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Amyotrophic Lateral Sclerosis (ALS)

Dr Rohit Bhaskar
Dr Rohit Bhaskar
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Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease, is a progressive neuromuscular disease.

ALS is characterized by a progressive degeneration of motor nerve cells in the brain (upper motor neurons) and spinal cord (lower motor neurons). When the motor neurons can no longer send impulses to the muscles, the muscles begin to waste away (atrophy), causing increased muscle weakness.

ALS does not impair a person’s intellectual reasoning, vision, hearing or sense of taste, smell and touch. In most cases, ALS does not affect a person’s sexual, bowel or bladder functions.

ALS is often referred to as a syndrome, because the disease itself becomes apparent in various patterns. ALS occurs rarely and without regularity.

Amyotrophic Lateral Sclerosis (ALS)

What are the symptoms of ALS?

The following symptoms become more noticeable as ALS progresses:

  • Twitching and cramping of muscles, especially those in the hands and feet.
  • Impaired use of arms and legs.
  • Weakness and fatigue.
  • Weight loss.
  • “Thick” speech and difficulty projecting voice.

These symptoms characterize ALS as the disease becomes more severe:

Please note that severe pain is not a typical symptom of ALS.

Who is affected by ALS?

Throughout the world, an average of 1 to 2 per 100,000 people are diagnosed with ALS every year. Men are affected more frequently than women. Most people are diagnosed with ALS in their mid-50s, but ALS can also affect young adults in their mid-teens. However, onset of the disease before 30 years of age is rare.

What are the classifications of ALS?

Because no specific qualities can be predicted of all people diagnosed with ALS, the cause is unknown. The following classification is used by physicians:

Classical ALS

A progressive neurological disease characterized by a deterioration of upper and lower motor nerve cells (neurons). This type of ALS affects over two-thirds of all people with ALS.

Primary Lateral Sclerosis

A progressive neurological disease in which the upper motor nerve cells (neurons) deteriorate. If the lower motor neurons are not affected within two years, the disease usually remains a pure upper motor neuron disease. This is the rarest of all forms of ALS.

Progressive Bulbar Palsy (PBP)

A condition that starts with difficulties speaking, chewing and swallowing due to lower motor nerve cell (neuron) deterioration. This disorder affects about 25% of all people with ALS.

Progressive Muscular Atrophy (PMA)

A progressive neurological disease in which the lower motor neurons deteriorate. If the upper motor neurons are not affected within two years, the disease usually remains a pure lower motor neuron disease.

Familial ALS

A progressive neurological disease that affects more than one member of the same family. This type of ALS accounts for a very small number of people with ALS in the United States (5-10%).

How is ALS diagnosed?

A complete physical exam and an evaluation of the person’s medical history are required to properly diagnose ALS. Several laboratory and radiographic tests are also conducted to confirm the diagnosis, including:

How is ALS treated?

There is currently no cure for ALS, yet there are several treatment options that can make people diagnosed with ALS more comfortable.

Treatment options include:

  • Medications to relieve painful muscle cramps, excessive salivation and other symptoms.
  • Physical therapy to maintain mobility and ease the discomfort of muscle stiffness, cramps and fluid retention.
  • Nutritional counseling to promote good nutrition and recommend other dietary options when swallowing becomes difficult.
  • Speech therapy and communication training to maintain as many verbal communication skills as possible. Communication training also includes non-verbal techniques.
  • Assistive devices such as splints, corrective braces, grab bars, reach devices, etc. to help with daily activities such as dressing, eating, toileting and bathing.
  • Special equipment such as wheelchairs, electric beds or mattresses and boards to maximize functional independence.

What is the Care Path For People With Presumed ALS?

Initial Visit

  • Neurological evaluation is conducted
  • Reasons for other tests are discussed
  • Laboratory studies are conducted
  • Additional testing is performed, such as an electromyogram (EMG), neuro-imaging and muscle testing

Neurological evaluation is conducted

Follow-Up Visit in 1 to 3 weeks

  • Test results are discussed
  • ALS diagnosis is introduced
  • Services offered at The Cleveland Clinic’s Center for ALS and Related Disorders are described
  • Holistic care philosophy is explained
  • ALS informational booklet is provided
  • Research and clinical trial opportunities are discussed

Research Candidate

  • Specific research options are explained

Specific research options are explained

Non-Research Candidate

  • ALS patient attends the ALS Center every 3 months
  • Non-IND trials are discussed as an option

Follow-Up Care For All ALS Patients

  • ALS patient attends the ALS Center every 3-6 months or as needed
  • Consultation with the Pulmonary Care Team is scheduled if necessary
  • Consultation with a gastroenterologist is scheduled to discuss the percutaneous endoscopic gastrostomy procedure
  • Patients’ legal options – including durable power of attorney and living wills – are explained
  • Home care referrals are discussed
  • Hospice referrals are discussedAs the muscles weaken in the voice box (larynx), throat (pharynx), roof of the mouth (soft palate) tongue and lips, people with amyotrophic lateral sclerosis (ALS) may find it difficult to pronounce words clearly.

Some resulting speech impairments may be:

  • Hoarse or strained voice
  • Muffled or nasal-sounding voice
  • Unclear or slurred speech

Speech-Language Pathologists

Speech-language pathologists can help people with ALS maintain as many communication skills as possible. They also teach techniques that conserve energy, including non-verbal communication skills. Speech-language pathologists are also available to:

  • Recommend appropriate communication technologies to provide treatment that will aid in the success of daily activities.
  • Treat all types of speech, language and communication problems.
  • Evaluate swallowing function and recommend changes as necessary.

Tips to Maintain and Enhance Communication

  • Choose an environment with reduced noise – it can be quite tiring to try to “talk over” the television or radio.
  • Speak slowly.
  • Be certain your listener can see your face. Look at the person while you are talking. A well-lit room enhances face-to-face conversation, increasing intelligibility.
  • Use short phrases – say one or two words or syllables per breath.
  • Over-articulate your speech by prolonging the vowels and exaggerating the consonants.
  • Choose a comfortable posture and position that provide support during long and stressful conversations.
  • Be aware that exercises intended to strengthen weakening muscles may be counter-productive. Always ask your speech-language pathologist which exercises are right for you.
  • Plan periods of vocal rest before planned conversations or phone calls. Know that fatigue significantly affects your speaking ability. Techniques that work in the morning may not work later in the day.
  • If you are soft spoken and your voice has become low, consider using an amplifier.
  • If you are on a respirator (with fully inflated cuffs), an electrolarynx or respiratory tube that provides an alternative air source may be used.
  • If some people have difficulty understanding you, the following strategies may help:
  • If you are able to write without difficulty, always carry a paper and pen as a backup so you can write down what you are trying to say.
  • If writing is difficult, use an alphabet board to point or scan to the first letter of the words that are spoken.
  • Spell words out loud or on an alphabet board if they are not understood.
  • Establish the topic before speaking.
  • Use telegraphic speech – leave out unnecessary words to communicate the meaning of the topic.

Nonverbal Communication

Nonverbal communication, also called augmentative and alternative communication (AAC), is a method of communicating without spoken words. When communication needs cannot be met through speech, the following techniques can be practiced by people with ALS:

  • Residual speech (or, making the best use out of what speaking ability is left).
  • Effective use of expressions and gestures.

Non-verbal communication can help people with speech difficulties actually speak better by:

  • Reducing the frustration and stress of being unable to communicate.
  • Alleviating the pressure to speak.
  • Allowing the person to be more relaxed and come across in a more intelligible manner.

Assistive Devices

Here’s a sample of the assistive devices that are available to help people with ALS communicate more clearly.

  • Palatal lift – a dental apparatus that is similar to a retainer. It lifts the soft palate and stops air from escaping out of the nose during speech.
  • Amplification – a personal amplifier that can be used to increase vocal loudness in soft-spoken people. The amplifier also decreases voice fatigue.
  • TTY telephone relay system – a telephone equipped with a keyboard so speech can be typed and read by a relay operator to the listener. Either the whole message can be typed or just the words that are not understood can be typed.
  • Low technology devices – notebooks and language boards can be used as an alternative communication techniques.
  • High technology electronic speech enhancers, communication devices – computers with voice synthesizers and dedicated communication devices are available.

Patients who are interested in purchasing an electronic communication aid should discuss this with the speech-language pathologist before contacting sales representatives for these devices.

Communication Partners

Here are some ways in which listeners can help people who have difficulty speaking and communicating:

  • Talk to the person only face-to-face and look at the person as he/she is speaking.
  • Ask questions that require a “yes” or “no” answer.
  • Repeat the part of the sentence that you understood. (For example, “You want me to go upstairs and get the what?”)
  • Ask the person to repeat what he/she has said, or ask him/her to speak slower or spell out the words that you did not understand.

Preparing for Emergencies

  • Use an intercom system or baby monitor to alert others that there is an emergency.
  • Use bells or buzzers if you are not able to speak. Use “codes” that signify urgency – for example, a tinkling bell may mean, “I’d like company” while an air horn means there’s an emergency.
  • Carry a portable phone that is equipped with pre-programmed numbers.
  • Pre-program all of your telephones so they can automatically dial the necessary emergency number(s).
  • Consider a “Life Call” button if you spend time alone.

Physical therapy can help people with amyotrophic lateral sclerosis (ALS) learn to adjust to their physical disabilities. Physical therapy can maximize existing capabilities and prevent further loss of motion or prevent pain that may develop from stiff joints.

Physical Therapist

By evaluating each patient’s joint range of motion, strength and general mobility skills, physical therapists can provide guidance for such tasks as walking or getting out of a chair. They specialize in:

  • Improving function and providing instruction on managing physical disabilities.
  • Recommending appropriate exercises to maintain flexibility, while preventing and reducing physical pain.
  • Providing instruction regarding the use of assistive devices, braces or other mobility aids to maximize independence.

Exercise Guidelines

Aerobic exercise can increase muscle efficiency and endurance by increasing heart rate, respiratory rate and overall cardiovascular fitness. Remember to exercise in moderation - exercising to the point of fatigue may actually result in increased muscle weakness.

Stretching exercises

May help decrease the frequency or intensity of muscle cramping. These should be done daily to prevent pain and stiffness.

Range of motion exercises (ROM)

Help move the joints through their full range of motion. These should be performed actively, if possible, or passively if muscle weakness limits movement. These should be done daily to prevent pain and stiffness.

Strengthening exercises

Are not recommended. Working out with weights will not strengthen those muscles already weakened by ALS and could result in increased muscle weakness.

Equipment Recommendation

Assistive Devices

There are various types of assistive devices that can make walking safer. A cane may be the most useful tool when one leg is weaker than the other, or when there are mild balance deficits. Here are some guidelines for cane use:

  • The cane is held on the stronger side of the body while the weight is shifted away from the weaker side.
  • A quad cane (or four-legged cane) provides more stability than a standard cane.
  • Walkers may be more appropriate when there is significant leg weakness. They can also provide support for maintaining balance. Wheels or platforms may be added to the walker if necessary.


Weakness of the leg muscles may make it more difficult to maneuver on stairs, rise from a chair or walk. Bracing or other aids may be recommended.

An ankle-foot brace can stabilize the ankle when there is weakness in the foot muscles. This brace fits into an ordinary shoe and prevents the toes from dragging during the swing phase of walking.

Weakness in the neck muscles may make it difficult to hold your head up. A neck brace may be recommended to make you more comfortable.


Wheelchairs may provide patients with more independence and less reliance on others. Wheelchairs are usually recommended when a patient experiences excessive fatigue, unsteadiness or occasional falls.

How does ALS affect breathing?

Breathing is a function of lungs as well as the muscles that move the chest and lungs. The breathing muscles include the diaphragm (the major muscle that separates the chest from the abdomen, used for inhaling) and the muscles between the ribs. Amyotrophic Lateral Sclerosis (ALS) often involves breathing muscles, resulting in ineffective breathing or respiratory failure.

What pulmonary problems can I get with ALS?

  • Shortness of breath when walking or during rest
  • Inability to lie flat in bed
  • Poor strength of cough
  • Inability to clear secretions from the throat and lungs
  • Increased salivary secretions
  • Recurrent chest infections and pneumonia
  • Respiratory failure (high blood carbon dioxide level)

How can pulmonary therapy help breathing?

The pulmonary care team (including doctors and respiratory therapists) can work with you to:

  • Monitor the strength of your respiratory muscles to diagnose early evidence of weakness
  • Provide breathing assistive devices, such as:
  • Suction machine to clear mouth and throat secretions
  • Non-invasive ventilation
  • In-Exsufflator
  • Flu vaccine and pneumovax (pneumonia vaccine)

What are some concerns regarding non-invasive ventilation?

  • Mask is uncomfortable, puts pressure on the nose and face and makes you feel claustrophobic
  • Causes dry nose
  • Causes red eyes
  • Machine is too noisy
  • Air can leak from the mask
  • Poor coordination of breathing

What are some recommendations to improve breathing?


  • Do not lie down immediately after eating.
  • Avoid eating large meals which can prevent diaphragm from expanding fully.
  • Maintaining good nutritional status is essential to ensure optimal functional capability of breathing muscles.


Elevate your head with pillows from 15 to 30 degrees to help keep abdominal organs from the diaphragm.

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