired subsample, respectively. […] Taken together, our results demonstrate the usefulness of NOS for correctly distinguishing MSA from PD and HC, which outperformed the classification obtained with diffusion tensor-derived metrics.
  • #35 Differentiation of multiple system atrophy from Parkinson’s disease by structural connectivity derived from probabilistic tractography | Scientific Reports
    https://www.nature.com/articles/s41598-019-52829-8
    Recent studies combining diffusion tensor-derived metrics and machine learning have shown promising results in the discrimination of multiple system atrophy (MSA) and Parkinsons disease (PD) patients. […] The aim of this work is assessing whether the strength of structural connectivity between subcortical structures, measured as the number of streamlines (NOS) derived from tractography, can be used to classify MSA and PD patients at the single-patient level. […] Our findings suggest that structural connectivity derived from tractography has the potential to correctly distinguish between MSA and PD patients. Furthermore, NOS measures obtained from tractography might be more useful than diffusion tensor-derived metrics for the detection of MSA. […] Although MSA may resemble Parkinsons disease (PD) in its early stages, brain damage is more aggressive, with usually no response to dopaminergic medication, and leading to a rapidly progressive disease course with a fatal prognosis.
  • #36 Multiple System Atrophy (MSA): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17250-multiple-system-atrophy
    Multiple system atrophy is a degenerative brain disease, meaning it causes parts of your brain to deteriorate. This disease is usually fatal within 10 years, but may have a shorter or longer life expectancy depending on severity. […] MSA is a rare neurological disease that causes certain brain areas to deteriorate. This disease is ultimately fatal. […] People who have MSA will usually develop movement-related symptoms first. This condition gets progressively worse over time. About half of people with this condition need help walking within this time frame. […] The average survival time for this condition is six to 10 years. In less severe cases, people can survive up to 15 years. However, in very severe cases, survival time may be much lower. […] The outlook for MSA is poor. The symptoms of this condition get progressively worse and always disrupt body function, leading to deadly complications.
  • #37 Multiple System Atrophy (MSA): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17250-multiple-system-atrophy
    Multiple system atrophy is a degenerative brain disease, meaning it causes parts of your brain to deteriorate. This disease is usually fatal within 10 years, but may have a shorter or longer life expectancy depending on severity. […] MSA is a rare neurological disease that causes certain brain areas to deteriorate. This disease is ultimately fatal. […] People who have MSA will usually develop movement-related symptoms first. This condition gets progressively worse over time. About half of people with this condition need help walking within this time frame. […] The average survival time for this condition is six to 10 years. In less severe cases, people can survive up to 15 years. However, in very severe cases, survival time may be much lower. […] The outlook for MSA is poor. The symptoms of this condition get progressively worse and always disrupt body function, leading to deadly complications.
  • #38 Differentiation of multiple system atrophy from Parkinson’s disease by structural connectivity derived from probabilistic tractography | Scientific Reports
    https://www.nature.com/articles/s41598-019-52829-8
    Recent studies combining diffusion tensor-derived metrics and machine learning have shown promising results in the discrimination of multiple system atrophy (MSA) and Parkinsons disease (PD) patients. […] The aim of this work is assessing whether the strength of structural connectivity between subcortical structures, measured as the number of streamlines (NOS) derived from tractography, can be used to classify MSA and PD patients at the single-patient level. […] Our findings suggest that structural connectivity derived from tractography has the potential to correctly distinguish between MSA and PD patients. Furthermore, NOS measures obtained from tractography might be more useful than diffusion tensor-derived metrics for the detection of MSA. […] Although MSA may resemble Parkinsons disease (PD) in its early stages, brain damage is more aggressive, with usually no response to dopaminergic medication, and leading to a rapidly progressive disease course with a fatal prognosis.
  • #39 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #40 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    To elucidate the biomarkers related to survival in multiple system atrophy(MSA), we analyzed the predictability of retrospectively collected blood markers for survival in 650 probable MSA. High absolute neutrophil count, red-cell distribution width, C-reactive protein, erythrocyte sedimentation rate, and low hemoglobin, protein, albumin, and creatinine were correlated with higher mortality in MSA. Systemic alteration in inflammation and nutritional status in the early stage are associated with higher mortality in MSA. […] Thus, it is important to establish a biomarker that predicts the prognosis in the early phase of the disease to optimize disease monitoring and to develop novel neuroprotective strategies. […] Among clinical biomarkers, early autonomic failure, older age of onset, and absence of response to levodopa have been associated with shorter survival.
  • #41 Predicting the Prognosis of Multiple System Atrophy Using Cluster and Principal Component Analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC10578219/
    Multiple system atrophy (MSA) is an intractable neurodegenerative disorder with poorly understanding of prognostic factors. […] The prognosis for MSA varies widely, with an average survival time of 610 years. […] A better understanding of the prognostic factors of MSA can guide the design of therapeutic interventions to extend survival and improve the quality of life of MSA patients. […] The median survival time from symptom onset to death (estimated using data from all patients by Kaplan-Meier analysis) was 6.3 (95% CI=6.16.7) years. […] The survival model showed that a shorter survival time was associated with motor principal component (PC)1 (HR=1.71, 95% CI: 1.262.30, p0.001) and nonmotor PC3 (HR=1.68, 95% CI: 1.312.10, p0.001) through PCA. […] Multivariate Cox regression indicated that Cluster 3 (HR=4.15, 95% CI: 1.739.90, p=0.001) and Cluster 4 (HR=4.18, 95% CI: 1.7310.1, p=0.002) were independently associated with shorter survival time.
  • #42 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    To elucidate the biomarkers related to survival in multiple system atrophy(MSA), we analyzed the predictability of retrospectively collected blood markers for survival in 650 probable MSA. High absolute neutrophil count, red-cell distribution width, C-reactive protein, erythrocyte sedimentation rate, and low hemoglobin, protein, albumin, and creatinine were correlated with higher mortality in MSA. Systemic alteration in inflammation and nutritional status in the early stage are associated with higher mortality in MSA. […] Thus, it is important to establish a biomarker that predicts the prognosis in the early phase of the disease to optimize disease monitoring and to develop novel neuroprotective strategies. […] Among clinical biomarkers, early autonomic failure, older age of onset, and absence of response to levodopa have been associated with shorter survival.
  • #43 Multiple System Atrophy (MSA): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17250-multiple-system-atrophy
    Multiple system atrophy is a degenerative brain disease, meaning it causes parts of your brain to deteriorate. This disease is usually fatal within 10 years, but may have a shorter or longer life expectancy depending on severity. […] MSA is a rare neurological disease that causes certain brain areas to deteriorate. This disease is ultimately fatal. […] People who have MSA will usually develop movement-related symptoms first. This condition gets progressively worse over time. About half of people with this condition need help walking within this time frame. […] The average survival time for this condition is six to 10 years. In less severe cases, people can survive up to 15 years. However, in very severe cases, survival time may be much lower. […] The outlook for MSA is poor. The symptoms of this condition get progressively worse and always disrupt body function, leading to deadly complications.
  • #44 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Disease
    https://www.nature.com/articles/s41531-022-00413-9
    In orthostatic hypotension, sBP drop 30 significantly predicted higher mortality in MSA patients. […] The current study presents novel findings regarding high inflammation and poor nutritional status associated with higher mortality in MSA. […] In this study, we showed that low albumin, protein, and hemoglobin, which reflect poor nutritional status, were associated with higher mortality in MSA. […] These observations suggest that close observation of the nutritional state from an early stage of the disease would be important in the prognosis of MSA. […] In conclusion, a systemic alteration in inflammation and nutritional status was associated with a poor prognosis in MSA. Thus, close observation of inflammation and nutritional state from an early stage of the disease would be important in the prognosis of MSA.