Atrofia wieloukładowa
Objawy
Atrofia wieloukładowa (MSA) to rzadkie, postępujące schorzenie neurodegeneracyjne, które obejmuje dysfunkcję zarówno ośrodkowego, jak i autonomicznego układu nerwowego. Choroba manifestuje się w dwóch głównych podtypach: MSA-P z dominującymi objawami parkinsonizmu (sztywność mięśni, bradykinezja, drżenie, dyzartria, zaburzenia postawy) oraz MSA-C z przewagą ataksji móżdżkowej (zaburzenia koordynacji, dyzartria, dysfagia, zaburzenia widzenia). W obu typach wczesne objawy autonomiczne, takie jak hipotensja ortostatyczna, zaburzenia funkcji pęcherza moczowego (nietrzymanie, zatrzymanie moczu), dysfunkcje seksualne (zwłaszcza zaburzenia erekcji u mężczyzn) oraz zaburzenia snu (REM sleep behavior disorder, bezdech senny, stridor), często poprzedzają objawy ruchowe. Średni czas przeżycia od wystąpienia symptomów wynosi około 7-10 lat, a progresja choroby jest szybka, prowadząc do znacznej niepełnosprawności i powikłań takich jak infekcje dróg moczowych, zachłystowe zapalenie płuc czy zatorowość płucna.
- Wprowadzenie do atrofii wieloukładowej
- Typy atrofii wieloukładowej
- Objawy autonomiczne w MSA
- Zaburzenia ciśnienia krwi
- Zaburzenia układu moczowego i pokarmowego
- Zaburzenia termoregulacji
- Zaburzenia funkcji seksualnych
- Zaburzenia snu i oddychania
- Zaburzenia emocjonalne i poznawcze
- Progresja choroby
- Diagnostyka MSA
- Leczenie i postępowanie
- Stadia progresji MSA
- Stadium 1: Wczesne objawy
- Stadium 2: Progresja objawów
- Stadium 3: Znacząca niepełnosprawność
- Stadium 4: Choroba końcowego stadium
- Różnice między MSA a chorobą Parkinsona
- Podsumowanie
Wprowadzenie do atrofii wieloukładowej
Atrofia wieloukładowa (Multiple System Atrophy, MSA) jest rzadkim, postępującym schorzeniem neurodegeneracyjnym, które charakteryzuje się połączeniem objawów wpływających zarówno na ośrodkowy układ nerwowy (kontrolujący sposób poruszania się), jak i na autonomiczny układ nerwowy, który kontroluje funkcje mimowolne, takie jak ciśnienie krwi, trawienie i inne podstawowe funkcje organizmu1. Choroba zazwyczaj rozpoczyna się u osób dorosłych, najczęściej w wieku 50-60 lat, choć może wystąpić kiedykolwiek po 30. roku życia23. MSA dotyka w równym stopniu mężczyzn i kobiety4.
Objawy atrofii wieloukładowej odzwierciedlają postępującą utratę funkcji i obumieranie różnych typów komórek nerwowych w mózgu i rdzeniu kręgowym1. Choroba ma charakter postępujący – objawy nasilają się z czasem, a średni czas przeżycia od momentu ich wystąpienia wynosi około 7-10 lat, chociaż przeżywalność może się znacznie różnić56. Śmierć najczęściej następuje w wyniku problemów z oddychaniem, infekcji lub zakrzepów krwi w płucach5.
Obecnie nie istnieje lekarstwo na MSA ani leczenie, które mogłoby zatrzymać jej postęp. Terapia koncentruje się na łagodzeniu objawów i poprawie jakości życia pacjentów2.
Typy atrofii wieloukładowej
Atrofia wieloukładowa występuje w dwóch głównych podtypach, które różnią się dominującymi objawami w momencie rozpoznania7:
MSA-P (typ parkinsonowski)
Jest to najczęstszy typ MSA. Objawami przypomina chorobę Parkinsona i charakteryzuje się:78
- Sztywnością mięśni
- Trudnościami w zginaniu rąk i nóg
- Spowolnieniem ruchów (bradykinezą)
- Drżeniem spoczynkowym lub podczas ruchu kończyn
- Zaburzeniami mowy – mowa jest spowolniona, cicha lub niewyraźna (dyzartria)
- Problemami z postawą ciała i równowagą
MSA-C (typ móżdżkowy)
W tym typie dominują objawy związane z zaburzeniami koordynacji mięśniowej (ataksja):98
- Problemy z ruchem i koordynacją, w tym utrata równowagi i niestabilny chód
- Niewyraźna, spowolniona lub cicha mowa (dyzartria)
- Zaburzenia widzenia, w tym nieostre lub podwójne widzenie i trudności z koncentracją wzroku
- Problemy z żuciem lub połykaniem (dysfagia)
Mimo że MSA początkowo może rozwijać się jako jeden z tych typów, z czasem pojawiają się objawy charakterystyczne dla drugiego typu. Po około 5 latach objawy zazwyczaj są podobne, niezależnie od początkowego typu choroby4. Ważne jest, aby pamiętać, że u każdego pacjenta choroba może przebiegać inaczej, a szybkość postępu i nasilenie objawów mogą się znacznie różnić10.
Objawy autonomiczne w MSA
W obu typach MSA występują zaburzenia funkcji autonomicznego układu nerwowego, który kontroluje mimowolne funkcje organizmu9. Te zaburzenia często pojawiają się jako jedne z pierwszych objawów, a nawet mogą wyprzedzać pojawienie się objawów ruchowych o miesiące lub lata1112.
Zaburzenia ciśnienia krwi
Najbardziej charakterystycznym objawem autonomicznym w MSA jest hipotensja ortostatyczna, czyli znaczny spadek ciśnienia krwi podczas wstawania9. Osoby z tym typem niedociśnienia odczuwają zawroty głowy lub uczucie pustki w głowie podczas wstawania po siedzeniu lub leżeniu. Mogą nawet tracić przytomność13. Warto zaznaczyć, że nie wszyscy pacjenci z MSA doświadczają hipotensji ortostatycznej13.
Pacjenci z MSA mogą również rozwinąć niebezpiecznie wysokie ciśnienie krwi podczas leżenia, co określa się jako nadciśnienie w pozycji leżącej (supine hypertension)14.
Zaburzenia układu moczowego i pokarmowego
Częste problemy autonomiczne w MSA obejmują:14
- Zaparcia
- Utratę kontroli nad pęcherzem moczowym lub jelitami (nietrzymanie)
- Problemy z oddawaniem moczu, takie jak nagłe parcie na mocz, trudności z opróżnianiem pęcherza lub zatrzymanie moczu11
Dysfunkcja układu moczowego często prowadzi do zakażeń dróg moczowych, a ponad 50% pacjentów z MSA cierpi na nawracające infekcje dolnych dróg moczowych, co może prowadzić do poważnych powikłań15.
Zaburzenia termoregulacji
Osoby z atrofią wieloukładową mogą doświadczać:14
- Zmniejszonej produkcji potu
- Nietolerancji ciepła z powodu zmniejszonego pocenia się
- Słabej kontroli temperatury ciała, co często powoduje zimne dłonie lub stopy
Zaburzenia funkcji seksualnych
Dysfunkcje seksualne są często jednymi z pierwszych objawów MSA:1611
- Problemy z osiągnięciem lub utrzymaniem erekcji (zaburzenia erekcji)
- Trudności z lubrykacją podczas stosunku i osiąganiem orgazmu
- Utrata zainteresowania seksem
Zaburzenia erekcji to jeden z najczęstszych pierwszych objawów MSA u mężczyzn i może wyprzedzać diagnozę nawet o 4 lata lub więcej1117.
Zaburzenia snu i oddychania
Zaburzenia snu są powszechne u pacjentów z MSA i mogą być jednymi z pierwszych objawów choroby18. Obejmują one:1614
- Zaburzenia zachowania w fazie REM (REM sleep behavior disorder, RBD) – pacjenci „odgrywają” swoje sny podczas snu, co przejawia się chaotycznymi ruchami, mówieniem lub krzyczeniem. Ten objaw występuje u większości pacjentów z MSA i często pojawia się przed wystąpieniem innych objawów ruchowych1819.
- Bezdech senny (sleep apnea) – okresowe zatrzymanie oddychania podczas snu
- Stridor – wysoki, świszczący dźwięk podczas oddychania, szczególnie w nocy
Problemy z oddychaniem mogą rozwinąć się we wczesnych stadiach MSA i mogą prowadzić do poważnych komplikacji18. Są to między innymi: bezdech senny, duszność i inspiracyjny stridor18. Zaburzenia oddychania są jedną z głównych przyczyn śmierci u pacjentów z MSA20.
Zaburzenia emocjonalne i poznawcze
U pacjentów z MSA mogą wystąpić różne zaburzenia emocjonalne i poznawcze:52114
- Trudności z kontrolowaniem emocji, takie jak nieoczekiwany śmiech lub płacz (labilność emocjonalna)
- Zmiany nastroju, w tym depresja i lęk
- Zaburzenia funkcji poznawczych, w tym dysfunkcje wykonawcze i upośledzenie pamięci werbalnej
Badania wykazały, że do 49% pacjentów z MSA ma zaburzenia funkcji wykonawczych22, a około 40% pacjentów doświadcza łagodnych do umiarkowanych zaburzeń poznawczych, którym może towarzyszyć depresja (28% pacjentów) i zaburzenia lękowe (22% pacjentów)23.
Progresja choroby
Atrofia wieloukładowa jest chorobą postępującą, której przebieg różni się u poszczególnych pacjentów, ale generalnie charakteryzuje się szybką progresją w porównaniu z chorobą Parkinsona24.
Wczesne objawy
Pierwsze objawy MSA są często trudne do odróżnienia od objawów choroby Parkinsona1. Mogą obejmować:
- Spowolnienie ruchów, drżenie lub sztywność
- Niezgrabność lub brak koordynacji
- Ochrypły, drżący głos
- Omdlenia lub zawroty głowy
- Problemy z kontrolą pęcherza moczowego
Warto zauważyć, że wczesne objawy MSA są często autonomiczne i mogą wyprzedzać rozpoznanie objawów ruchowych22. Szczególnie u mężczyzn zaburzenia erekcji są często pierwszym objawem choroby, występującym średnio 4,2 ± 2,6 lat przed diagnozą11.
Postęp choroby
MSA charakteryzuje się szybkim postępem objawów24:
- Większość pacjentów z MSA będzie potrzebowała pomocy przy chodzeniu, takiej jak laska lub chodzik, w ciągu kilku lat od wystąpienia objawów24
- Około 50% pacjentów wymaga pomocy przy chodzeniu w ciągu 3 lat od wystąpienia objawów ruchowych25
- Około 60% pacjentów wymaga wózka inwalidzkiego po 5 latach od wystąpienia objawów ruchowych26
- Prawie 80% pacjentów jest niepełnosprawnych w ciągu 5 lat od wystąpienia objawów ruchowych27
W miarę postępu choroby objawy się nasilają, a leczenie staje się mniej skuteczne28. Pacjenci mogą doświadczać trudności z używaniem sztućców, połykaniem pokarmów stałych lub płynów, chodzeniem i kontrolowaniem pęcherza moczowego lub jelit28.
Zaawansowane stadium choroby
W zaawansowanym stadium MSA pacjenci mają zwiększone ryzyko infekcji28:
- Objawy ze strony pęcherza moczowego mogą prowadzić do zakażeń dróg moczowych28
- Problemy z połykaniem zwiększają ryzyko zachłystowego zapalenia płuc28
- Wielu pacjentów rozwija stridor krtaniowy i trudności w połykaniu, co może prowadzić do zapalenia płuc21
Najczęstszymi przyczynami śmierci u pacjentów z MSA są zator płucny, bezdech i infekcje współistniejące20.
Rokowanie
Rokowanie w MSA jest niepomyślne. Średni czas przeżycia od wystąpienia pierwszych objawów wynosi około 6-10 lat, choć przeżywalność może się znacznie różnić56. Tylko około 20% pacjentów z MSA przeżywa ponad 12 lat29.
Czynniki związane z krótszym czasem przeżycia obejmują1526:
- Starszy wiek w momencie wystąpienia objawów
- Wczesna cewnikowanie pęcherza moczowego
- Wczesna uogólniona niewydolność autonomiczna
- Niski poziom witaminy B1230
- Markery odzwierciedlające wysoki stan zapalny31
Diagnostyka MSA
Diagnoza atrofii wieloukładowej może być trudna, ponieważ wiele jej objawów, takich jak sztywność i problemy z chodzeniem, występuje również w innych chorobach, w tym w chorobie Parkinsona32.
Neurologowie mogą zdiagnozować MSA na podstawie objawów, czasu ich trwania i szybkości ich nasilania się2. Diagnoza kliniczna MSA wymaga obecności różnych kombinacji niewydolności autonomicznej i/lub parkinsonizmu lub ataksji móżdżkowej23.
Według nowych kryteriów diagnostycznych, mediana czasu od początku choroby do spełnienia kryteriów diagnozy „prawdopodobnej MSA” wynosi około dwóch lat23. Pacjenci często mają tylko niewydolność autonomiczną, parkinsonizm lub ataksję móżdżkową we wczesnym stadium choroby23.
MSA można podejrzewać, jeśli33:
- Objawy szybko postępują
- Pacjent doświadczył upadków we wczesnych stadiach choroby
- Pacjent nie reaguje dobrze na leczenie lewodopą
- Mowa pacjenta została poważnie dotknięta chorobą
- Pacjent dyszący i oddycha hałaśliwie
Leczenie i postępowanie
Obecnie nie ma lekarstwa na MSA ani metod leczenia, które mogłyby zatrzymać jej postęp33. Leczenie koncentruje się na łagodzeniu objawów i poprawie jakości życia32.
Leczenie farmakologiczne
Leki stosowane w leczeniu objawów MSA obejmują:
- Leki przeciwparkinsonowskie: Wielu pacjentów z MSA nie reaguje na leki stosowane w chorobie Parkinsona. Leki te mogą również stać się mniej skuteczne po kilku latach32. Tylko około 30-60% pacjentów reaguje na typowe leki na Parkinsona, takie jak karbidopa/lewodopa (Sinemet)12.
- Leki na niedociśnienie ortostatyczne: Mogą być stosowane leki zwiększające objętość krwi (fludrokortyzon) lub leki presyjne (np. midodryna), aby zapobiec spadkom ciśnienia krwi34.
- Leki na problemy z pęcherzem moczowym: Leki mogą pomóc w kontrolowaniu pęcherza moczowego we wczesnych stadiach35.
Terapie niefarmakologiczne
Inne metody leczenia obejmują:
- Fizjoterapia: Może pomóc w utrzymaniu jak największej możliwej zdolności poruszania się i siły w miarę postępu choroby35.
- Terapia zajęciowa: Może doradzić w kwestii sprzętu, który pomoże żyć bezpiecznie33.
- Terapia mowy i języka: Może pomóc w problemach z mową i połykaniem (dysfagia)36.
- Dietetyka: Dietetyk pomoże poradzić, jak jeść zdrową, zbilansowaną dietę, w tym więcej pokarmów i płynów, które są łatwe do połknięcia36.
Postępowanie w zaawansowanej chorobie
W zaawansowanych stadiach MSA mogą być konieczne bardziej inwazyjne interwencje:
- Jeśli objawy połykania lub oddychania się pogorszą, może być konieczna operacja w celu wszczepienia rurki do karmienia lub oddychania32. Rurka gastrostomijna dostarcza pokarm bezpośrednio do żołądka32.
- W miarę postępu MSA może być konieczne wprowadzenie miękkiej rurki w celu opróżnienia pęcherza. Miękka rurka jest znana jako cewnik35.
Postępowanie w MSA wymaga wielodyscyplinarnego podejścia, a pacjenci powinni być ściśle monitorowani pod kątem poważnych problemów, takich jak zaburzenia oddychania podczas snu, nadciśnienie w pozycji leżącej i hipotensja ortostatyczna37.
Stadia progresji MSA
W przeciwieństwie do niektórych innych chorób, atrofia wieloukładowa nie ma wyraźnie zdefiniowanych stadiów38. Jednak choroba ma tendencję do stałego postępu po diagnozie, a jej przebieg można podzielić na kilka ogólnych etapów39.
Stadium 1: Wczesne objawy
W początkowym stadium MSA objawy mogą być łagodne40:
- Objawy ruchowe: Łagodna ataksja (niezgrabność lub niestabilność), łagodny parkinsonizm (subtelna sztywność lub spowolnienie ruchów)
- Objawy autonomiczne: Hipotensja ortostatyczna, objawy ze strony układu moczowego (zwiększona częstotliwość, pilność lub trudności w oddawaniu moczu)
- Inne objawy: Zaburzenia snu (RBD), łagodne zmiany poznawcze (trudności z koncentracją lub funkcjami wykonawczymi)
Stadium 2: Progresja objawów
W tym stadium objawy stają się bardziej widoczne41:
- Objawy ruchowe: Zwiększona sztywność mięśni, drżenie (może się rozwinąć lub stać się bardziej zauważalne), problemy z równowagą prowadzące do zwiększonego ryzyka upadków
- Objawy autonomiczne: Pogorszenie hipotensji ortostatycznej, dysfunkcja pęcherza moczowego (nietrzymanie moczu lub zatrzymanie staje się bardziej problematyczne), dysfunkcja seksualna
- Inne objawy: Nasilenie zaburzeń snu, nieprawidłowości w poceniu się
Stadium 3: Znacząca niepełnosprawność
W tym stadium choroba powoduje poważne ograniczenia4142:
- Objawy ruchowe: Ciężka ataksja i parkinsonizm (chodzenie staje się bardzo trudne, wielu pacjentów wymaga pomocy lub urządzeń wspomagających poruszanie się), dyzartria (mowa staje się niewyraźna, utrudniająca komunikację), dysfagia (trudności w połykaniu zwiększające ryzyko zadławienia lub aspiracji)
- Objawy autonomiczne: Ekstremalne wahania ciśnienia krwi, problemy żołądkowo-jelitowe (zaparcia), problemy z regulacją temperatury
- Inne objawy: Pogorszenie funkcji poznawczych, problemy ze wzrokiem
Stadium 4: Choroba końcowego stadium
W końcowym stadium MSA pacjenci doświadczają skrajnej niepełnosprawności42:
- Objawy ruchowe: Utrata większości lub całej mobilności (często pacjenci są przykuci do łóżka), ciężka dyzartria i dysfagia (komunikacja jest poważnie utrudniona, a karmienie może wymagać stosowania sondy z powodu ryzyka związanego z połykaniem)
- Objawy autonomiczne: Krytyczna niewydolność autonomiczna (zagrażające życiu spadki ciśnienia krwi, poważnie upośledzona regulacja temperatury ciała), powikłania oddechowe (zwiększone ryzyko zapalenia płuc z powodu aspiracji lub osłabionych mięśni oddechowych)
- Inne objawy: Znaczne upośledzenie poznawcze, ogólne pogorszenie stanu zdrowia (zwiększona podatność na infekcje, odleżyny z powodu unieruchomienia i inne powikłania)
Warto podkreślić, że progresja MSA jest indywidualna, a objawy mogą różnić się intensywnością i czasem wystąpienia. Wczesne rozpoznanie tych stadiów może kierować postępowaniem medycznym, koncentrując się na kontroli objawów, bezpieczeństwie i jakości życia43.
Różnice między MSA a chorobą Parkinsona
Atrofia wieloukładowa i choroba Parkinsona są postępującymi zaburzeniami układu nerwowego, które wpływają na codzienne ruchy. MSA jest rzadsza i zazwyczaj postępuje szybciej44.
Kluczowe różnice między MSA a chorobą Parkinsona obejmują:
- Progresja: MSA postępuje szybciej niż choroba Parkinsona24. Większość pacjentów z MSA będzie potrzebowała pomocy przy chodzeniu w ciągu kilku lat od wystąpienia objawów24.
- Odpowiedź na leczenie: Pacjenci z MSA generalnie słabo reagują na leki stosowane w chorobie Parkinsona45. Korzyści obserwowane we wczesnej fazie choroby często zanikają z czasem12.
- Drżenie: W przeciwieństwie do choroby Parkinsona, MSA zwykle nie powoduje drżenia spoczynkowego i dyskinezy45. Tylko około 9% pacjentów z MSA z drżeniem wykazuje typowe dla choroby Parkinsona drżenie typu „kręcenia pigułki”46.
- Dysfunkcja autonomiczna: Wczesna i ciężka dysfunkcja autonomiczna jest bardziej charakterystyczna dla MSA niż dla choroby Parkinsona47.
- Zaburzenia pęcherza: Pomiaru objętości zalegającego po mikcji (PVR) może również pomóc odróżnić chorobę Parkinsona od MSA. PVR jest podwyższony u pacjentów z MSA z powodu większej retencji moczu w porównaniu do pacjentów z chorobą Parkinsona48.
Podsumowanie
Atrofia wieloukładowa (MSA) jest rzadkim, postępującym schorzeniem neurodegeneracyjnym, które wpływa na funkcje autonomiczne, ruchowe i równowagę. Jej objawy mogą przypominać chorobę Parkinsona, ale MSA postępuje szybciej i ma gorsze rokowanie.
Początkowe objawy są często autonomiczne i mogą wyprzedzać rozpoznanie objawów ruchowych. U mężczyzn zaburzenia erekcji są często pierwszym objawem. Inne wczesne objawy obejmują hipotensję ortostatyczną, problemy z pęcherzem moczowym i zaburzenia zachowania w fazie REM snu.
MSA ma dwa główne podtypy: MSA-P z dominującymi objawami parkinsonizmu i MSA-C z dominującymi objawami móżdżkowymi. Z czasem objawy obu typów zazwyczaj się nakładają.
Nie istnieje obecnie lekarstwo na MSA ani leczenie, które mogłoby zatrzymać jej postęp. Terapia koncentruje się na łagodzeniu objawów i poprawie jakości życia pacjentów. Wielodyscyplinarne podejście obejmujące neurologów, fizjoterapeutów, terapeutów zajęciowych i innych specjalistów jest kluczowe dla skutecznego zarządzania tą złożoną chorobą.
Średni czas przeżycia od wystąpienia objawów wynosi około 7-10 lat, choć przeżywalność może się znacznie różnić. Choroba prowadzi do postępującej niepełnosprawności, a ostatecznie do śmierci, najczęściej z powodu problemów z oddychaniem, infekcji lub zakrzepów krwi w płucach.
Badania nad MSA trwają, a naukowcy poszukują nowych metod wczesnej diagnozy, skuteczniejszych terapii i potencjalnych strategii modyfikujących przebieg choroby. Mimo że MSA pozostaje nieuleczalna, odpowiednia opieka może znacząco poprawić jakość życia pacjentów i ich rodzin.
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Materiały źródłowe
- #1 Multiple System Atrophy | National Institute of Neurological Disorders and Strokehttps://www.ninds.nih.gov/health-information/disorders/multiple-system-atrophy
Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the central nervous system (which controls how a person moves), and the autonomic nervous system, which controls involuntary functions such as blood pressure or digestion. […] The symptoms of MSA reflect the progressive loss of function and death of different types of nerve cells in the brain and spinal cord. […] The initial symptoms can be difficult to distinguish from those of Parkinson’s disease, and can include: Slowness of movement, tremor, or stiffness, Clumsiness or lack of coordination, Croaky, quivering voice, Fainting or lightheadedness, Bladder control problems. […] Symptoms tend to appear in a person’s 50s and advance rapidly over the course of five to 10 years. A person with MSA will have increased difficulty with movement and eventually become bedridden. People with MSA often develop swallowing problems that can lead to pneumonia in the later stages of the disease.
- #2 Multiple system atrophy (MSA)https://www.nhs.uk/conditions/multiple-system-atrophy/
The main symptom of multiple system atrophy (MSA) is losing control of muscles in the body. This happens gradually in some people and quickly in others. […] MSA affects people in different ways, depending on which muscles are affected. Some of the most common symptoms include: bladder problems, such as needing to pee more often or being unable to pee; feeling dizzy and faint when standing up; erectile dysfunction (impotence); slow, clumsy or unsteady movement; slurred speech; stiff, tense muscles that may be painful. […] The symptoms of MSA most often begin in people over 50 years old. It does not usually affect people under 30 years old. […] A neurologist may diagnose MSA based on your symptoms, how long you’ve had them and how quickly they’re getting worse. […] There’s currently no cure for multiple system atrophy (MSA) and no treatments that can stop it getting worse. […] Multiple system atrophy (MSA) affects how long someone lives and can sometimes get worse quickly. Most people eventually need some help and support with their daily living. […] The type of support and how quickly it’s needed can vary, as MSA affects everyone differently.
- #3 Multiple system atrophy (MSA) | NHS informhttps://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/multiple-system-atrophy-msa/
Everyone with MSA will experience different symptoms. Symptoms often start between the ages of 50 and 60, but they can begin anytime from the age of 30. […] You’re likely to experience muscle control or balance problems, like those of Parkinson’s disease or Ataxia. It’s a progressive disease which means symptoms will worsen over a period of time. […] MSA can cause symptoms including: bladder problems like a constant need to pee, loss of bladder control (urinary incontinence) or being unable to pee; low blood pressure when standing up which might cause dizziness or fainting (postural hypotension); erection problems like erectile dysfunction; problems with co-ordination, balance and speech (cerebellar ataxia); tremor, slowness and muscle stiffness; constipation. […] Someone with MSA might also have: uncontrollable laughing or crying; problems with sleep like insomnia, restless legs or nightmares; noisy breathing; unintentional sighing; weak, quiet voice; shoulder and neck pain; problems controlling sweating; swallowing problems including difficult chewing and choking episodes; blurred vision; cold hands and feet.
- #4 Multiple System Atrophy (MSA) – Brain, Spinal Cord, and Nerve Disorders – Merck Manual Consumer Versionhttps://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/autonomic-nervous-system-disorders/multiple-system-atrophy-msa
Multiple system atrophy is a progressive, fatal disorder that causes symptoms resembling those of Parkinson disease (parkinsonism), loss of coordination, and malfunction of internal body processes (such as blood pressure and bladder control). […] Symptoms include symptoms that resemble those of Parkinson disease, loss of coordination, low blood pressure when a person stands (orthostatic hypotension), problems with urination, and constipation. […] Multiple system atrophy usually begins when people are in their 50s. It affects men and women equally. […] Both types involve autonomic nervous system dysfunction. Although multiple system atrophy begins as one of these types, symptoms of the other type eventually develop. After about 5 years, symptoms tend to be similar regardless of which disorder developed first.
- #5 Multiple system atrophy – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/symptoms-causes/syc-20356153
MSA may cause: Color changes in the hands and feet. […] People with multiple system atrophy also may experience: Trouble controlling emotions, such as laughing or crying when not expected. […] Complications of multiple system atrophy (MSA) vary from person to person. But for everyone with the disease, MSA symptoms get worse over time. The symptoms can make daily activities harder as time goes on. […] People typically live about 7 to 10 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Death is often due to trouble breathing, infections or blood clots in the lungs.
- #6 Multiple System Atrophy (MSA): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/17250-multiple-system-atrophy
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.
- #7 Multiple system atrophy – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/symptoms-causes/syc-20356153
Multiple system atrophy, also called MSA, causes people to lose coordination and balance or become slow and stiff. It also causes changes in speech and loss of control of other bodily functions. […] MSA is a rare condition. It sometimes shares symptoms with Parkinson’s disease, including slow movement, rigid muscles and poor balance. […] Treatment includes medicines and lifestyle changes to help manage symptoms, but there is no cure. The condition gets worse over time and eventually leads to death. […] Multiple system atrophy (MSA) symptoms affect many parts of the body. Symptoms start in adulthood, usually in the 50s or 60s. […] There are two types of MSA: parkinsonian and cerebellar. The type depends on the symptoms a person has when diagnosed. […] This is the most common type of MSA. Symptoms are similar to those of Parkinson’s disease, such as: Stiff muscles. Trouble bending the arms and legs. Slow movement, known as bradykinesia. Tremors at rest or when moving the arms or legs. Slurred, slow or soft speech, known as dysarthria. Trouble with posture and balance.
- #8 Multiple system atrophy (MSA) // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/multiple-system-atrophy-msa
Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body’s involuntary (autonomic) functions, including blood pressure, and motor control. […] MSA shares many symptoms with Parkinson’s disease, such as slow movement, rigid muscles and poor balance. […] The condition progresses gradually and eventually leads to death. […] Symptoms usually start in adulthood, usually in the 50s or 60s. […] The type depends on the symptoms you have when you’re diagnosed. […] The signs and symptoms are similar to those of Parkinson’s disease, such as: Stiff muscles, Difficulty bending your arms and legs, Slow movement (bradykinesia), Tremors can occur at rest or when moving your arms or legs, Soft voice, Problems with posture and balance. […] The main signs and symptoms are problems with muscle coordination (ataxia), but others may include: Impaired movement and coordination, such as unsteady gait and loss of balance, Slurred, slow or low-volume speech (dysarthria), Visual disturbances, such as blurred or double vision and difficulty focusing your eyes, Difficulty swallowing (dysphagia) or chewing, Changes in speech, such as slurred speech.
- #9 Multiple system atrophy – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/symptoms-causes/syc-20356153
The main symptoms of the cerebellar type involve poor muscle coordination, known as ataxia. Symptoms may include: Trouble with movement and coordination. This includes loss of balance and not being able to walk steadily. Slurred, slow or soft speech, known as dysarthria. Changes in vision. This may include blurred or double vision and not being able to focus the eyes. Trouble chewing or swallowing, known as dysphagia. […] For both types of multiple system atrophy, the autonomic nervous system doesn’t work properly. The autonomic nervous system controls involuntary functions in the body, such as blood pressure. When this system doesn’t work properly, it can cause the following symptoms. […] Postural hypotension is a form of low blood pressure. People who have this type of low blood pressure feel dizzy or lightheaded when they stand up after sitting or lying down. They may even faint. Not everyone with MSA has postural hypotension.
- #10 Symptoms – Multiple System Atrophy Trusthttps://www.msatrust.org.uk/what-is-msa/symptoms/
Having a diagnosis of MSA does not mean all possible symptoms will be experienced. Symptoms can change over time and progression will be individual. […] There is currently no specific treatment to prevent MSA from progressing (although clinical trials and research are being undertaken) but there are ways of assisting and alleviating symptoms. These include lifestyle adaptations, medications for specific symptoms and ensuring you get the support and advice from the professionals involved in your care. […] MSA is degenerative, which means that unfortunately symptoms are likely to worsen over a period of time. Over time the need to rely on others for help may arise. The speed of these changes is difficult to predict as people with MSA experience it differently and the speed of progression varies from person to person.
- #11 First symptoms in multiple system atrophyhttps://pmc.ncbi.nlm.nih.gov/articles/PMC5859695/
The initial symptoms of multiple system atrophy (MSA) and, in particular, early autonomic symptoms, have received less attention than motor symptoms. […] The first symptom of MSA was autonomic in 22 (73%) and motor in 3 (10%) subjects (p 0.0001). The most frequent first symptom was erectile failure, which occurred in all men beginning 4.2 2.6 years prior to diagnosis. […] The first symptoms of MSA are frequently autonomic and may predate recognition of motor manifestations. Orthostatic hypotension and, in men, erectile failure are among the first symptoms that, when evaluated in the context of associated clinical findings, may facilitate accurate and earlier diagnosis. […] The results of this study indicate that early symptoms in MSA are frequently autonomic and predate recognition of motor manifestations. At the time of initial diagnosis, careful attention to historical details found that a majority (77%) of MSA patients recalled early autonomic symptoms that initially were not recognized as being a manifestation of a neurodegenerative disease. A history of erectile failure, in particular, in men was invariably present as early as 4 or more years prior to diagnosis and very often was the first symptom. […] This study highlights the importance of paying close attention to autonomic symptoms as potential early manifestations of MSA.
- #12https://movementdisorders.ufhealth.org/for-patients/movement-disorder-information/multiple-system-atrophy-primer-many-faces-same-disease/
Importantly, it is possible for these other symptoms to precede motor symptoms by months to years. […] Disease progression unfortunately is usually more rapid than in PD and reflects a more widespread neurodegeneration in the brain. […] The combination of autonomic and motor symptoms, particularly if early in onset, can be fraught with more complications and shorter survival. […] Treatment of MSA remains largely supportive. […] About 30-60% of patients respond to typical Parkinsons medications such as carbidopa/levodopa (Sinemet), and dose trial of up to 1 gram/day of levodopa for a few months is recommended. […] Benefit seen early in disease often fades though, or becomes fraught with complications. […] Two major complications include exacerbation of orthostatic symptoms (lightheadedness, dizziness, or fainting on standing) and dyskinesias, or abnormal involuntary movements that often involve the jaw or face. […] Although there is often focus on motor symptoms, the non-motor symptoms of MSA can also be just as disabling. […] Orthostatic symptoms, dizziness and even fainting, can become very limiting and lead to the wheelchair or recliner-bound patient.
- #13 Multiple system atrophy | Altru Health Systemhttps://www.altru.org/health-library/conditions/multiple-system-atrophy
The main symptoms of the cerebellar type involve poor muscle coordination, known as ataxia. Symptoms may include: Trouble with movement and coordination. This includes loss of balance and not being able to walk steadily. Slurred, slow or soft speech, known as dysarthria. Changes in vision. This may include blurred or double vision and not being able to focus the eyes. Trouble chewing or swallowing, known as dysphagia. […] For both types of multiple system atrophy, the autonomic nervous system doesn’t work properly. The autonomic nervous system controls involuntary functions in the body, such as blood pressure. When this system doesn’t work properly, it can cause the following symptoms. […] Postural hypotension is a form of low blood pressure. People who have this type of low blood pressure feel dizzy or lightheaded when they stand up after sitting or lying down. They may even faint. Not everyone with MSA has postural hypotension.
- #14 Multiple system atrophy | Altru Health Systemhttps://www.altru.org/health-library/conditions/multiple-system-atrophy
People with MSA also can develop dangerously high blood pressure levels while lying down. This is called supine hypertension. […] These symptoms include: Constipation. Loss of bladder or bowel control, known as incontinence. […] People with multiple system atrophy may: Produce less sweat. Have heat intolerance because they sweat less. Have poor body temperature control, often causing cold hands or feet. […] Sleep symptoms may include: Agitated sleep due to „acting out” dreams. This is known as rapid eye movement (REM) sleep behavior disorder. Breathing that stops and starts during sleep, known as sleep apnea. A high-pitched whistling sound while breathing, called stridor. […] MSA may cause: Color changes in the hands and feet. […] People with multiple system atrophy also may experience: Trouble controlling emotions, such as laughing or crying when not expected.
- #15 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiologyhttps://emedicine.medscape.com/article/1154583-overview
Patients with MSA have a poor prognosis. The disease progresses rapidly. Median survivals of 6.2-9.5 years from the onset of first symptoms have been reported since the late 20th century. No current therapeutic modality reverses or halts the progress of this disease. MSA-P and MSA-C have the same survival times, but MSA-P shows more rapid dysfunctional progression. […] An older age at onset has been associated with shorter duration of survival in MSA. The overall striatonigral cell loss is correlated with the severity of disease at the time of death. […] Bronchopneumonia (48%) and sudden death (21%) are common terminal conditions in MSA. Urinary dysfunction in MSA often leads to lower urinary tract infections (UTIs); more than 50% of patients with MSA suffer from recurrent lower UTIs and a significant number die of related complications.
- #16 Multiple system atrophy – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/symptoms-causes/syc-20356153
People with MSA also can develop dangerously high blood pressure levels while lying down. This is called supine hypertension. […] These symptoms include: Constipation. Loss of bladder or bowel control, known as incontinence. […] People with multiple system atrophy may: Produce less sweat. Have heat intolerance because they sweat less. Have poor body temperature control, often causing cold hands or feet. […] Sleep symptoms may include: Agitated sleep due to „acting out” dreams. This is known as rapid eye movement (REM) sleep behavior disorder. Breathing that stops and starts during sleep, known as sleep apnea. A high-pitched whistling sound while breathing, called stridor. […] These symptoms may include: Trouble getting or keeping an erection, known as erectile dysfunction. Trouble with lubrication during sex and having an orgasm. Loss of interest in sex.
- #17https://link.springer.com/article/10.1007/s10286-017-0500-0
The first symptom of MSA was autonomic in 22 (73%) and motor in 3 (10%) subjects. […] The most frequent first symptom was erectile failure, which occurred in all men beginning 4.2 2.6 years prior to diagnosis. […] After erectile failure, postural lightheadness or fatigue following exercise, urinary urgency or hesitancy, and violent dream enactment behavior consistent with REM behavioral sleep disorder were the most frequent initial symptoms. […] The first symptoms of MSA are frequently autonomic and may predate recognition of motor manifestations. […] Orthostatic hypotension and, in men, erectile failure are among the first symptoms that, when evaluated in the context of associated clinical findings, may facilitate accurate and earlier diagnosis. […] The first presenting symptom of MSA that was recalled in retrospect was autonomic in 22 (73%), sleep-related in 5 (17%), and motor in 3 (10%) of subjects.
- #18 Multiple system atrophy | Monash Healthhttps://monashhealth.org/services/movement-disorders-program/understanding-movement-disorders/multiple-system-atrophy/
MSA can cause a range of breathing problems, such as sleep apnoea and dyspnoea and inspiratory stridor. Breathing problems can develop in the early stages of MSA. […] Sleep disorders, in particular rapid eye movement sleep behaviour disorder (RBD), may be one of the first symptoms presenting in MSA. RBD affects most people with MSA. […] Mood changes are common and may include depression, anxiety and mood swings. […] Changes to thought processes, known as cognitive changes, also occur with MSA.
- #19 The Pathobiology of Behavioral Changes in Multiple System Atrophy: An Updatehttps://www.mdpi.com/1422-0067/25/13/7464
Anxiety is defined as a neuropsychiatric disorder characterized by nervousness and loss of concentration, due to the anticipation of impending danger. At baseline, anxiety may be mild, but its severity is associated with increased disease duration and severity. […] Patients with MSA-P show deficits in executive function and higher anxiety (and depression) scores than healthy controls. […] Impulse control disorders are a group of symptoms that, according to the DSM-5, are characterized by destructive behaviors related to impulse control, including problems with self-control over oneâs emotions and behaviors. […] Isolated RBD, a parasomnia, was recently recognized as a risk factor for MSA. It is estimated that 4â5% of patients with idiopathic RBD will develop MSA, and approximately 30â40% of those with MSA exhibit RBD symptoms prior to the onset of the disease.
- #20 Multiple System Atrophy/Shy-Drager Syndrome | Vanderbilt Autonomic Dysfunction Centerhttps://www.vumc.org/autonomic-dysfunction-center/multiple-system-atrophyshy-drager-syndrome
The most common causes of death in patients with MSA are pulmonary embolus, apnea, and intercurrent infection. […] Symptoms of MSA include: Orthostatic hypotension, or a significant fall in blood pressure when standing, causing dizziness, lightheadedness, fainting, or blurred vision; urinary difficulties or constipation; motor control symptoms, including tremor, rigidity, and loss of muscle coordination, loss of balance; male impotence (inability to achieve or maintain an erection); speech or swallowing difficulties.
- #21 Multiple System Atrophy/Shy-Drager Syndrome | Vanderbilt Autonomic Dysfunction Centerhttps://www.vumc.org/autonomic-dysfunction-center/multiple-system-atrophyshy-drager-syndrome
Patients frequently note emotional lability, with short (sometimes only one or two minutes) episodes of crying due to happiness or sadness in response to relatively minor environmental stimulus, such as a song, a television program, or a movie. […] A final symptom which occurs in occasional patients with multiple system atrophy is diplopia, not unlike that seen in multiple sclerosis. […] The prognosis is more guarded in the multiple system atrophy patient than in pure autonomic failure. […] It is rare for a patient to survive 10 years. […] A significant number of patients develop laryngeal stridor and difficulty swallowing, which can lead to pneumonia. […] In addition, many patients with MSA experience Cheyne-Stokes or periodic respiration and in some cases this may lead to a critical loss of respiratory drive, so called Ondine’s curse.
- #22 Multiple System Atrophy: Causes and Treatment | Doctorhttps://patient.info/doctor/multiple-system-atrophy
Multiple system atrophy (MSA) is a rare progressive neurodegenerative disorder, caused by cell loss in areas of the brain and the spinal cord, leading to a variety of symptoms affecting especially the functions of the autonomic nervous system and the motor system. These are characterised by Parkinsonian features of varying severity, cerebellar ataxia and autonomic (particularly urogenital) dysfunction. […] The first symptoms are often autonomic and may predate recognition of motor manifestations. Orthostatic hypotension and, in men, erectile failure are among the first symptoms. Patients may also present with Parkinsonian symptoms, often with a poor or temporary response to levodopa therapy, or cerebellar dysfunction. […] Up to 49% of patients with MSA have been shown to have executive dysfunction. Cognitive impairment is more prominent in older patients with greater physical disability. Other neuropsychiatric problems may include depression, insomnia, daytime sleepiness, restless legs, hallucinations and dementia.
- #23 Multiple System Atrophy: Advances in Diagnosis and Therapyhttps://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082
Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by varying degrees of autonomic failure (AF), parkinsonism, and cerebellar ataxia. […] Symptomatic treatment has limited efficacy in MSA, and no treatments suppress or alleviate disease progression. […] The clinical diagnosis of MSA requires the presence of various combinations of AF and/or parkinsonism or cerebellar ataxia. […] The median time from disease onset that meets the criteria for probable diagnosis based on the second consensus criteria is approximately two years. […] Patients often have only AF, parkinsonism, or cerebellar ataxia early during the disease. […] In a study, following the onset of MSA, 40% of patients had mild to moderate cognitive dysfunction with or without orthostatic hypotension, which was characterized by executive dysfunction and verbal memory impairment, with depression in 28% and anxiety disorders in 22% of patients.
- #24 Multiple System Atrophy | National Institute of Neurological Disorders and Strokehttps://www.ninds.nih.gov/health-information/disorders/multiple-system-atrophy
MSA tends to progress more rapidly than Parkinson’s disease, and most people with MSA will require an aid for walking, such as a cane or walker, within a few years after symptoms begin. […] Other symptoms of MSA include: Stiffness in the hands or limbs caused by contractures (chronic shortening of muscles or tendons around joints, which prevents the joints from moving freely), A posture in which the body leans involuntarily to one side (known as Pisa syndrome), Anterocollis, in which the neck bends forward and the head drops down, Anxiety or depression, REM (Rapid Eye Movement) sleep behavior disorder (RBD) – a tendency to act out dreams, Other sleep disorders, including sleep apnea.
- #25https://link.springer.com/article/10.1007/s00415-020-09881-6
In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. […] We describe the progression of clinical features to aid the diagnosis of MSA early in the disease course. […] Their presence at presentation, even in the absence of ataxia and parkinsonism, should be regarded as highly suggestive of the premotor phase of MSA. […] MSA is characterized by worsening of motor and non-motor features over an average of 10 years, with a fast progression from disease onset, helping to distinguish it from similar degenerative conditions, described above. […] Approximately 50% of patients require walking aids within 3 years from the onset of motor symptoms; 60% require a wheelchair after 5 years with a median time to becoming bedridden of 68 years.
- #26 Multiple system atrophy – Wikipediahttps://en.wikipedia.org/wiki/Multiple_system_atrophy
The average lifespan after the onset of symptoms in patients with MSA is 6-10 years. Approximately 60% of patients require a wheelchair within five years of onset of the motor symptoms, and few patients survive beyond 12 years. The disease progresses without remission at a variable rate. Those who present at an older age, those with parkinsonian features, and those with severe autonomic dysfunction have a poorer prognosis. Those with predominantly cerebellar features and those who display autonomic dysfunction later have a better prognosis.
- #27 What is MSA? – Defeat MSA New Zealand Trusthttps://defeatmsa.org.nz/what-is-msa/
MSA is a rare, rapidly progressing neurodegenerative disorder that affects the brain. MSA impairs the systems that regulate blood pressure, heart rate and the bladder many of the basic bodily functions that people take for granted every day. People with MSA suffer from dangerously low blood pressure, speech and swallowing difficulties, sleep disturbances, breathing problems, rigidity and tremors. The life expectancy for those with MSA is typically 5 to 10 years. There is no remission of the disease. Almost 80% of patients are disabled within five years of the onset of the motor symptoms, and less than 20% of the cases survive beyond 10 years. Rate of progression and the speed of decline may vary widely from case to case. Patients with advanced MSA often become bed-bound, unable to speak and immobile.
- #28 Multiple System Atrophy: Essential Facts for Patientshttps://www.movementdisorders.org/MDS/Resources/Patient-Education/Multiple-System-Atrophy.htm
Multiple System Atrophy (MSA) is a rare disorder that affects the functioning of multiple systems in the brain. Patients may experience: Slowness of movement, muscle stiffness and/or shaking/tremor. Problems with balance and coordination. Feeling lightheaded or dizzy while standing. Problems controlling bladder function and constipation. […] Over time, symptoms increase and treatments become less effective. You may experience difficulties using utensils, swallowing solid food or liquids, walking and controlling bladder or bowels. […] In advanced stages, MSA patients have increased risk of infections. Bladder symptoms can lead to urinary tract infections. Swallowing problems increase the risk of pneumonia. Overall, long-term care planning may be needed as the disease progresses.
- #29 Dysautonomia International: Summary of Multiple System Atrophyhttp://www.dysautonomiainternational.org/page.php?ID=33
Unfortunately, MSA usually progresses rapidly over a period of 7 to 10 years, with the mean survival rate of 9.3 years from the time of the first symptom. About 80% of patients are disabled within 5 years of the onset of motor symptoms. It is estimated that only 20% of MSA patients survive beyond 12 years. Patients continue to experience neurological degeneration until they lose motor skills, become confined to bed, and eventually pass away. Many MSA patients succumb to pneumonia and other respiratory infections, choking or cardiac arrest. MSA does not go into remission and there is no cure at this time.
- #30 Multiple System Atrophy: Advances in Diagnosis and Therapyhttps://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082
A natural history study showed that low vitamin B12 levels were associated with shorter survival, increased frequency of falls within three years of onset, and a lower body mass index, suggesting a potential new target for therapeutic intervention. […] Further studies are needed to determine whether serotonin-targeted therapy alleviates several symptoms and modifies the disease course of MSA.
- #31 Laboratory prognostic factors for the long-term survival of multiple system atrophy | npj Parkinson’s Diseasehttps://www.nature.com/articles/s41531-022-00413-9
Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disease with a mean survival of 6-10 years from disease onset. […] Among clinical biomarkers, early autonomic failure, older age of onset, and absence of response to levodopa have been associated with shorter survival. […] 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.
- #32 Multiple system atrophy – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/diagnosis-treatment/drc-20356157
Diagnosing multiple system atrophy (MSA) can be challenging. Symptoms such as stiffness and trouble walking can happen in other diseases, including Parkinson’s disease. This can make MSA hard to diagnose. […] Treatment for multiple system atrophy (MSA) involves managing your symptoms. There’s no cure for MSA. Managing the disease can make you as comfortable as possible and help you maintain your body functions. […] Many people with multiple system atrophy do not respond to Parkinson’s medicines. The medicines also may become less effective after a few years. […] If you have trouble swallowing, try eating softer foods. If swallowing or breathing symptoms become worse, you might need surgery to insert a feeding or breathing tube. A gastrostomy tube delivers food directly into your stomach.
- #33 Multiple system atrophy (MSA) | NHS informhttps://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/multiple-system-atrophy-msa/
It’s more likely you have MSA if: your symptoms have progressed quickly; you’ve had falls in the early stages of the condition; you don’t respond well to the medicine levodopa; your speech has been severely affected; you gasp and breathe noisily. […] There isn’t a cure for MSA and there isn’t a way of slowing its progress. […] As symptoms worsen, you might need help managing day to day life. […] Movement problems: You might be prescribed medication to help with the stiffness and slowness caused by MSA. Their benefits are limited and they can make blood pressure problems worse. […] Balance difficulties: Physiotherapists and occupational therapists can provide advice and equipment to help you live safely. […] Bladder control: Urology and continence specialists can recommend treatment to help manage bladder problems.
- #34 Multiple System Atrophy – Parkinson’s Grouphttps://parkinsonstreatment.org/multiple-system-atrophy/
The condition progresses gradually and eventually leads to death. […] More crippling symptoms, such as severe tightness muscles or contractures can be treated with muscle relaxants and sometimes by injection of botulinum toxin (i.e., Botox). […] Prevention of falls and aspiration (pneumonia) are major goals as these frequently lead to worsening disability and even death. […] If these are not enough, drugs like droxidopa and blood volume increasers (fludrocortisone) or pressor agents (e.g., midodrine) are used to prevent changes in blood pressures.
- #35 Multiple system atrophy – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/diagnosis-treatment/drc-20356157
If you have trouble with bladder control, medicines can help in the earlier stages. But as MSA gets worse, you may need to have a soft tube inserted to drain your bladder. The soft tube is known as a catheter. […] A physical therapist can help you maintain as much of your movement and strength as possible as the disease gets worse.
- #36 Multiple system atrophy (MSA) | NHS informhttps://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/multiple-system-atrophy-msa/
Constipation: You should try to drink plenty fluids and eat a well balanced diet to try to avoid becoming constipated. […] Sexual problems: Erectile dysfunction (inability to get or maintain an erection) is often the first symptom of MSA. […] Dizziness: You might feel dizzy when you stand up or change position because your blood pressure falls quickly. […] Communication and swallowing problems: You might see a speech and language therapist who’ll try to help with your problems with speech and swallowing (dysphagia). […] Eating problems: A dietitian will help advise you how to eat a healthy balanced diet including more food and liquids that are easy to swallow. […] Breathing problems: You might be referred to a respiratory team if you have breathing problems like sleep apnoea or stridor.
- #37 Multiple System Atrophy Symptoms & Treatment | Pacific Movement Disordershttps://www.pacificneuroscienceinstitute.org/movement-disorders/conditions/atypical-parkinsonism/multiple-system-atrophy/
Cerebellar MSA, previously known as Olivopontocerebellar atrophy (OPCA) or olivopontocerebellar degeneration (OPCD). This combines parkinsonism, autonomic dysfunction as above, and cerebellar findings such as imbalance, staggering gait, lack of coordination, and eye movement abnormalities. The cerebellar findings may precede the parkinsonism. […] Parkinsonian MSA, previously known as striatonigral degeneration (SND), manifests with parkinsonism (moving slowly, tremor and stiffness) and autonomic dysfunction. Other symptoms associated with MSA include REM sleep behavior disorder (RBD), which is where there is an absence of the usual paralysis during dream sleep (rapid eye movement REM), resulting in acting out dreams; contractures; leaning to one side; depression and anxiety. […] The treatment of MSA is primarily based on alleviating symptoms; no cure exists at this time. Patients should be monitored closely for severe issues such as breathing problems during sleep, supine hypertension (high blood pressure when lying down) and orthostatic hypotension (low blood pressure upon standing).
- #38 Multiple System Atrophy: Symptoms & Treatment | Massachusetts General Hospitalhttps://www.massgeneral.org/neurology/treatments-and-services/multiple-system-atrophy
In addition to the shared symptoms of MSA, patients with cerebellar MSA may experience: Movement problems: Poor coordination and balance can make walking difficult or cause falls. Speech problems: You may notice slurred speech, slow speech patterns, or speaking at a very low volume. Vision problems: Blurred vision, double vision, or trouble focusing may occur. […] MSA-P symptoms can be similar to Parkinson’s disease. Common symptoms include: Low vocal volume, Muscle stiffness that makes it difficult to bend your arms and legs, Posture and balance issues, Slow movement, Abnormal movements. […] Unlike some other diseases (like cancer), multiple system atrophy doesn’t have clearly defined stages. However, MSA tends to progress steadily after diagnosis. One of the earliest symptoms for many people is sexual dysfunction, such as erectile dysfunction in men or decreased sensitivity in women. However, most cases of sexual dysfunction are not caused by MSA. Other early symptoms can include orthostatic hypotension, urinary retention, and REM sleep behavior disorder. End-stage complications often include pneumonia and issues related to prolonged bedrest or breathing problems, such as aspiration pneumonia or pulmonary embolus.
- #39https://pspawareness.com/blogs/psp-q-a/navigating-the-four-stages-of-multiple-system-atrophy-msa?srsltid=AfmBOorCnP_Gi19iPDsvf2OSd7e2tmSIafnfpuOrnC3RP_Pyb13FavQU
Multiple System Atrophy (MSA) is a rare, degenerative neurological disorder that impacts various systems in the body, notably the autonomic functions, movement, and sometimes cognition. While the progression of MSA can differ greatly from one individual to another, understanding the typical stages can be invaluable for planning care, setting realistic expectations, and preparing for the journey ahead. Here, we outline the four general stages of MSA, each marked by distinctive symptoms and challenges. […] It’s essential to approach this guide with the understanding that MSA does not follow a one-size-fits-all path. Each person’s experience with this condition can vary in symptoms, progression speed, and severity. This blog post serves as a guideline to help anticipate potential changes, but always consult with healthcare professionals for advice tailored to your or your loved one’s unique circumstances.
- #40https://pspawareness.com/blogs/psp-q-a/understanding-the-four-stages-of-multiple-system-atrophy-msa-with-detailed-symptom-description?srsltid=AfmBOor7BW-hQO2cmitOgETtHpW9WHioi-jLBdcNPeBXlje86juRA5v0
Multiple System Atrophy (MSA) is a rare, progressive neurodegenerative disorder that impacts movement, balance, and autonomic functions. […] Here’s a closer look at the symptoms across its four stages: […] Stage 1: Early Symptoms […] Motor Symptoms: – Mild Ataxia: Slight awkwardness or unsteadiness, particularly noticeable when walking or performing fine motor tasks. – Mild Parkinsonism: Subtle rigidity or slowness in movement, often mistaken for early Parkinson’s disease. […] Autonomic Symptoms: – Orthostatic Hypotension: Dizziness or lightheadedness upon standing due to a drop in blood pressure. – Urinary Symptoms: Increased frequency, urgency, or hesitancy in urination. […] Other Symptoms: – Sleep Disorders: REM Sleep Behavior Disorder, where individuals act out their dreams, often leading to disrupted sleep. – Mild Cognitive Changes: Slight difficulties with concentration or executive function.
- #41https://pspawareness.com/blogs/psp-q-a/understanding-the-four-stages-of-multiple-system-atrophy-msa-with-detailed-symptom-description?srsltid=AfmBOor7BW-hQO2cmitOgETtHpW9WHioi-jLBdcNPeBXlje86juRA5v0
Stage 2: Progression of Symptoms […] Motor Symptoms: – Increased Rigidity: More pronounced stiffness in muscles. – Tremor: May develop or become more noticeable, particularly postural or action tremor. – Balance Issues: Increased risk of falls due to worsening ataxia or Parkinsonism. […] Autonomic Symptoms: – Worsening Orthostatic Hypotension: More frequent and severe drops in blood pressure upon standing. – Bladder Dysfunction: Incontinence or retention becomes more problematic. – Sexual Dysfunction: Reduced libido or erectile dysfunction in men, vaginal dryness in women. […] Other Symptoms: – Sleep Disorders: Intensification of sleep issues, including loud snoring or sleep apnea. – Sweating Abnormalities: Either excessive or reduced sweating. […] Stage 3: Significant Disability […] Motor Symptoms: – Severe Ataxia and Parkinsonism: Walking becomes challenging; many require assistance or mobility aids. – Dysarthria: Speech becomes slurred, making communication difficult. – Dysphagia: Swallowing difficulties, increasing the risk of choking or aspiration.
- #42https://pspawareness.com/blogs/psp-q-a/understanding-the-four-stages-of-multiple-system-atrophy-msa-with-detailed-symptom-description?srsltid=AfmBOor7BW-hQO2cmitOgETtHpW9WHioi-jLBdcNPeBXlje86juRA5v0
Autonomic Symptoms: – Extreme Blood Pressure Fluctuations: Can lead to syncope (fainting) or severe hypertension. – Gastrointestinal Issues: Constipation or, less commonly, diarrhea. – Thermal Regulation Problems: Difficulty maintaining body temperature. […] Other Symptoms: – Cognitive Decline: More pronounced issues with memory, attention, or executive functions. – Vision Problems: Blurred vision or difficulty with eye movements. […] Stage 4: End-Stage Disease […] Motor Symptoms: – Severe Mobility Limitation: Most or all mobility lost, often bedridden. – Severe Dysarthria and Dysphagia: Communication is severely impaired, and feeding might require tube feeding due to swallowing risks. […] Autonomic Symptoms: – Critical Autonomic Failures: Life-threatening drops in blood pressure, severely impaired body temperature regulation. – Respiratory Complications: Increased risk of pneumonia due to aspiration or weakened respiratory muscles.
- #43https://pspawareness.com/blogs/psp-q-a/understanding-the-four-stages-of-multiple-system-atrophy-msa-with-detailed-symptom-description?srsltid=AfmBOor7BW-hQO2cmitOgETtHpW9WHioi-jLBdcNPeBXlje86juRA5v0
Other Symptoms: – Cognitive Impairment: Significant cognitive decline, potentially leading to dementia-like symptoms. – General Health Decline: Increased susceptibility to infections, pressure sores from immobility, and other complications. […] The progression of MSA is individual, with symptoms varying in intensity and onset. Early recognition of these stages can guide medical management, focusing on symptom control, safety, and quality of life.
- #44 Multiple System Atrophy vs. Parkinsonâs Diseasehttps://www.healthline.com/health/parkinsons/multiple-system-atrophy-vs-parkinsons
Multiple system atrophy and Parkinsons disease are progressive nervous system disorders that affect everyday movements. Multiple system atrophy is rarer and tends to progress more quickly. […] Both MSA and PD progress over time if they are not treated. Depending on the MSA subtype, the two conditions can have some of the same symptoms. […] MSA can also cause: joint stiffness, Pisa syndrome (sideways leaning), anterocollis (a forward tilt of your head), sleep apnea, autonomic dysfunction, such as problems with your heart rate and digestion. […] MSA can progress rapidly, within 5 to 10 years. Many people need walking aids within a few years of diagnosis, and many lose the ability to move around. […] Life expectancy for people with MSA is 7 to 9 years.
- #45 Multiple System Atrophy (MSA) – Neurologic Disorders – Merck Manual Professional Editionhttps://www.merckmanuals.com/professional/neurologic-disorders/autonomic-nervous-system/multiple-system-atrophy-msa
Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. Symptoms include hypotension, urinary retention, constipation, ataxia, parkinsonism, and postural instability. […] After symptoms appear, patients live about 9 to 10 years. […] Both types involve autonomic nervous system dysfunction. Although multiple system atrophy begins as one type, symptoms of the other type eventually develop. After about 5 years, symptoms tend to be similar regardless of which disorder developed first. […] Parkinsonian symptoms predominate in striatonigral degeneration. They include rigidity, bradykinesia, postural instability, and jerky postural tremor. […] In contrast to Parkinson disease, multiple system atrophy usually does not usually cause resting tremor and dyskinesia, and symptoms respond poorly and transiently to levodopa.
- #46 Multiple system atrophy – Wikipediahttps://en.wikipedia.org/wiki/Multiple_system_atrophy
Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by tremors, slow movement, muscle rigidity, postural instability (collectively known as parkinsonism), autonomic dysfunction and ataxia. This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. […] Many people affected by MSA experience dysfunction of the autonomic nervous system, which commonly manifests as orthostatic hypotension, impotence, loss of sweating, dry mouth and urinary retention and incontinence. Palsy of the vocal cords is an important and sometimes initial clinical manifestation of the disorder. […] MSA often presents with some of the same symptoms as Parkinson’s disease. However, those with MSA generally show little response to the dopamine agonists used to treat Parkinson’s disease and only about 9% of MSA patients with tremor exhibit a true parkinsonian pill-rolling tremor.
- #47 Multiple System Atrophy-D Maybe Something AltogetherâDifferenthttps://practicalneurology.com/diseases-diagnoses/movement-disorders/multiple-system-atrophy-d-maybe-something-altogetherdifferent/30257/
The incidence of MSA is 3:100,000 in adults aged over 50 years, and the course of illness lasts 5 to 10 years. There are 2 known groups: MSA-P (parkinsonian) and MSA-C (cerebellar) that have clinical presentations associating them closely with more common movement disorders (eg, Parkinsons disease [PD] and ataxia). Patients with MSA often move slowly and have gait and balance problems, autonomic dysfunction, corticospinal tract findings, and sometimes tremor or stiffness. MSA is not as responsive to treatment, and the clinical course of worsening symptoms seems accelerated compared to PD. […] Many nonmotor symptoms seen in patients with PD are more clinically significant in patients with MSA. […] For establishing orthostatic hypotension to diagnose probable MSA, the 2008 consensus criteria give a definition of a drop in systolic blood pressure by 30 mm Hg or more, or a drop in diastolic blood pressure by 15 mm Hg or more after 3 minutes. Diagnosis of possible MSA does not require such strict parameters for orthostatic hypotension.
- #48 Multiple System Atrophy-D Maybe Something AltogetherâDifferenthttps://practicalneurology.com/diseases-diagnoses/movement-disorders/multiple-system-atrophy-d-maybe-something-altogetherdifferent/30257/
Bladder dysfunction occurs in patients with both PD and MSA, and measurement of postvoid residuals (PVRs) can also help distinguish between PD and MSA. PVR is elevated in patients with MSA because there is more urinary retention compared to those with PD, which has more prevalence of detrusor overactivity. […] Although it is likely that MSA and PD will always be intertwined, MSA is emerging as a distinct pathology.