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ALS is a neurological disease that causes significant disability. If diagnosed early, physicians are able to treat some ALS symptoms and help the patient keep control of muscles a little longer. This article outlines early and advanced symptoms of ALS.

Amyotrophic lateral sclerosis (ALS) is a rare neurological disease that destroys nerve cells (neurons) and causes significant disability.

Symptoms refer to what an individual experiences or feels, while a sign is what can be seen or measured.

As the disease has a variable course depending on the type of ALS, the symptoms and signs of ALS tend to vary from individual to individual.

In the early stages of the disease, the signs can seem almost non-important and can often be dismissed as normal signs of aging. Most often, ALS starts in the hands or limbs at the early stage. Then, it spreads to other parts of the body. As the disease progresses and more neurons deteriorate, the muscles continue to progressively weaken. Eventually, in the later stages, the disease affects the ability to eat, speak and breathe.

While ALS does not have a cure yet, if diagnosed early, physicians are able to treat some symptoms and help the patient keep control of muscles a little longer. Therefore, it is important to be aware of the symptoms and know when to go see a physician.

Early signs of ALS

As ALS specifically affects motor neurons (nerve cells that are responsible for voluntary movements such as walking or talking), ALS generally starts with muscle twitching, weakness in a limb, or slurred speech.

Signs of ALS tend to appear slowly and over time. In fact, it can start off which something very simple, such as a weak feeling in hands or feet. For example, the patient may notice a funny feeling when trying to do basic muscle movements such as gripping the handle of a door or slur some words when speaking or have a hard time walking in a straight line. Each patient will experience different symptoms in different sequences, particularly at the beginning.

The early symptoms and signs of ALS can include:

  • Problems conducting everyday activities such as eating
  • Tripping, stumbling and falling when walking
  • Weakness in limbs including the leg, feet or ankles
  • Weakness when using hands
  • Clumsiness and dropping things
  • Issues talking such as slurred or “thick” speech
  • Problems swallowing food
  • Muscle cramps and twitching in arms, shoulders, and tongue
  • Difficulty holding head up
  • Issues with keeping good posture or balance.
  • Weight loss
  • Reduced muscle tone and mass
  • Shortness of breath
  • Increased or decreased reflexes
  • Pseudobulbar effect (uncontrollable periods of laughing or crying)
  • Feeling weak
  • Fatigued
  • Muscle cramping and twitching
  • Muscle stiffness or rigidity

At this stage, ALS doesn't usually affect cognition, senses (sight, touch, hearing, taste and smell) or thinking ability. Hence, it is possible to remain actively involved with family and friends. Additionally, for most people, muscles of the eyes and bladder remain functional at this stage.

While pain is not commonly reported by people living with ALS, some people experience physical pain, joint discomfort or cramping.

Advanced Symptoms

Over time, ALS worsens and affects almost all muscles that are under voluntary control. ALS symptoms worsen progressively as the disease advances and eventually will affect the patient’s ability to move, eat, talk and breathe. However, ALS doesn’t affect all muscles and organs in the body, such as the heart.

Ultimately, ALS tends to weaken the diaphragm (a muscle needed for lungs to work). Hence, patients with advanced ALS have trouble breathing. Patients with ALS are encouraged to discuss breathing support options as their breathing function will seriously decline and communication at a later stage is difficult.

ALS patients will also find it hard to walk and move around and will require support, such as power wheelchairs, in order to be able to do so.

30-50% of ALS patients will also eventually experience a change in their cognition or behavior, which means that their thinking and behavior will be affected. At the advanced stage, patients with ALS can display inattention, slowed thinking, difficulty communicating verbally, trouble with facial or reading comprehension, obsessive or strange behavior as well as apathy and depressive behavior.

Approximately 20-25% of patients will develop severe cognitive impairment in the form of a disease known as frontal lobe dementia, which is a condition which causes severe deterioration of different parts of the brain.

These are some of the signs and symptoms of advanced ALS:

  • Muscles become ever weaker
  • Muscle mass decreases significantly
  • Difficulties eating, particularly chewing and swallowing
  • Coughing when eating or drinking a beverage
  • Decreased food intake
  • Difficulties talking and being understood
  • Significant trouble breathing
  • Fatigue due to muscle exhaustion
  • Pronounced Weight Loss
  • Insomnia due to discomfort
  • Excessive saliva or dry mouth
  • Severe headaches and dizziness
  • Cognitive and behavior issues
  • Mood swings

Not all patients with ALS will experience all of these symptoms. Additionally, while some of these symptoms will stay the same for a period of time, they will not improve and will worsen over time. At the end stage of the disease, the patient will die likely due to a lack of air and the inability to use lung muscles. Patients can also die due to choking from swallowing food. Finally, patients may also die from heart disease, such as a heart attack.

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