Stwardnienie zanikowe boczne
Objawy

Stwardnienie zanikowe boczne (ALS) to postępująca, nieuleczalna choroba neurodegeneracyjna charakteryzująca się zwyrodnieniem górnych i dolnych neuronów ruchowych w mózgu i rdzeniu kręgowym. Średni wiek zachorowania wynosi 40-70 lat, z dominującym początkiem między 55 a 75 rokiem życia. Choroba manifestuje się osłabieniem mięśni, fascykulacjami, spastycznością, dyzartrią i dysfagią, a jej przebieg jest zmienny – od szybkiego pogorszenia w ciągu kilku miesięcy do stabilizacji trwającej miesiące. ALS dzieli się na postać kończynową (70-80% przypadków) i opuszkową (20-25%), z różnym profilem objawów początkowych. Progresja choroby prowadzi do nasilającego się zaniku mięśni, paraliżu, zaburzeń mowy, połykania oraz niewydolności oddechowej, która jest główną przyczyną zgonów. Średni czas przeżycia od diagnozy wynosi 2-5 lat, choć 5-10% pacjentów żyje ponad 10 lat.

Charakterystyka stwardnienia zanikowego bocznego

Stwardnienie zanikowe boczne (ALS, choroba Lou Gehriga) to postępująca, nieuleczalna choroba neurodegeneracyjna, która prowadzi do nieodwracalnego zwyrodnienia neuronów ruchowych w mózgu i rdzeniu kręgowym. ALS wpływa na górne neurony ruchowe znajdujące się w mózgu oraz dolne neurony ruchowe zlokalizowane w pniu mózgu i rdzeniu kręgowym. Neurony ruchowe są odpowiedzialne za kontrolowanie ruchów mięśni, a ich stopniowe obumieranie prowadzi do postępującego osłabienia mięśni, ich zaniku i ostatecznie paraliżu.123

Średni wiek zachorowania na ALS wynosi 40-70 lat, przy czym najczęściej choroba pojawia się między 55 a 75 rokiem życia. Choroba zwykle postępuje w ciągu 2-5 lat od diagnozy, jednak około 20% pacjentów żyje dłużej niż 5 lat, a około 5-10% żyje 10 lat lub dłużej.456

Pierwsze objawy stwardnienia zanikowego bocznego

Początkowo objawy ALS mogą być subtelne i często pozostają niezauważone. Każdy przypadek ALS jest unikalny, a przebieg choroby może się znacznie różnić między pacjentami.7 Objawy mogą pojawić się stopniowo lub rozwinąć się szybko, a następnie stabilizować przez pewien okres.8

Objawy początkowe w zależności od miejsca wystąpienia

ALS może rozpocząć się od objawów w różnych częściach ciała. Rozróżniamy dwa główne typy początku choroby:

  1. Postać kończynowa (limb-onset) – dotyczy około 70-80% pacjentów. Pierwsze objawy pojawiają się w kończynach górnych lub dolnych.910
    • W kończynach górnych: zmniejszona zręczność palców, trudności z chwytaniem przedmiotów, osłabienie dłoni, skurcze i sztywność mięśni, opadanie nadgarstka.1112
    • W kończynach dolnych: potykanie się, upadki, niezgrabność podczas chodzenia lub biegania, osłabienie mięśni nóg, stóp lub kostek, opadanie stopy.1314
  2. Postać opuszkowa (bulbar-onset) – występuje u około 20-25% pacjentów. Pierwsze objawy dotyczą mięśni twarzy, szyi, języka i gardła.1516
    • Trudności w mówieniu (dyzartria), bełkotliwa lub nosowa mowa17
    • Problemy z przełykaniem (dysfagia)18
    • Osłabienie mięśni twarzy i języka19

Najczęstsze wczesne objawy ALS

Do typowych wczesnych objawów ALS należą:

  • Osłabienie mięśni – początkowo może dotyczyć tylko jednej kończyny lub obszaru ciała20
  • Fascykulacje (drżenia pęczkowe mięśni) – niekontrolowane drgania mięśni, szczególnie w rękach, nogach, ramionach lub języku21
  • Skurcze mięśni – mogą być bolesne i często występują we wczesnej fazie choroby22
  • Sztywność i napięcie mięśni (spastyczność)23
  • Zaburzenia mowy – mowa staje się bełkotliwa, nosowa lub zamazana24
  • Trudności z przełykaniem lub żuciem25
  • Nadmierne zmęczenie26
  • Zaburzenia równowagi i problemy z chodzeniem27
  • Osłabiony chwyt28

We wczesnym stadium ALS większość pacjentów zachowuje znaczną funkcjonalność i niezależność. Ten etap choroby zwykle trwa około roku.29

Progresja choroby

Stwardnienie zanikowe boczne jest chorobą postępującą, co oznacza, że objawy z czasem ulegają nasileniu. Progresja ALS może być różna u każdego pacjenta – u niektórych choroba postępuje szybko w ciągu kilku miesięcy, u innych może rozwijać się powoli przez wiele lat.30

Stadium średniozaawansowane

W miarę postępu choroby objawy stają się bardziej rozpowszechnione i intensywne:31

  • Osłabienie mięśni rozprzestrzenia się na inne części ciała32
  • Nasilenie zaniku mięśni (atrofia)33
  • Niektóre mięśnie są całkowicie sparaliżowane, podczas gdy inne są osłabione lub niezmienione34
  • Fascykulacje (drżenia pęczkowe) nadal występują35
  • Problemy z mową stają się bardziej nasilone – mowa jest wolniejsza, bełkotliwa i trudna do zrozumienia36
  • Nasilenie trudności z przełykaniem – pacjenci mają problemy z połykaniem płynów i pokarmów, co może prowadzić do zakrztuszeń i aspiracji3738
  • Nadmierne ślinienie się z powodu osłabienia mięśni twarzy i trudności z połykaniem śliny39
  • Problemy z oddychaniem – osłabienie mięśni oddechowych prowadzi do niewydolności oddechowej, szczególnie w pozycji leżącej40
  • Zwiększona męczliwość41
  • Problemy z poruszaniem się – pacjenci mogą potrzebować pomocy w chodzeniu, a z czasem wózka inwalidzkiego42

W tym stadium pacjenci zachowują zdolność do samodzielnego poruszania niektórymi częściami ciała, jednak zmiany związane z ALS zaczynają znacząco ograniczać mobilność.43

Stadium zaawansowane

W zaawansowanym stadium ALS większość mięśni odpowiedzialnych za ruchy dowolne ulega paraliżowi:44

  • Prawie całkowity paraliż mięśni szkieletowych – pacjenci mają bardzo ograniczoną lub żadną możliwość poruszania się samodzielnie45
  • Poważne problemy z oddychaniem – zdolność przepływu powietrza przez płuca jest znacznie ograniczona, co często wymaga wspomagania oddychania (wentylacji mechanicznej)4647
  • Całkowita utrata zdolności mówienia48
  • Niemożność samodzielnego jedzenia i picia – pacjenci wymagają karmienia przez zgłębnik49
  • Całkowita zależność od opiekunów – wszystkie potrzeby pacjenta muszą być zaspokajane przez opiekunów50
  • Problemy z koncentracją, bóle głowy i zwiększona podatność na zapalenie płuc z powodu niewydolności oddechowej51

W tym stadium pacjenci zazwyczaj wymagają całodobowej opieki i wsparcia w wykonywaniu wszystkich codziennych czynności.52 Ostatnie stadium ALS może trwać od kilku tygodni do kilku miesięcy, w zależności od tempa progresji choroby.53

Specyficzne objawy i powikłania ALS

Problemy z oddychaniem

Osłabienie mięśni oddechowych jest jednym z najpoważniejszych powikłań ALS:54

  • Stopniowe osłabienie przepony i mięśni międzyżebrowych prowadzi do niewydolności oddechowej55
  • Duszność początkowo pojawia się podczas aktywności fizycznej, a następnie także w spoczynku i w pozycji leżącej56
  • Pacjenci mają trudności z wytworzeniem silnego kaszlu, co zwiększa ryzyko zakrztuszenia i aspiracyjnego zapalenia płuc57
  • W miarę postępu choroby pacjenci mogą wymagać nieinwazyjnej wentylacji (NIV), a następnie wentylacji mechanicznej58
  • Niewydolność oddechowa jest najczęstszą przyczyną śmierci u pacjentów z ALS59

Problemy z mową i połykaniem

Zaburzenia mowy i połykania są częstymi objawami ALS, szczególnie w postaci opuszkowej:

  • Dyzartria (zaburzenia artykulacji) – mowa staje się bełkotliwa, nosowa i trudna do zrozumienia60
  • Dysfagia (trudności w połykaniu) – początkowo dotyczy płynów, a później także pokarmów stałych61
  • Zwiększone ryzyko zakrztuszenia i aspiracji pokarmu do płuc62
  • Ślinotok spowodowany trudnościami w połykaniu śliny63
  • Problemy z odżywianiem prowadzące do niedożywienia i odwodnienia64

Zaburzenia poznawcze i emocjonalne

Choć ALS przede wszystkim wpływa na funkcje motoryczne, u niektórych pacjentów występują również zmiany poznawcze i emocjonalne:

  • U około 15-50% pacjentów z ALS rozwija się otępienie czołowo-skroniowe (FTD) lub łagodniejsze zaburzenia poznawcze6566
  • Zaburzenia funkcji wykonawczych i podejmowania decyzji67
  • Zmiany osobowości i zachowania68
  • Pseudoopuszkowy afekt – niekontrolowane wybuchy śmiechu lub płaczu, nieadekwatne do sytuacji6970
  • Depresja – często występuje jako reakcja na postępującą chorobę71

Inne objawy towarzyszące

  • Zmęczenie – może być spowodowane zwiększonym wysiłkiem potrzebnym do wykonywania codziennych czynności, zaburzeniami oddychania lub metabolicznymi zmianami związanymi z chorobą72
  • Utrata masy ciała – wynika z zaniku mięśni, trudności w połykaniu i zwiększonego zapotrzebowania metabolicznego73
  • Zaparcia – mogą być wynikiem osłabienia mięśni odpowiedzialnych za perystaltykę jelitową lub skutkiem ubocznym niektórych leków74

Nietypowy przebieg ALS

Chociaż ALS zwykle charakteryzuje się postępującym pogorszeniem funkcji motorycznych, istnieją pewne odchylenia od typowego przebiegu choroby:75

  • U niektórych pacjentów mogą występować okresy stabilizacji objawów trwające kilka tygodni lub miesięcy, podczas których nie obserwuje się znaczącej utraty funkcji76
  • W bardzo rzadkich przypadkach (mniej niż 1% pacjentów) może wystąpić przejściowa poprawa funkcji, choć zazwyczaj jest ona krótkotrwała77
  • Badania wykazały, że progresja ALS często przebiega nieliniowo, z okresami stabilnej choroby poprzedzającymi lub następującymi po okresach szybkiego pogorszenia78
  • Zidentyfikowano różne wzorce progresji ALS, w tym: szybką progresję sigmoidalną, powolną stabilną progresję, niestabilną powolną progresję oraz niestabilną progresję o średnim nasileniu79

Czynniki wpływające na szybkość progresji

Tempo progresji ALS może się znacznie różnić między pacjentami. Na szybkość postępu choroby wpływają różne czynniki:80

  • Wiek zachorowania – młodsi pacjenci zwykle doświadczają wolniejszej progresji choroby81
  • Miejsce początkowego wystąpienia objawów – postać opuszkowa (bulbar-onset) zwykle postępuje szybciej niż postać kończynowa (limb-onset)82
  • Czynniki genetyczne – niektóre mutacje genetyczne są związane z szybszym lub wolniejszym postępem choroby83
  • Obecność zaburzeń poznawczych – pacjenci z towarzyszącym otępieniem czołowo-skroniowym (FTD) zwykle mają gorsze rokowanie84
  • Wczesne wystąpienie objawów opuszkowych lub oddechowych – wiąże się z gorszym rokowaniem85

Skale oceny progresji ALS

Do monitorowania postępu choroby stosuje się różne skale oceny, najczęściej:

  • ALS Functional Rating Scale – Revised (ALSFRS-R) – 12-punktowa skala oceniająca funkcjonowanie pacjenta w różnych obszarach. Wynik waha się od 48 (prawidłowa funkcja) do 0 (ciężka niepełnosprawność). Średnio pacjenci z ALS tracą około 1 punkt ALSFRS-R miesięcznie, choć stopień zmienności jest wysoki.86
  • Skala King’s – ocenia progresję ALS na podstawie liczby zajętych obszarów ośrodkowego układu nerwowego i obecności niewydolności oddechowej lub żywieniowej.8788
  • Skala Milano-Torino (MiToS) – opiera się na utracie kluczowych funkcji życiowych.89

Podsumowanie progresji ALS

Stwardnienie zanikowe boczne jest chorobą postępującą, której przebieg może się różnić między pacjentami. Ogólny schemat progresji obejmuje:

  1. Wczesne stadium – subtelne objawy ograniczone do jednego obszaru ciała, najczęściej kończyn lub mięśni opuszkowych
  2. Stadium średniozaawansowane – rozprzestrzenianie się objawów na inne części ciała, nasilenie osłabienia mięśni, trudności w mówieniu i połykaniu, problemy z oddychaniem
  3. Stadium zaawansowane – paraliż większości mięśni szkieletowych, utrata zdolności samodzielnego oddychania, mówienia i jedzenia, całkowita zależność od opiekunów

Średni czas przeżycia od wystąpienia pierwszych objawów wynosi 2-5 lat, choć około 10% pacjentów żyje 10 lat lub dłużej.9091 Niewydolność oddechowa jest najczęstszą przyczyną śmierci u pacjentów z ALS.92

Chociaż nie ma obecnie leków, które mogłyby odwrócić uszkodzenia spowodowane przez ALS, dostępne są terapie mogące spowolnić postęp choroby i poprawić jakość życia pacjentów. Kompleksowe podejście do leczenia, obejmujące leki, rehabilitację, wsparcie oddechowe i żywieniowe, może pomóc pacjentom zachować funkcjonalność i komfort przez dłuższy czas.9293

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

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

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

Materiały źródłowe

  • #1 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), known as ALS, is a nervous system disease that affects nerve cells in the brain and spinal cord. ALS causes loss of muscle control. The disease gets worse over time. […] ALS often begins with muscle twitching and weakness in an arm or leg, trouble swallowing or slurred speech. Eventually ALS affects control of the muscles needed to move, speak, eat and breathe. There is no cure for this fatal disease. […] Symptoms of ALS vary from person to person. Symptoms depend on which nerve cells are affected. ALS generally begins with muscle weakness that spreads and gets worse over time. Symptoms might include: Trouble walking or doing usual daily activities. Tripping and falling. Weakness in the legs, feet or ankles. Hand weakness or clumsiness. Slurred speech or trouble swallowing. Weakness associated with muscle cramps and twitching in the arms, shoulders and tongue. Untimely crying, laughing or yawning. Thinking or behavioral changes.
  • #2 ALS – Wikipedia
    https://en.wikipedia.org/wiki/ALS
    Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or Lou Gehrig’s disease, is a rare, terminal neurodegenerative disorder that results in the progressive loss of both upper and lower motor neurons that normally control voluntary muscle contraction. ALS often presents in its early stages with gradual muscle stiffness, twitches, weakness, and wasting. Motor neuron loss typically continues until the abilities to eat, speak, move, and, lastly, breathe are all lost. While only 15% of people with ALS also fully develop frontotemporal dementia, an estimated 50% face at least some minor difficulties with thinking and behavior. Depending on which of the aforementioned symptoms develops first, ALS is classified as limb-onset (begins with weakness in the arms or legs) or bulbar-onset (begins with difficulty in speaking or swallowing).
  • #3 The 7 Stages of ALS: The Progression of a Nonlinear Disease
    https://www.targetals.org/2022/01/04/the-stages-of-als-framing-the-progression-of-a-nonlinear-disease/
    Amyotrophic lateral sclerosis (ALS) is a progressive, incurable neurodegenerative disease that leads to the irreversible degeneration of motor neurons. […] Typically, the disease will progress over 2 to 5 years after diagnosis. However, 20% of patients live for more than 5 years, and about 5% live for 20 years or more. […] As the disease advances, muscle weakness and atrophy spread, leading to difficulties with moving, speaking or forming words (dysarthria), breathing (dyspnea) and swallowing (dysphagia). […] Early symptoms can include muscle weakness, spasticity (tightness), cramping, and/or fasciculations (twitching). […] Bulbar-onset ALS begins in the face or neck. Early symptoms include slurred speech and hoarseness. […] In the middle stage of ALS, the early-stage symptoms become worse. Muscle atrophy will spread to other parts of the body, increasing weakness.
  • #4 Amyotrophic Lateral Sclerosis (ALS) > Fact Sheets > Yale Medicine
    https://www.yalemedicine.org/conditions/amyotrophic-lateral-sclerosis
    Symptoms usually begin gradually and worsen over time. The rate of progression, however, can vary greatly from person to person. Most people with the condition eventually die of respiratory failure. On average, people with ALS live 3 to 5 years after symptoms begin. However, about 30% of people with ALS live five years, 13% will survive 10 years, and some will live 20 years or more.
  • #5 The 7 Stages of ALS: The Progression of a Nonlinear Disease
    https://www.targetals.org/2022/01/04/the-stages-of-als-framing-the-progression-of-a-nonlinear-disease/
    Amyotrophic lateral sclerosis (ALS) is a progressive, incurable neurodegenerative disease that leads to the irreversible degeneration of motor neurons. […] Typically, the disease will progress over 2 to 5 years after diagnosis. However, 20% of patients live for more than 5 years, and about 5% live for 20 years or more. […] As the disease advances, muscle weakness and atrophy spread, leading to difficulties with moving, speaking or forming words (dysarthria), breathing (dyspnea) and swallowing (dysphagia). […] Early symptoms can include muscle weakness, spasticity (tightness), cramping, and/or fasciculations (twitching). […] Bulbar-onset ALS begins in the face or neck. Early symptoms include slurred speech and hoarseness. […] In the middle stage of ALS, the early-stage symptoms become worse. Muscle atrophy will spread to other parts of the body, increasing weakness.
  • #6 Amyotrophic Lateral Sclerosis (ALS) | Living With Paralysis
    https://www.christopherreeve.org/todays-care/living-with-paralysis/health/causes-of-paralysis/als/
    Amyotrophic Lateral Sclerosis (ALS) (sometimes called Lou Gehrigs Disease) is a neurological disease that primarily affects the nerves of the brainstem, spinal cord and cerebral cortex of the brain. […] ALS affects function most often as upper extremity muscle weakness and wasting. Some individuals will also have the same muscle effects in the lower extremities. Difficulty in controlling speech (dysarthria) and swallowing (dysphagia) occur. As ALS progresses breathing can be affected. […] Symptoms of ALS appear and progress differently in individuals. Some people will notice difficulty with extremities while others will first notice problems with vocal changes. ALS manifests and develops in different ways, depending on which muscles weaken first and how the disease progresses. Symptoms may include: Gradual onset, generally painless, general muscle weakness; Abnormal fatigue of the arms, legs or entire body: dropping things, tripping and falling; Loss of control in the hands and arms; Difficulty speaking, chewing, swallowing and/or breathing, slurred or nasal speech; Muscle cramps (spasms) or twitches (fasciculations) in the shoulders, tongue or legs; Muscle weakness in arm, leg or diaphragm; Uncontrollable periods of laughing or crying.
  • #7 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #8 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #9 ALS (Lou Gehrig’s Disease): Causes, Symptoms & Treatment
    https://www.healthline.com/health/amyotrophic-lateral-sclerosis
    Amyotrophic lateral sclerosis (ALS) is a degenerative disease that affects the brain and spinal cord. It causes a worsening loss of voluntary muscle control affecting movements like talking, swallowing, and walking. […] Both sporadic and familial ALS are associated with a progressive loss of motor neurons. As motor neurons become damaged, a person with ALS will start to have difficulty with voluntary movements in their limbs, mouth, or throat. […] Around 70% of people with ALS have whats known as limb onset ALS. This means that symptoms first appear in the arms or legs. […] Symptoms in the arms (upper limb onset) include: weakness in hands, stiff arms or hands, cramps in arms or hands, loss of dexterity, fumbling, or dropping objects. […] Symptoms in the legs (lower limb onset) include: trouble with walking or running, tripping or stumbling, difficulty lifting the front half of the foot when walking, known as foot drop.
  • #10 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Amyotrophic lateral sclerosis (ALS) is the most common degenerative disease of the motor neuron system. Although ALS is incurable and fatal, with median survival of 3 years, treatment can extend the length and meaningful quality of life for patients. […] In 7580% of patients, symptoms begin with limb involvement. Initial complaints in patients with lower limb onset are often as follows: Tripping, stumbling, or awkwardness when running; Loss of lower extremity muscle bulk, strength, or endurance; Foot drop; patients may report a „slapping” gait. […] Initial complaints with upper limb onset include the following: Reduced finger dexterity, cramping, stiffness, and weakness or wasting of intrinsic hand muscles; Wrist drop interfering with work performance. […] With bulbar onset (2025% of patients), initial complaints are as follows: Slurred speech, hoarseness, or decreased volume of speech; Aspiration or choking during a meal.
  • #11 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Amyotrophic lateral sclerosis (ALS) is the most common degenerative disease of the motor neuron system. Although ALS is incurable and fatal, with median survival of 3 years, treatment can extend the length and meaningful quality of life for patients. […] In 7580% of patients, symptoms begin with limb involvement. Initial complaints in patients with lower limb onset are often as follows: Tripping, stumbling, or awkwardness when running; Loss of lower extremity muscle bulk, strength, or endurance; Foot drop; patients may report a „slapping” gait. […] Initial complaints with upper limb onset include the following: Reduced finger dexterity, cramping, stiffness, and weakness or wasting of intrinsic hand muscles; Wrist drop interfering with work performance. […] With bulbar onset (2025% of patients), initial complaints are as follows: Slurred speech, hoarseness, or decreased volume of speech; Aspiration or choking during a meal.
  • #12 Amyotrophic Lateral Sclerosis: Lou Gehrig’s Disease | AAFP
    https://www.aafp.org/pubs/afp/issues/1999/0315/p1489.html
    Patients with lower limb onset may complain of tripping, stumbling or awkwardness when walking or running. Those with upper limb onset may first notice difficulty in actions such as buttoning clothes, picking up small objects or turning keys. […] As symptoms progressively worsen and spread, muscle atrophy becomes apparent and upper motor neuron symptoms such as spasticity complicate gait (in lower limb involvement) and manual dexterity (in upper limb involvement). […] Patients with the bulbar form of ALS develop problems in swallowing that are worse for saliva and liquids than for solids. […] The patients progress to a state of profound disability, and many eventually require ventilatory assistance and interventions such as gastrostomy for nutrition. […] In spite of the widespread devastating effects of ALS, certain functions are conspicuously unaffected; extraocular muscle movement, bladder and bowel control, sensory function and skin integrity are usually preserved.
  • #13 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), known as ALS, is a nervous system disease that affects nerve cells in the brain and spinal cord. ALS causes loss of muscle control. The disease gets worse over time. […] ALS often begins with muscle twitching and weakness in an arm or leg, trouble swallowing or slurred speech. Eventually ALS affects control of the muscles needed to move, speak, eat and breathe. There is no cure for this fatal disease. […] Symptoms of ALS vary from person to person. Symptoms depend on which nerve cells are affected. ALS generally begins with muscle weakness that spreads and gets worse over time. Symptoms might include: Trouble walking or doing usual daily activities. Tripping and falling. Weakness in the legs, feet or ankles. Hand weakness or clumsiness. Slurred speech or trouble swallowing. Weakness associated with muscle cramps and twitching in the arms, shoulders and tongue. Untimely crying, laughing or yawning. Thinking or behavioral changes.
  • #14 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Amyotrophic lateral sclerosis (ALS) is the most common degenerative disease of the motor neuron system. Although ALS is incurable and fatal, with median survival of 3 years, treatment can extend the length and meaningful quality of life for patients. […] In 7580% of patients, symptoms begin with limb involvement. Initial complaints in patients with lower limb onset are often as follows: Tripping, stumbling, or awkwardness when running; Loss of lower extremity muscle bulk, strength, or endurance; Foot drop; patients may report a „slapping” gait. […] Initial complaints with upper limb onset include the following: Reduced finger dexterity, cramping, stiffness, and weakness or wasting of intrinsic hand muscles; Wrist drop interfering with work performance. […] With bulbar onset (2025% of patients), initial complaints are as follows: Slurred speech, hoarseness, or decreased volume of speech; Aspiration or choking during a meal.
  • #15 ALS (Lou Gehrig’s Disease): Causes, Symptoms & Treatment
    https://www.healthline.com/health/amyotrophic-lateral-sclerosis
    Bulbar onset ALS is less common. In this type, ALS first affects a part of the brainstem known as the corticobulbar area. Symptoms include difficulty with speech and swallowing and muscle spasms in the face or throat. […] The earliest symptoms of ALS may include small muscle twitches in your mouth, throat, face, limbs. […] Other early signs of ALS may include difficulty performing some everyday tasks. This could mean difficulty climbing stairs or getting up from a chair. Its also possible to have difficulty speaking or swallowing, or weakness in the arms, hands, or legs. You may also notice cramping. […] As the condition progresses, the symptoms generally spread to both sides of the body. Muscle weakness, weight loss, and muscle atrophy are common. In the late stages of ALS, paralysis of the muscles occurs. Paralysis means the complete loss of voluntary movements.
  • #16 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Amyotrophic lateral sclerosis (ALS) is the most common degenerative disease of the motor neuron system. Although ALS is incurable and fatal, with median survival of 3 years, treatment can extend the length and meaningful quality of life for patients. […] In 7580% of patients, symptoms begin with limb involvement. Initial complaints in patients with lower limb onset are often as follows: Tripping, stumbling, or awkwardness when running; Loss of lower extremity muscle bulk, strength, or endurance; Foot drop; patients may report a „slapping” gait. […] Initial complaints with upper limb onset include the following: Reduced finger dexterity, cramping, stiffness, and weakness or wasting of intrinsic hand muscles; Wrist drop interfering with work performance. […] With bulbar onset (2025% of patients), initial complaints are as follows: Slurred speech, hoarseness, or decreased volume of speech; Aspiration or choking during a meal.
  • #17 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    The other third of patients have bulbar-onset ALS, and their first symptoms result from weakness in the bulbar muscles — that is, the muscles around the mouth and throat. This can lead to problems with articulation and slurring speech, an unusually hoarse or quiet voice, and difficulty swallowing or chewing. […] The majority of patients retain a fair amount of functionality and independence during the early stage of ALS, which typically lasts about a year. […] As ALS progresses into the middle stage, muscle weakness becomes more severe and spreads, affecting more regions of the body. In this stage of disease, some muscles become completely paralyzed and others are weakened, though some muscles may remain unaffected. […] Patients in the middle stage of ALS usually retain the ability to move some parts of their body under their own power, but ALS-related changes will begin to hamper mobility in this stage.
  • #18 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    The other third of patients have bulbar-onset ALS, and their first symptoms result from weakness in the bulbar muscles — that is, the muscles around the mouth and throat. This can lead to problems with articulation and slurring speech, an unusually hoarse or quiet voice, and difficulty swallowing or chewing. […] The majority of patients retain a fair amount of functionality and independence during the early stage of ALS, which typically lasts about a year. […] As ALS progresses into the middle stage, muscle weakness becomes more severe and spreads, affecting more regions of the body. In this stage of disease, some muscles become completely paralyzed and others are weakened, though some muscles may remain unaffected. […] Patients in the middle stage of ALS usually retain the ability to move some parts of their body under their own power, but ALS-related changes will begin to hamper mobility in this stage.
  • #19 ALS (Amyotrophic Lateral Sclerosis) and Frontotemporal Degeneration | AFTD
    https://www.theaftd.org/what-is-ftd/ftd-and-als-ftd-als/
    In persons with FTD-ALS, symptoms of one of the FTD disorders (usually behavioral variant FTD) are often first to appear, and can include changes in behavior, personality, and language. Motor symptoms that are more typically associated with ALS – including difficulty walking, swallowing, and/or using one’s hands – develop later. The former symptoms are caused by the degeneration of the brain’s frontal and temporal lobes, while the latter are a result of the deterioration of the nerves in the spinal cord that communicate with muscles in the rest of the body. […] Other motor symptoms associated with FTD-ALS are: Weakness in legs leading to frequent trips, stumbles, and falls; Difficulty lifting arms above the head; Clumsiness with hands or fingers that cause difficulties with fine motor skills (tying shoes, grasping a cup or pen, etc.); Changes in walking speed or stride; Drooling, difficulty swallowing, or slurred speech; Difficulty breathing, including choking, gagging, and coughing; Impaired swallowing.
  • #20 Amyotrophic Lateral Sclerosis (ALS): What It Is & Symptoms
    https://my.clevelandclinic.org/health/diseases/16729-amyotrophic-lateral-sclerosis-als
    ALS (amyotrophic lateral sclerosis) is a neurodegenerative condition that affects how nerve cells communicate with your muscles. It leads to muscle weakness that gets worse over time. Symptoms can affect how you move, speak and breathe. […] Symptoms worsen over time. […] You might notice muscle weakness and muscle twitching that affect your ability to walk independently, reach for objects, chew food and talk. ALS eventually causes your muscles to waste away (atrophy). Atrophy can make it harder to breathe and may lead to life-threatening outcomes. […] Common symptoms of ALS include muscle weakness, tremors and speech challenges. ALS causes symptoms that progressively get worse over time. […] Amyotrophic lateral sclerosis symptoms include: Muscle weakness (arms, legs and neck), Muscle cramps, Twitching in your hands, feet, shoulders and/or tongue, Stiff muscles (spasticity), Speech challenges (slurring words, trouble forming words), Drooling, Unintentional emotional expressions (like laughing or crying), Fatigue, Trouble swallowing (dysphagia).
  • #21 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    Amyotrophic lateral sclerosis (ALS), formerly known as Lou Gehrig’s disease, is a neurological disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing. As motor neurons degenerate and die, they stop sending messages to the muscles, which causes the muscles to weaken, start to twitch (fasciculations), and waste away (atrophy). Eventually, in people with ALS, the brain loses its ability to initiate and control voluntary movements such as walking, talking, chewing and other functions, as well as breathing. ALS is progressive, meaning the symptoms get worse over time. […] Early symptoms include: Muscle twitches in the arm, leg, shoulder, or tongue, Muscle cramps, Tight and stiff muscles (spasticity), Muscle weakness affecting an arm, a leg, or the neck, Slurred and nasal speech, Difficulty chewing or swallowing.
  • #22 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    Amyotrophic lateral sclerosis (ALS), formerly known as Lou Gehrig’s disease, is a neurological disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing. As motor neurons degenerate and die, they stop sending messages to the muscles, which causes the muscles to weaken, start to twitch (fasciculations), and waste away (atrophy). Eventually, in people with ALS, the brain loses its ability to initiate and control voluntary movements such as walking, talking, chewing and other functions, as well as breathing. ALS is progressive, meaning the symptoms get worse over time. […] Early symptoms include: Muscle twitches in the arm, leg, shoulder, or tongue, Muscle cramps, Tight and stiff muscles (spasticity), Muscle weakness affecting an arm, a leg, or the neck, Slurred and nasal speech, Difficulty chewing or swallowing.
  • #23 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    Amyotrophic lateral sclerosis (ALS), formerly known as Lou Gehrig’s disease, is a neurological disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing. As motor neurons degenerate and die, they stop sending messages to the muscles, which causes the muscles to weaken, start to twitch (fasciculations), and waste away (atrophy). Eventually, in people with ALS, the brain loses its ability to initiate and control voluntary movements such as walking, talking, chewing and other functions, as well as breathing. ALS is progressive, meaning the symptoms get worse over time. […] Early symptoms include: Muscle twitches in the arm, leg, shoulder, or tongue, Muscle cramps, Tight and stiff muscles (spasticity), Muscle weakness affecting an arm, a leg, or the neck, Slurred and nasal speech, Difficulty chewing or swallowing.
  • #24 Amyotrophic Lateral Sclerosis (ALS)
    https://www.webmd.com/brain/understanding-als-basics
    ALS Symptoms […] Amyotrophic lateral sclerosis affects everyone differently, but it causes a gradual loss of control in your voluntary muscles — the ones you use to walk, talk, move, speak, eat, drink, and breathe. […] Early symptoms of ALS […] Early symptoms of amyotrophic lateral sclerosis may include: […] Muscle twitching in the arm, leg, shoulder, or tongue […] Muscle cramps, especially in your hands and feet […] Muscle tightness or stiffness […] Weakness in your hands, arm, leg, or only one side of the body […] Trouble speaking loudly […] Slurred speech […] Problems chewing or swallowing. […] Symptoms of ALS progression […] Later on, amyotrophic lateral sclerosis can cause you to: […] Trip or fall more often […] Drop things a lot […] Lose weight […] Drool or choke on liquid […] Feel tired all the time […] Have trouble controlling facial expressions […] Laugh or cry without meaning to.
  • #25 Amyotrophic Lateral Sclerosis (ALS)
    https://www.webmd.com/brain/understanding-als-basics
    ALS Symptoms […] Amyotrophic lateral sclerosis affects everyone differently, but it causes a gradual loss of control in your voluntary muscles — the ones you use to walk, talk, move, speak, eat, drink, and breathe. […] Early symptoms of ALS […] Early symptoms of amyotrophic lateral sclerosis may include: […] Muscle twitching in the arm, leg, shoulder, or tongue […] Muscle cramps, especially in your hands and feet […] Muscle tightness or stiffness […] Weakness in your hands, arm, leg, or only one side of the body […] Trouble speaking loudly […] Slurred speech […] Problems chewing or swallowing. […] Symptoms of ALS progression […] Later on, amyotrophic lateral sclerosis can cause you to: […] Trip or fall more often […] Drop things a lot […] Lose weight […] Drool or choke on liquid […] Feel tired all the time […] Have trouble controlling facial expressions […] Laugh or cry without meaning to.
  • #26 Amyotrophic Lateral Sclerosis (ALS) > Fact Sheets > Yale Medicine
    https://www.yalemedicine.org/conditions/amyotrophic-lateral-sclerosis
    Symptoms include progressive muscle weakness; speech and/or swallowing difficulties; muscle twitching, cramping, and/or stiffness […] Symptoms of ALS include: Muscle weakness in a foot, arm, and/or leg. Muscle wasting. Muscle twitching. Muscle tightness and stiffness (spasticity). Muscle cramps. Weight loss. Difficulty swallowing or chewing. Slurred or nasal speech. Difficulty breathing. Falling episodes and excessive tripping. Difficulty with fine movements, such as shirt buttoning. Excessive drooling. Mood changes with uncontrolled, unexpected crying or laughing spells. Difficulty holding head up or head drop. […] Over time, as the disease progresses and muscle weakness becomes more pronounced, people with ALS have difficulty performing daily activities, such as bathing, eating, using the bathroom, and talking. Eventually, people with ALS become unable to walk, stand, move their limbs, use their hands, and/or breathe without the support of mechanical ventilation.
  • #27 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #28 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #29 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    The other third of patients have bulbar-onset ALS, and their first symptoms result from weakness in the bulbar muscles — that is, the muscles around the mouth and throat. This can lead to problems with articulation and slurring speech, an unusually hoarse or quiet voice, and difficulty swallowing or chewing. […] The majority of patients retain a fair amount of functionality and independence during the early stage of ALS, which typically lasts about a year. […] As ALS progresses into the middle stage, muscle weakness becomes more severe and spreads, affecting more regions of the body. In this stage of disease, some muscles become completely paralyzed and others are weakened, though some muscles may remain unaffected. […] Patients in the middle stage of ALS usually retain the ability to move some parts of their body under their own power, but ALS-related changes will begin to hamper mobility in this stage.
  • #30 Amyotrophic Lateral Sclerosis (ALS) | University Hospitals
    https://www.uhhospitals.org/health-information/health-and-wellness-library/article/diseases-and-conditions/amyotrophic-lateral-sclerosis-als
    For most people, the disease will progress over 3 to 5 years, making voluntary movements of arms and legs impossible. In other people, the disease can rapidly get worse over a few months or have a very slow course over many years. Each person is affected differently. […] Over time, ALS will lead to disability and death. Your ability to move and breathe independently will be affected, but your intelligence and ability to think is usually not. […] ALS affects voluntary control of arms and legs and leads to trouble breathing.
  • #31 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #32 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    ALS often starts in the hands, feet, arms or legs. Then it spreads to other parts of the body. Muscles get weaker as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing. […] There’s generally no pain in the early stages of ALS. Pain also is not common in the later stages. ALS doesn’t usually affect bladder control. It also usually doesn’t affect the senses, including the ability to taste, smell, touch and hear. […] As the disease progresses, ALS causes complications, such as: Breathing problems. Over time, ALS leads to weakness of the muscles used to breathe. […] Most people with ALS develop weakness of the muscles used to form speech. This usually starts with slower speech and occasional slurring of words. It then becomes harder to speak clearly. This can progress to the point that others can’t understand the person’s speech. […] People with ALS can develop weakness of the muscles involved with swallowing. This can lead to malnutrition and dehydration. […] Some people with ALS have problems with language and decision-making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.
  • #33 Simply Stated: Updates in Amyotrophic Lateral Sclerosis (ALS) – Quest | Muscular Dystrophy Association
    https://mdaquest.org/simply-stated-updates-in-amyotrophic-lateral-sclerosis-als/
    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that causes muscle weakness, disability, and eventually death, often within three to five years from when the symptoms first appear. In ALS, motor neurons (nerve cells that control muscle cells) are gradually lost. As a result, the muscles they control become weak and then nonfunctional, leading to ALS symptoms. […] The onset of ALS can happen at any age, though most affected people begin experiencing symptoms between the ages of 40-70 years old. ALS typically affects the upper motor neurons, which are in the brain, and the lower motor neurons, which are in the brainstem and spinal cord. Motor neurons are the large nerve cells that control voluntary movement. Degeneration of the upper motor neurons can cause muscle stiffness (spasticity), slowness of movement (bradykinesia), incoordination, and poor balance when walking or standing (postural instability). Degeneration of the lower motor neurons can cause muscle weakness, shrinkage (atrophy), twitching (fasciculations), and sometimes cramps. Generally, upper and lower motor neurons are affected at the same time in ALS; however, the body site where symptoms begin, the pattern and speed of disease spread, and the degree of upper and/or lower motor neuron dysfunction can be extremely variable between different people with ALS.
  • #34 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #35 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #36 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    ALS often starts in the hands, feet, arms or legs. Then it spreads to other parts of the body. Muscles get weaker as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing. […] There’s generally no pain in the early stages of ALS. Pain also is not common in the later stages. ALS doesn’t usually affect bladder control. It also usually doesn’t affect the senses, including the ability to taste, smell, touch and hear. […] As the disease progresses, ALS causes complications, such as: Breathing problems. Over time, ALS leads to weakness of the muscles used to breathe. […] Most people with ALS develop weakness of the muscles used to form speech. This usually starts with slower speech and occasional slurring of words. It then becomes harder to speak clearly. This can progress to the point that others can’t understand the person’s speech. […] People with ALS can develop weakness of the muscles involved with swallowing. This can lead to malnutrition and dehydration. […] Some people with ALS have problems with language and decision-making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.
  • #37 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Each occurrence of ALS is unique, and there is no clear-cut time frame for how an individual’s disease will progress. […] Symptoms may appear gradually over time, or they may occur rapidly and then plateau. […] Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). […] Symptoms may be limited to a single body region or mild symptoms may affect more than one region. […] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. […] Symptoms become more widespread. […] Some muscles are paralyzed while others are weakened or unaffected. Fasciculations may continue. […] Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva.
  • #38 The 7 Stages of ALS: The Progression of a Nonlinear Disease
    https://www.targetals.org/2022/01/04/the-stages-of-als-framing-the-progression-of-a-nonlinear-disease/
    Swallowing and chewing may become increasingly difficult over time, and the risk of choking increases. Breathing also becomes harder. […] In advanced ALS, most voluntary muscles are paralyzed, including those of the mouth and throat. So are those involved in breathing. […] The final stage of ALS varies quite a bit from person to person. During this phase, patients typically experience significant muscle weakness, respiratory challenges, and require extensive support for daily activities. […] The duration of this last stage can range anywhere from several months to a few years.
  • #39 Amyotrophic Lateral Sclerosis Clinical Presentation: History, Physical Examination, Diagnostic Criteria
    https://emedicine.medscape.com/article/1170097-clinical
    A mixture of spastic and flaccid components may characterize speech, resulting in a dysarthria with severe disintegration and slowness of articulation. Hypernasality occurs from palatal weakness, and patients may develop a strained, strangled vocal quality. With time, speech may be lost, and patients may become dependent on other forms of communication, such as writing, communication boards, or speech-generation devices. […] Patients with bulbar involvement may develop swallowing difficulties (dysphagia). Swallowing liquids requires the greatest oropharyngeal muscle control; therefore, patients usually report more difficulty with liquids than with solids. Aspiration or choking during a meal may occur. […] Drooling affects some patients and results from a combination of excessive salivation, poor swallowing, and poor lip control. The problem can usually be ameliorated effectively with oral medications or a scopolamine patch, but other methods, such as salivary gland irradiation or injection of botulinum toxin, are sometimes required. […] While the symptoms of motor dysfunction in ALS are best recognized, affecting all patients with the disease, a fair proportion of patients also experience emotional and special cognitive difficulties. Emotional manifestations of ALS include involuntary laughing or crying and/or depression. Cognitive difficulties involve executive function impairment and/or behavioral changes.
  • #40 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #41 ALS Progression Timeline: What to Expect | ALS United Greater Chicago
    https://alsunitedchicago.org/als-progression-timeline/
    ALS is a progressive neurodegenerative disorder affecting motor neurons, leading to muscle weakness and loss of voluntary movement. […] Early signs of ALS often manifest subtly, with muscle twitches in the arm, leg, shoulder, or tongue being a common initial symptom. Individuals may experience muscle cramps, stiffness, or spasticity, accompanied by localized weakness affecting an arm, leg, or neck. […] As the disease progresses, muscle weakness and atrophy spread, leading to more pronounced symptoms. These may include fasciculations (muscle twitches), loss of muscle bulk, fatigue, poor balance, and a weak grip. […] The early stage of ALS is characterized by subtle, localized symptoms that may initially go unnoticed, with muscle weakness typically manifesting in one region of the body.
  • #42 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    The other third of patients have bulbar-onset ALS, and their first symptoms result from weakness in the bulbar muscles — that is, the muscles around the mouth and throat. This can lead to problems with articulation and slurring speech, an unusually hoarse or quiet voice, and difficulty swallowing or chewing. […] The majority of patients retain a fair amount of functionality and independence during the early stage of ALS, which typically lasts about a year. […] As ALS progresses into the middle stage, muscle weakness becomes more severe and spreads, affecting more regions of the body. In this stage of disease, some muscles become completely paralyzed and others are weakened, though some muscles may remain unaffected. […] Patients in the middle stage of ALS usually retain the ability to move some parts of their body under their own power, but ALS-related changes will begin to hamper mobility in this stage.
  • #43 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    The other third of patients have bulbar-onset ALS, and their first symptoms result from weakness in the bulbar muscles — that is, the muscles around the mouth and throat. This can lead to problems with articulation and slurring speech, an unusually hoarse or quiet voice, and difficulty swallowing or chewing. […] The majority of patients retain a fair amount of functionality and independence during the early stage of ALS, which typically lasts about a year. […] As ALS progresses into the middle stage, muscle weakness becomes more severe and spreads, affecting more regions of the body. In this stage of disease, some muscles become completely paralyzed and others are weakened, though some muscles may remain unaffected. […] Patients in the middle stage of ALS usually retain the ability to move some parts of their body under their own power, but ALS-related changes will begin to hamper mobility in this stage.
  • #44 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    By the late stages of ALS, most muscles involved in voluntary movement become paralyzed. Patients generally have very limited or no ability to move under their own power, and will require assistance with most day-to-day activities. […] Most individuals in late-stage ALS will require ventilatory support when breathing function declines significantly. […] ALS is a fatal disease, and most people with the disorder will die within about two to five years after symptom onset. […] The speed at which amyotrophic lateral sclerosis (ALS) progresses can vary a lot from person to person, but typically the disease progresses quite rapidly. Most patients reach the late stage of ALS within two to three years after symptom onset, and the average survival time is about two to five years from onset. […] Although there is no cure for amyotrophic lateral sclerosis (ALS), there are several approved therapies that can slow disease progression, helping patients to maintain functionality for longer and prolonging survival.
  • #45 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #46 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #47 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    By the late stages of ALS, most muscles involved in voluntary movement become paralyzed. Patients generally have very limited or no ability to move under their own power, and will require assistance with most day-to-day activities. […] Most individuals in late-stage ALS will require ventilatory support when breathing function declines significantly. […] ALS is a fatal disease, and most people with the disorder will die within about two to five years after symptom onset. […] The speed at which amyotrophic lateral sclerosis (ALS) progresses can vary a lot from person to person, but typically the disease progresses quite rapidly. Most patients reach the late stage of ALS within two to three years after symptom onset, and the average survival time is about two to five years from onset. […] Although there is no cure for amyotrophic lateral sclerosis (ALS), there are several approved therapies that can slow disease progression, helping patients to maintain functionality for longer and prolonging survival.
  • #48 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #49 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #50 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #51 Stages of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als
    Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. […] Most voluntary muscles are paralyzed. […] The ability to move air in and out of the lungs is severely compromised. […] Mobility is extremely limited; needs must be attended to by a caregiver. […] Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. […] Speech may be no longer possible. […] Eating and drinking by mouth aren’t possible.
  • #52 The 7 Stages of ALS: How They Could Be Broken Down | ALS United Ohio
    https://alsohio.org/the-7-stages-of-als-how-they-could-be-broken-down/
    As the disease advances to the middle stages, patients often require assistive devices like walkers or wheelchairs, and driving is typically discontinued. […] Stage 5 of ALS marks a period of rapid decline in physical function, characterized by widespread paralysis of voluntary muscles. […] At this stage, mobility becomes extremely limited, with patients requiring round-the-clock care for most daily activities. […] Stage 6 of ALS represents a critical phase characterized by extensive paralysis and the necessity for continuous respiratory support. […] End-stage ALS is characterized by near-total paralysis of voluntary muscles, rendering patients completely dependent on caregivers for all aspects of daily living. […] Patients typically lose the ability to speak, chew, and drink, relying entirely on feeding tubes for nutrition. […] The risk of secondary complications, such as pneumonia and pressure sores, increases significantly due to immobility and compromised immune function. […] Comprehensive palliative care and support for both patients and caregivers are crucial in the final stage of ALS.
  • #53 The 7 Stages of ALS: The Progression of a Nonlinear Disease
    https://www.targetals.org/2022/01/04/the-stages-of-als-framing-the-progression-of-a-nonlinear-disease/
    Swallowing and chewing may become increasingly difficult over time, and the risk of choking increases. Breathing also becomes harder. […] In advanced ALS, most voluntary muscles are paralyzed, including those of the mouth and throat. So are those involved in breathing. […] The final stage of ALS varies quite a bit from person to person. During this phase, patients typically experience significant muscle weakness, respiratory challenges, and require extensive support for daily activities. […] The duration of this last stage can range anywhere from several months to a few years.
  • #54 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    ALS often starts in the hands, feet, arms or legs. Then it spreads to other parts of the body. Muscles get weaker as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing. […] There’s generally no pain in the early stages of ALS. Pain also is not common in the later stages. ALS doesn’t usually affect bladder control. It also usually doesn’t affect the senses, including the ability to taste, smell, touch and hear. […] As the disease progresses, ALS causes complications, such as: Breathing problems. Over time, ALS leads to weakness of the muscles used to breathe. […] Most people with ALS develop weakness of the muscles used to form speech. This usually starts with slower speech and occasional slurring of words. It then becomes harder to speak clearly. This can progress to the point that others can’t understand the person’s speech. […] People with ALS can develop weakness of the muscles involved with swallowing. This can lead to malnutrition and dehydration. […] Some people with ALS have problems with language and decision-making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.
  • #55 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    A treatment plan for ALS usually includes rehabilitation, which should be tailored to the persons individual needs and may include physical, occupational, and speech therapy. […] As the muscles responsible for breathing start to weaken, individuals with ALS may have shortness of breath during physical activity and difficulty breathing at night or when lying down. Noninvasive ventilation (NIV) is a type of breathing support that is usually delivered through a mask over the nose and/or mouth. It may help decrease the discomfort of breathing in some individuals with ALS. Initially, NIV may only be necessary at night, but may eventually be used full time. As the disease progresses, the person may need the support of respirators (mechanical ventilators) to inflate and deflate the lungs. […] Because the muscles that control breathing become weak, people with ALS also may have trouble generating a strong cough.
  • #56 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    A treatment plan for ALS usually includes rehabilitation, which should be tailored to the persons individual needs and may include physical, occupational, and speech therapy. […] As the muscles responsible for breathing start to weaken, individuals with ALS may have shortness of breath during physical activity and difficulty breathing at night or when lying down. Noninvasive ventilation (NIV) is a type of breathing support that is usually delivered through a mask over the nose and/or mouth. It may help decrease the discomfort of breathing in some individuals with ALS. Initially, NIV may only be necessary at night, but may eventually be used full time. As the disease progresses, the person may need the support of respirators (mechanical ventilators) to inflate and deflate the lungs. […] Because the muscles that control breathing become weak, people with ALS also may have trouble generating a strong cough.
  • #57 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    A treatment plan for ALS usually includes rehabilitation, which should be tailored to the persons individual needs and may include physical, occupational, and speech therapy. […] As the muscles responsible for breathing start to weaken, individuals with ALS may have shortness of breath during physical activity and difficulty breathing at night or when lying down. Noninvasive ventilation (NIV) is a type of breathing support that is usually delivered through a mask over the nose and/or mouth. It may help decrease the discomfort of breathing in some individuals with ALS. Initially, NIV may only be necessary at night, but may eventually be used full time. As the disease progresses, the person may need the support of respirators (mechanical ventilators) to inflate and deflate the lungs. […] Because the muscles that control breathing become weak, people with ALS also may have trouble generating a strong cough.
  • #58 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    A treatment plan for ALS usually includes rehabilitation, which should be tailored to the persons individual needs and may include physical, occupational, and speech therapy. […] As the muscles responsible for breathing start to weaken, individuals with ALS may have shortness of breath during physical activity and difficulty breathing at night or when lying down. Noninvasive ventilation (NIV) is a type of breathing support that is usually delivered through a mask over the nose and/or mouth. It may help decrease the discomfort of breathing in some individuals with ALS. Initially, NIV may only be necessary at night, but may eventually be used full time. As the disease progresses, the person may need the support of respirators (mechanical ventilators) to inflate and deflate the lungs. […] Because the muscles that control breathing become weak, people with ALS also may have trouble generating a strong cough.
  • #59 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    As the disease progresses, muscle weakness and atrophy spread to other parts of your body. People with ALS may develop problems with: Chewing food and swallowing (dysphagia), Drooling (sialorrhea), Speaking or forming words (dysarthria), Breathing (dyspnea), Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms), Constipation, Maintaining weight and getting enough nutrients. […] Eventually, people with ALS will not be able to stand or walk, get in or out of bed on their own, use their hands and arms, or breathe on their own. […] Most people with ALS die from being unable to breathe on their own (known as respiratory failure,) usually within three to five years from when the symptoms first appear. However, about 10% survive for a decade or more. […] There is no treatment to reverse damage to motor neurons or cure ALS at this time. However, some treatments may slow progression of the disease, improve quality of life, and extend survival.
  • #60 Amyotrophic Lateral Sclerosis Clinical Presentation: History, Physical Examination, Diagnostic Criteria
    https://emedicine.medscape.com/article/1170097-clinical
    A mixture of spastic and flaccid components may characterize speech, resulting in a dysarthria with severe disintegration and slowness of articulation. Hypernasality occurs from palatal weakness, and patients may develop a strained, strangled vocal quality. With time, speech may be lost, and patients may become dependent on other forms of communication, such as writing, communication boards, or speech-generation devices. […] Patients with bulbar involvement may develop swallowing difficulties (dysphagia). Swallowing liquids requires the greatest oropharyngeal muscle control; therefore, patients usually report more difficulty with liquids than with solids. Aspiration or choking during a meal may occur. […] Drooling affects some patients and results from a combination of excessive salivation, poor swallowing, and poor lip control. The problem can usually be ameliorated effectively with oral medications or a scopolamine patch, but other methods, such as salivary gland irradiation or injection of botulinum toxin, are sometimes required. […] While the symptoms of motor dysfunction in ALS are best recognized, affecting all patients with the disease, a fair proportion of patients also experience emotional and special cognitive difficulties. Emotional manifestations of ALS include involuntary laughing or crying and/or depression. Cognitive difficulties involve executive function impairment and/or behavioral changes.
  • #61 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Emotional difficulties in some ALS patients are as follows: Involuntary laughing or crying; Depression; Impaired executive function and other cognitive changes; Behavioral changes. […] Some patients will show cognitive and behavioral changes due to involvement of the prefrontal cortex. In their extreme form, these changes will meet criteria for behavioral-variant frontotemporal dementia (bvFTD). This occurs in 16% of patients with ALS; 33% will have either behavioral or cognitive changes not meeting the threshold to be classified as bvFTD. […] Features of more-advanced disease are as follows: Muscle atrophy becomes more apparent; Spasticity may compromise gait and manual dexterity; Muscle cramps are common; Rarely, painful joint contractures may result from immobility. […] Progression of bulbar disease leads to the following: Voice changes: Hypernasality and development of a strained, strangled vocal quality; eventually, speech may be lost; Swallowing difficulties, usually starting with liquids; Drooling.
  • #62 The 7 Stages of ALS: The Progression of a Nonlinear Disease
    https://www.targetals.org/2022/01/04/the-stages-of-als-framing-the-progression-of-a-nonlinear-disease/
    Swallowing and chewing may become increasingly difficult over time, and the risk of choking increases. Breathing also becomes harder. […] In advanced ALS, most voluntary muscles are paralyzed, including those of the mouth and throat. So are those involved in breathing. […] The final stage of ALS varies quite a bit from person to person. During this phase, patients typically experience significant muscle weakness, respiratory challenges, and require extensive support for daily activities. […] The duration of this last stage can range anywhere from several months to a few years.
  • #63 Amyotrophic Lateral Sclerosis Clinical Presentation: History, Physical Examination, Diagnostic Criteria
    https://emedicine.medscape.com/article/1170097-clinical
    A mixture of spastic and flaccid components may characterize speech, resulting in a dysarthria with severe disintegration and slowness of articulation. Hypernasality occurs from palatal weakness, and patients may develop a strained, strangled vocal quality. With time, speech may be lost, and patients may become dependent on other forms of communication, such as writing, communication boards, or speech-generation devices. […] Patients with bulbar involvement may develop swallowing difficulties (dysphagia). Swallowing liquids requires the greatest oropharyngeal muscle control; therefore, patients usually report more difficulty with liquids than with solids. Aspiration or choking during a meal may occur. […] Drooling affects some patients and results from a combination of excessive salivation, poor swallowing, and poor lip control. The problem can usually be ameliorated effectively with oral medications or a scopolamine patch, but other methods, such as salivary gland irradiation or injection of botulinum toxin, are sometimes required. […] While the symptoms of motor dysfunction in ALS are best recognized, affecting all patients with the disease, a fair proportion of patients also experience emotional and special cognitive difficulties. Emotional manifestations of ALS include involuntary laughing or crying and/or depression. Cognitive difficulties involve executive function impairment and/or behavioral changes.
  • #64 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    ALS often starts in the hands, feet, arms or legs. Then it spreads to other parts of the body. Muscles get weaker as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing. […] There’s generally no pain in the early stages of ALS. Pain also is not common in the later stages. ALS doesn’t usually affect bladder control. It also usually doesn’t affect the senses, including the ability to taste, smell, touch and hear. […] As the disease progresses, ALS causes complications, such as: Breathing problems. Over time, ALS leads to weakness of the muscles used to breathe. […] Most people with ALS develop weakness of the muscles used to form speech. This usually starts with slower speech and occasional slurring of words. It then becomes harder to speak clearly. This can progress to the point that others can’t understand the person’s speech. […] People with ALS can develop weakness of the muscles involved with swallowing. This can lead to malnutrition and dehydration. […] Some people with ALS have problems with language and decision-making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.
  • #65 Simply Stated: Updates in Amyotrophic Lateral Sclerosis (ALS) – Quest | Muscular Dystrophy Association
    https://mdaquest.org/simply-stated-updates-in-amyotrophic-lateral-sclerosis-als/
    A person with ALS may experience impairments of arm/leg (limb), face/head/neck (bulbar), trunk (axial), and breathing (respiratory) muscles. Most cases begin with persistent weakness or spasticity in an arm or leg. In some cases, the problem begins in the muscles controlling speech or swallowing. […] Problems with mental processes (cognitive impairment) occur in some people with ALS and may come before or after the onset of upper motor neuron and/or lower motor neuron symptoms. A condition known as frontotemporal dementia (FTD), which impacts a person’s behavior and ability to produce and understand speech, is associated with ALS in 15-50% of cases. ALS can also present with pseudobulbar affect (PBA), a condition in which the affected person experiences outbursts of uncontrolled laughing, crying, or yawning.
  • #66 Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
    ALS often starts in the hands, feet, arms or legs. Then it spreads to other parts of the body. Muscles get weaker as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing. […] There’s generally no pain in the early stages of ALS. Pain also is not common in the later stages. ALS doesn’t usually affect bladder control. It also usually doesn’t affect the senses, including the ability to taste, smell, touch and hear. […] As the disease progresses, ALS causes complications, such as: Breathing problems. Over time, ALS leads to weakness of the muscles used to breathe. […] Most people with ALS develop weakness of the muscles used to form speech. This usually starts with slower speech and occasional slurring of words. It then becomes harder to speak clearly. This can progress to the point that others can’t understand the person’s speech. […] People with ALS can develop weakness of the muscles involved with swallowing. This can lead to malnutrition and dehydration. […] Some people with ALS have problems with language and decision-making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.
  • #67 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Emotional difficulties in some ALS patients are as follows: Involuntary laughing or crying; Depression; Impaired executive function and other cognitive changes; Behavioral changes. […] Some patients will show cognitive and behavioral changes due to involvement of the prefrontal cortex. In their extreme form, these changes will meet criteria for behavioral-variant frontotemporal dementia (bvFTD). This occurs in 16% of patients with ALS; 33% will have either behavioral or cognitive changes not meeting the threshold to be classified as bvFTD. […] Features of more-advanced disease are as follows: Muscle atrophy becomes more apparent; Spasticity may compromise gait and manual dexterity; Muscle cramps are common; Rarely, painful joint contractures may result from immobility. […] Progression of bulbar disease leads to the following: Voice changes: Hypernasality and development of a strained, strangled vocal quality; eventually, speech may be lost; Swallowing difficulties, usually starting with liquids; Drooling.
  • #68 Amyotrophic lateral sclerosis: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/amyotrophic-lateral-sclerosis/
    Approximately 20 percent of individuals with ALS also develop FTD. Changes in personality and behavior may make it difficult for affected individuals to interact with others in a socially appropriate manner. Communication skills worsen as the disease progresses. It is unclear how the development of ALS and FTD are related. Individuals who develop both conditions are diagnosed as having ALS-FTD.
  • #69 Amyotrophic Lateral Sclerosis: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1170097-overview
    Emotional difficulties in some ALS patients are as follows: Involuntary laughing or crying; Depression; Impaired executive function and other cognitive changes; Behavioral changes. […] Some patients will show cognitive and behavioral changes due to involvement of the prefrontal cortex. In their extreme form, these changes will meet criteria for behavioral-variant frontotemporal dementia (bvFTD). This occurs in 16% of patients with ALS; 33% will have either behavioral or cognitive changes not meeting the threshold to be classified as bvFTD. […] Features of more-advanced disease are as follows: Muscle atrophy becomes more apparent; Spasticity may compromise gait and manual dexterity; Muscle cramps are common; Rarely, painful joint contractures may result from immobility. […] Progression of bulbar disease leads to the following: Voice changes: Hypernasality and development of a strained, strangled vocal quality; eventually, speech may be lost; Swallowing difficulties, usually starting with liquids; Drooling.
  • #70 Signs and Symptoms of Amyotrophic Lateral Sclerosis (ALS) – Diseases | Muscular Dystrophy Association
    https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms
    Another potential ALS symptom not experienced by all is a temporary lapse of control over emotional expressions such as laughing or crying, a phenomenon called pseudobulbar affect. Laughing or crying bouts, often triggered by the smallest of things, are more related to the disease process rather than to actual feelings of happiness or sadness.
  • #71 Amyotrophic Lateral Sclerosis (ALS) | PM&R KnowledgeNow
    https://now.aapmr.org/amyotrophic-lateral-sclerosis/
    Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrigs disease, is a rapidly progressive neurodegenerative disease affecting voluntary motor control. ALS classically is characterized by a combination of both upper and lower motor neuron degeneration. […] Survival is reported to vary from 20-48 months after symptom onset. However, there is wide variability with up to 10% of patients surviving longer than 10 years. […] There are many secondary conditions that result from ALS. […] Muscle weakness […] Dysphagia […] Dysarthria […] Sialorrhea […] Malnutrition […] Depression/adjustment disorder […] Cognitive impairment. Frontotemporal dementia is thought to be on the same spectrum of disease as ALS and is most associated with TDP-43 mutations […] Spasticity […] Muscle cramping
  • #72 Amyotrophic Lateral Sclerosis (ALS) | PM&R KnowledgeNow
    https://now.aapmr.org/amyotrophic-lateral-sclerosis/
    Weight loss in ALS is multifactorial with etiologies including progressive dysphagia, significant respiratory burden, hypermetabolism, depression, functional inability to eat, and gastroparesis causing early satiety. […] Early disease weight loss has recently been linked to increased mortality. […] Dysarthria in ALS may be spastic, flaccid or a combination of these types. […] Spasticity can be both painful and functionally limiting for ALS patients. […] Fatigue may be multifactorial in ALS patients. Possible etiologies include hypercarbia from restrictive lung disease, depression, poor caloric intake, muscle weakness, poor sleep hygiene, anemia, hypothyroidism.
  • #73 Amyotrophic Lateral Sclerosis (ALS) | PM&R KnowledgeNow
    https://now.aapmr.org/amyotrophic-lateral-sclerosis/
    Weight loss in ALS is multifactorial with etiologies including progressive dysphagia, significant respiratory burden, hypermetabolism, depression, functional inability to eat, and gastroparesis causing early satiety. […] Early disease weight loss has recently been linked to increased mortality. […] Dysarthria in ALS may be spastic, flaccid or a combination of these types. […] Spasticity can be both painful and functionally limiting for ALS patients. […] Fatigue may be multifactorial in ALS patients. Possible etiologies include hypercarbia from restrictive lung disease, depression, poor caloric intake, muscle weakness, poor sleep hygiene, anemia, hypothyroidism.
  • #74 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    As the disease progresses, muscle weakness and atrophy spread to other parts of your body. People with ALS may develop problems with: Chewing food and swallowing (dysphagia), Drooling (sialorrhea), Speaking or forming words (dysarthria), Breathing (dyspnea), Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms), Constipation, Maintaining weight and getting enough nutrients. […] Eventually, people with ALS will not be able to stand or walk, get in or out of bed on their own, use their hands and arms, or breathe on their own. […] Most people with ALS die from being unable to breathe on their own (known as respiratory failure,) usually within three to five years from when the symptoms first appear. However, about 10% survive for a decade or more. […] There is no treatment to reverse damage to motor neurons or cure ALS at this time. However, some treatments may slow progression of the disease, improve quality of life, and extend survival.
  • #75 Amyotrophic Lateral Sclerosis: Disease State Overview
    https://www.ajmc.com/view/amyotrophic-lateral-sclerosis-disease-state-overview
    Atypical presentation includes emotional lability, frontal lobe-type cognitive dysfunction, weight loss, and fasciculations and cramps without muscle weakness. […] Symptoms may be described as limb onset and bulbar onset. […] The presence of the Babinski sign, or upward response of the plantar reflex, is also evidence of UMN dysfunction and is discovered in 30% to 50% of patients. […] They usually retain higher mental functions, such as problem solving, reasoning, understanding, and remembering. […] Additionally, patients do not always progress on a linear path. […] Weeks to months may pass where there is little to no function loss. […] A rare patient will have considerable improvements, such as recovering lost function. […] These ALS reversals and arrests are regrettably fleeting with fewer than 1% of patients maintaining marked improvement for at least 12 months.
  • #76 Amyotrophic Lateral Sclerosis: Disease State Overview
    https://www.ajmc.com/view/amyotrophic-lateral-sclerosis-disease-state-overview
    Atypical presentation includes emotional lability, frontal lobe-type cognitive dysfunction, weight loss, and fasciculations and cramps without muscle weakness. […] Symptoms may be described as limb onset and bulbar onset. […] The presence of the Babinski sign, or upward response of the plantar reflex, is also evidence of UMN dysfunction and is discovered in 30% to 50% of patients. […] They usually retain higher mental functions, such as problem solving, reasoning, understanding, and remembering. […] Additionally, patients do not always progress on a linear path. […] Weeks to months may pass where there is little to no function loss. […] A rare patient will have considerable improvements, such as recovering lost function. […] These ALS reversals and arrests are regrettably fleeting with fewer than 1% of patients maintaining marked improvement for at least 12 months.
  • #77 Amyotrophic Lateral Sclerosis: Disease State Overview
    https://www.ajmc.com/view/amyotrophic-lateral-sclerosis-disease-state-overview
    Atypical presentation includes emotional lability, frontal lobe-type cognitive dysfunction, weight loss, and fasciculations and cramps without muscle weakness. […] Symptoms may be described as limb onset and bulbar onset. […] The presence of the Babinski sign, or upward response of the plantar reflex, is also evidence of UMN dysfunction and is discovered in 30% to 50% of patients. […] They usually retain higher mental functions, such as problem solving, reasoning, understanding, and remembering. […] Additionally, patients do not always progress on a linear path. […] Weeks to months may pass where there is little to no function loss. […] A rare patient will have considerable improvements, such as recovering lost function. […] These ALS reversals and arrests are regrettably fleeting with fewer than 1% of patients maintaining marked improvement for at least 12 months.
  • #78 Identifying patterns in amyotrophic lateral sclerosis progression from sparse longitudinal data | Nature Computational Science
    https://www.nature.com/articles/s43588-022-00299-w
    The clinical presentation of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease, varies widely across patients, making it challenging to determine if potential therapeutics slow progression. […] We show that ALS progression is frequently nonlinear, with periods of stable disease preceded or followed by rapid decline. […] The median length of survival from symptom onset is approximately three years; however, some individuals survive decades with the disease. […] While people with ALS invariably decline over time, some of the measures can increase for short durations or reach plateaus. […] Despite the widespread use of linear models in predicting patient progression, there is evidence that ALS progression can be nonlinear and can vary across disease severity. […] The heterogeneity of ALS makes it difficult to determine if a disease-modifying therapy is effectively slowing progression.
  • #79 Identifying patterns in amyotrophic lateral sclerosis progression from sparse longitudinal data | Nature Computational Science
    https://www.nature.com/articles/s43588-022-00299-w
    Our results provide an advance in modeling progression patterns in ALS and other diseases. […] The nonlinear progression patterns are robust to sparse data and consistent across study populations, and correspond to survival outcomes. […] The dominant clinical progression patterns in ALS are sigmoidal fast progression, stable slow progression, unstable slow progression and unstable medium progression. […] Overall, the MoGP model promotes the ability to learn these complex disease progression trajectories better than currently used clinical models, while stratifying patients to reveal common patterns of disease.
  • #80 ALS Progression Timeline: What to Expect | ALS United Greater Chicago
    https://alsunitedchicago.org/als-progression-timeline/
    As ALS progresses into the middle stage, muscle weakness becomes more severe and widespread, affecting multiple regions of the body. […] In the late stage of ALS, advanced care and support needs become paramount. […] ALS progression varies significantly among individuals, influenced by multiple factors. […] Several factors influence the rate of ALS progression, contributing to the variability observed among individuals. […] Personalized care plans are crucial in managing ALS progression effectively. […] A multidisciplinary care approach is essential for managing the complex needs of individuals with ALS. […] As ALS progresses, adaptive equipment and assistive technologies become crucial for maintaining independence and quality of life. […] Emotional and psychological support is crucial for both individuals with ALS and their caregivers.
  • #81 The 7 Stages of ALS: How They Could Be Broken Down | ALS United Ohio
    https://alsohio.org/the-7-stages-of-als-how-they-could-be-broken-down/
    ALS progression exhibits significant variability among individuals, challenging the notion of a uniform disease trajectory. […] Factors influencing the rate of progression include age at symptom onset, site of initial symptoms, and genetic factors. […] Younger individuals typically experience slower disease advancement, while certain genetic mutations are linked to distinct clinical courses. […] Stage 1 of ALS is characterized by subtle signs that may initially go unnoticed. […] In this early phase, symptoms are usually mild and confined to one area of the body. […] For approximately two-thirds of patients, the disease begins in the limbs, affecting muscles in the hands, feet, calves, or forearms. […] The remaining third experience bulbar-onset ALS, where initial symptoms stem from weakness in the muscles around the mouth and throat, leading to problems with speech articulation, voice changes, or difficulties in swallowing and chewing.
  • #82 Stages of ALS: What Can You Expect?
    https://www.healthline.com/health/stages-of-als
    The average life expectancy for ALS is 3 to 5 years. Bulbar-onset ALS is considered a more aggressive subtype and may fully progress in up to 2 years or less. […] However, some people live with ALS for over 10 years after diagnosis. […] Early symptoms of ALS tend to impact one area of the body and cause mild impairment. As ALS progresses, more regions are affected and voluntary muscle control may be entirely lost. […] ALS is a terminal illness, but life expectancy varies. Some people live more than a decade after diagnosis. Treatment can help manage symptoms and slow disease progression.
  • #83 The 7 Stages of ALS: How They Could Be Broken Down | ALS United Ohio
    https://alsohio.org/the-7-stages-of-als-how-they-could-be-broken-down/
    ALS progression exhibits significant variability among individuals, challenging the notion of a uniform disease trajectory. […] Factors influencing the rate of progression include age at symptom onset, site of initial symptoms, and genetic factors. […] Younger individuals typically experience slower disease advancement, while certain genetic mutations are linked to distinct clinical courses. […] Stage 1 of ALS is characterized by subtle signs that may initially go unnoticed. […] In this early phase, symptoms are usually mild and confined to one area of the body. […] For approximately two-thirds of patients, the disease begins in the limbs, affecting muscles in the hands, feet, calves, or forearms. […] The remaining third experience bulbar-onset ALS, where initial symptoms stem from weakness in the muscles around the mouth and throat, leading to problems with speech articulation, voice changes, or difficulties in swallowing and chewing.
  • #84 Amyotrophic lateral sclerosis – Knowledge @ AMBOSS
    https://www.amboss.com/us/knowledge/amyotrophic-lateral-sclerosis/
    Asymmetric limb weakness that often begins in the hands and feet but may affect any part of the body. […] Respiratory muscle weakness causing: Dyspnea with hypoxia and/or hypercarbia. […] Median survival is 35 years after symptom onset. […] Early bulbar and/or respiratory symptoms are associated with a worse prognosis.
  • #85 Amyotrophic lateral sclerosis – Knowledge @ AMBOSS
    https://www.amboss.com/us/knowledge/amyotrophic-lateral-sclerosis/
    Asymmetric limb weakness that often begins in the hands and feet but may affect any part of the body. […] Respiratory muscle weakness causing: Dyspnea with hypoxia and/or hypercarbia. […] Median survival is 35 years after symptom onset. […] Early bulbar and/or respiratory symptoms are associated with a worse prognosis.
  • #86 ALS – Wikipedia
    https://en.wikipedia.org/wiki/ALS
    The rate of progression can be measured using the ALS Functional Rating Scale – Revised (ALSFRS-R), a 12-item instrument survey administered as a clinical interview or self-reported questionnaire that produces a score between 48 (normal function) and 0 (severe disability). Though the degree of variability is high and a small percentage of people have a much slower progression, on average people with ALS lose about 1 ALSFRS-R point per month. […] Difficulties with chewing and swallowing make eating very difficult (dysphagia) and increase the risk of choking or of aspirating food into the lungs. In later stages of the disorder, aspiration pneumonia can develop, and maintaining a healthy weight can become a significant problem that may require the insertion of a feeding tube. As the diaphragm and intercostal muscles of the rib cage that support breathing weaken, measures of lung function such as vital capacity and inspiratory pressure diminish. Individuals affected by the disorder may ultimately lose the ability to initiate and control all voluntary movement, known as locked-in syndrome.
  • #87 Simply Stated: Updates in Amyotrophic Lateral Sclerosis (ALS) – Quest | Muscular Dystrophy Association
    https://mdaquest.org/simply-stated-updates-in-amyotrophic-lateral-sclerosis-als/
    As ALS progresses, affected people may experience a variety of additional symptoms, including constipation, dysphagia (difficulty swallowing), and incomplete eye closure and drooling due to weakened facial muscles. Some may have symptoms similar to Parkinson’s disease, such as lack of facial expressions (facial masking), tremor, bradykinesia, and postural instability. About 20-30% of people with ALS may have sensory impairments, such as reduced ability to feel pain or temperature or reduced awareness of body positioning (proprioception). In some people with ALS, pain is a side effect of muscle problems (cramps, spasticity, etc.). […] ALS is relentlessly progressive. Symptoms typically spread within the location of onset (e.g. limb, bulbar) and then to other body regions in a predictable pattern. Eventually, ALS progression leads to life-threatening respiratory failure and/or dysphagia. In most cases, this occurs within 3-5 years following symptom onset, however, about 10% of people diagnosed with ALS survive for a decade or more.
  • #88 Stages of ALS: Progression Before and After Diagnosis
    https://www.verywellhealth.com/stages-of-als-progression-6829517
    The five stages are: Stage 1: One CNS area involved, Stage 2: Two CNS areas invoked, Stage 3: Three CNS areas involved, Stage 4: Nutritional or respiratory failure that requires a feeding tube or artificial respiration, Stage 5: Death. […] The stages are: Stage 0: No functional impairment, Stage 1: Loss of one type of function, Stage 2: Loss of two types of function, Stage 3: Loss of three types of function, Stage 4: Loss of four types of function, Stage 5: Death. […] Usually, ALS involves both the bulbar and the spinal regions although the sequence is not always consistent. […] A key feature of ALS is that it involves movement and not sensation. Additionally, this condition is one of only a few neurological disorders that cause both upper motor neuron and lower motor neuron involvement. Each of these effects leads to physical weakness.
  • #89 Stages of ALS: Progression Before and After Diagnosis
    https://www.verywellhealth.com/stages-of-als-progression-6829517
    The five stages are: Stage 1: One CNS area involved, Stage 2: Two CNS areas invoked, Stage 3: Three CNS areas involved, Stage 4: Nutritional or respiratory failure that requires a feeding tube or artificial respiration, Stage 5: Death. […] The stages are: Stage 0: No functional impairment, Stage 1: Loss of one type of function, Stage 2: Loss of two types of function, Stage 3: Loss of three types of function, Stage 4: Loss of four types of function, Stage 5: Death. […] Usually, ALS involves both the bulbar and the spinal regions although the sequence is not always consistent. […] A key feature of ALS is that it involves movement and not sensation. Additionally, this condition is one of only a few neurological disorders that cause both upper motor neuron and lower motor neuron involvement. Each of these effects leads to physical weakness.
  • #90 The stages of amyotrophic lateral sclerosis (ALS) | ALS News TodayEnvelope icon
    https://alsnewstoday.com/stages-of-als/
    By the late stages of ALS, most muscles involved in voluntary movement become paralyzed. Patients generally have very limited or no ability to move under their own power, and will require assistance with most day-to-day activities. […] Most individuals in late-stage ALS will require ventilatory support when breathing function declines significantly. […] ALS is a fatal disease, and most people with the disorder will die within about two to five years after symptom onset. […] The speed at which amyotrophic lateral sclerosis (ALS) progresses can vary a lot from person to person, but typically the disease progresses quite rapidly. Most patients reach the late stage of ALS within two to three years after symptom onset, and the average survival time is about two to five years from onset. […] Although there is no cure for amyotrophic lateral sclerosis (ALS), there are several approved therapies that can slow disease progression, helping patients to maintain functionality for longer and prolonging survival.
  • #91 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    As the disease progresses, muscle weakness and atrophy spread to other parts of your body. People with ALS may develop problems with: Chewing food and swallowing (dysphagia), Drooling (sialorrhea), Speaking or forming words (dysarthria), Breathing (dyspnea), Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms), Constipation, Maintaining weight and getting enough nutrients. […] Eventually, people with ALS will not be able to stand or walk, get in or out of bed on their own, use their hands and arms, or breathe on their own. […] Most people with ALS die from being unable to breathe on their own (known as respiratory failure,) usually within three to five years from when the symptoms first appear. However, about 10% survive for a decade or more. […] There is no treatment to reverse damage to motor neurons or cure ALS at this time. However, some treatments may slow progression of the disease, improve quality of life, and extend survival.
  • #92 Amyotrophic Lateral Sclerosis (ALS) | HealthLink BC
    https://www.healthlinkbc.ca/healthwise/amyotrophic-lateral-sclerosis-als
    Over time, ALS also causes: Muscle twitching. Trouble using your hands and fingers to do tasks. Problems with speaking, swallowing, eating, walking, and breathing. Problems with memory and thinking. Changes in personality. […] ALS doesn’t cause numbness or loss of feeling. […] How quickly ALS gets worse is different for everyone. But over time, people who have ALS gradually become more disabled. In most cases, death will occur within 3 to 5 years after symptoms begin, although some people do live for many years, even decades. […] Breathing problems and problems with swallowing and getting enough food are the most common serious problems from ALS. As the muscles in the throat and chest weaken, swallowing, coughing, and breathing problems tend to get worse. These problems can lead to injury or illness and, over time, to death. Respiratory failure is the most common cause of death.
  • #92 Amyotrophic Lateral Sclerosis (ALS) | National Institute of Neurological Disorders and Stroke
    https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
    As the disease progresses, muscle weakness and atrophy spread to other parts of your body. People with ALS may develop problems with: Chewing food and swallowing (dysphagia), Drooling (sialorrhea), Speaking or forming words (dysarthria), Breathing (dyspnea), Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms), Constipation, Maintaining weight and getting enough nutrients. […] Eventually, people with ALS will not be able to stand or walk, get in or out of bed on their own, use their hands and arms, or breathe on their own. […] Most people with ALS die from being unable to breathe on their own (known as respiratory failure,) usually within three to five years from when the symptoms first appear. However, about 10% survive for a decade or more. […] There is no treatment to reverse damage to motor neurons or cure ALS at this time. However, some treatments may slow progression of the disease, improve quality of life, and extend survival.
  • #93 Simply Stated: Updates in Amyotrophic Lateral Sclerosis (ALS) – Quest | Muscular Dystrophy Association
    https://mdaquest.org/simply-stated-updates-in-amyotrophic-lateral-sclerosis-als/
    As ALS progresses, affected people may experience a variety of additional symptoms, including constipation, dysphagia (difficulty swallowing), and incomplete eye closure and drooling due to weakened facial muscles. Some may have symptoms similar to Parkinson’s disease, such as lack of facial expressions (facial masking), tremor, bradykinesia, and postural instability. About 20-30% of people with ALS may have sensory impairments, such as reduced ability to feel pain or temperature or reduced awareness of body positioning (proprioception). In some people with ALS, pain is a side effect of muscle problems (cramps, spasticity, etc.). […] ALS is relentlessly progressive. Symptoms typically spread within the location of onset (e.g. limb, bulbar) and then to other body regions in a predictable pattern. Eventually, ALS progression leads to life-threatening respiratory failure and/or dysphagia. In most cases, this occurs within 3-5 years following symptom onset, however, about 10% of people diagnosed with ALS survive for a decade or more.