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Amyotrophic Lateral Sclerosis

Condition: Amyotrophic Lateral Sclerosis (ALS)

Brief Overview: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that damages motor neurons, the nerve cells in the brain and spinal cord responsible for controlling voluntary muscle movement. As motor neurons deteriorate, they lose the ability to communicate with muscles. This leads to progressive muscle weakness, muscle wasting, difficulty walking, speaking, swallowing, and eventually breathing.

ALS can begin in different ways. Some patients first notice weakness in an arm or leg, while others initially develop changes in speech or swallowing, known as bulbar-onset ALS. Disease progression and the order in which functions are affected vary substantially between patients.

Sensation such as touch, taste, hearing, and vision is generally preserved. Bladder control and eye movements also typically remain relatively preserved, although ALS is more complex than a purely motor disorder. Some patients develop cognitive or behavioral changes associated with the ALS-frontotemporal spectrum.

There is currently no cure for ALS, but medications may slow disease progression in some patients, and respiratory, nutritional, rehabilitation, communication, and supportive interventions can help maintain function and quality of life.

Prevalence: According to UpToDate, in Europe and North America, “prevalence rates range between 2.7 and 7.4 per 100,000 person-years.”

Etiology: Per UpToDate, “The etiology of ALS is unknown. A number of potential mechanisms have been proposed, including abnormal RNA processing, disorders of protein quality control, excitotoxicity, cytoskeletal derangements, mitochondrial dysfunction, viral infections, apoptosis, growth factor abnormalities, inflammatory responses, and others.”

Risk Factors:

  • Older age
  • Male sex
  • Family history
  • Tobacco use

Commonly Associated Conditions:

  • Dysphagia.
  • Dysarthria.
  • Respiratory muscle weakness.
  • Weak cough and difficulty clearing secretions.
  • Weight loss and malnutrition.
  • Muscle cramps.
  • Spasticity.
  • Pain related to immobility or positioning.
  • Constipation.
  • Excess saliva or difficulty managing secretions.
  • Pseudobulbar affect.
  • Depression or anxiety.
  • Sleep disturbance.
  • Falls and mobility impairment.
  • Cognitive or behavioral impairment.
  • Frontotemporal dementia in a subset of patients.

Up to approximately half of patients with ALS may develop some degree of cognitive or behavioral change, although many remain cognitively intact. Approximately 10–20% may develop frontotemporal dementia.

Common Medications:

  • Riluzole
  • Edaravone
  • Tofersen, for familial ALS associated with pathologic variants of the SOD1 gene
  • Medications for symptomatic treatment may be indicated

Common Labs, Imaging, and Tests:

  • Electrodiagnostic testing with nerve conduction studies and electromyography (EMG)
  • Neuroimaging – MRI
  • Labs – CBC with differential, electrolytes, liver function tests, thyroid function studies, creatine phosphokinase, erythrocyte sedimentation rate and high-sensitivity C-reactive protein, ANA and RF, vitamin B12 level, anti-GM1 antibody, serum protein electrophoresis with immunofixation and urine protein electrophoresis with immunofixation
  • Respiratory testing
  • Swallowing evaluation
  • Genetic testing
  • Neuromuscular ultrasound may be indicated
  • Muscle biopsy may be ordered for some

Common Symptoms:

Limb Symptoms

  • Progressive arm or leg weakness.
  • Hand weakness.
  • Dropping objects.
  • Difficulty buttoning clothing.
  • Difficulty lifting the arms.
  • Foot drop.
  • Tripping.
  • Difficulty climbing stairs.
  • Difficulty rising from a chair.
  • Difficulty walking.
  • Falls.
  • Muscle wasting.
  • Muscle twitching/fasciculations.
  • Muscle cramps.

Upper Motor Neuron Symptoms

  • Muscle stiffness.
  • Spasticity.
  • Hyperreflexia.
  • Clonus.
  • Difficulty coordinating voluntary movement because of increased tone.

Bulbar Symptoms

Bulbar symptoms result from weakness of muscles involved in speech, chewing, and swallowing.

Possible symptoms include:

  • Slurred speech.
  • Soft or nasal speech.
  • Voice changes.
  • Difficulty being understood.
  • Difficulty chewing.
  • Prolonged meals.
  • Difficulty swallowing.
  • Coughing or choking with food or liquids.
  • Drooling or difficulty controlling saliva.
  • Food or liquids entering the airway.
  • Weight loss.

ALS progressively weakens muscles of the mouth and throat, increasing choking and aspiration risk.

Respiratory Symptoms

Possible early signs of respiratory muscle weakness include:

  • Shortness of breath with activity.
  • Shortness of breath while talking.
  • Difficulty lying flat.
  • Needing to sleep more upright.
  • Frequent nighttime awakening.
  • Morning headaches.
  • Morning confusion or feeling "foggy."
  • Daytime fatigue.
  • Excessive daytime sleepiness.
  • Frequent sighing or yawning.
  • Weak cough.
  • Difficulty clearing mucus or secretions.

Care Coordinator Note: Do not rely on the absence of obvious respiratory distress to determine that breathing function is stable. Respiratory muscle weakness can initially be more noticeable during sleep or when lying flat.

Pseudobulbar Affect

Patients may experience:

  • Sudden uncontrollable crying.
  • Sudden uncontrollable laughing.
  • Emotional episodes that do not match how they actually feel.

PBA should not automatically be interpreted as depression or a psychiatric condition.

Cognitive and Behavioral Symptoms

Some patients may develop:

  • Reduced judgment.
  • Apathy.
  • Irritability.
  • Loss of social inhibition.
  • Difficulty with executive functioning.
  • Difficulty shifting between tasks.
  • Repetitive behaviors.
  • Language changes.
  • Reduced insight into illness.

These symptoms may affect adherence, safety decisions, advance-care planning, and caregiver needs.

Common Treatments: ALS management is best provided through a multidisciplinary ALS clinic/team that may include neurology, respiratory therapy, physical therapy, occupational therapy, speech-language pathology, nutrition, social work, mental health support, palliative care, and equipment specialists.

  • Medication (see above)
  • Noninvasive ventilation
  • Airway clearance
  • Feeding tube
  • Physical and occupational therapy
  • Speech therapy
  • Palliative care

Physical Findings:

Lower Motor Neuron Findings

  • Muscle weakness.
  • Muscle atrophy.
  • Fasciculations.
  • Reduced muscle tone in affected muscles.
  • Reduced reflexes in some regions.

Upper Motor Neuron Findings

  • Hyperreflexia.
  • Spasticity.
  • Increased muscle tone.
  • Pathologic reflexes.

ALS may produce a combination of upper and lower motor neuron findings.

Potential Complications and Contraindications:

  • Progressive weakness and paralysis.
  • Falls and injuries.
  • Loss of independent mobility.
  • Respiratory muscle failure.
  • Ineffective cough.
  • Retained secretions.
  • Respiratory infections.
  • Aspiration pneumonia.
  • Dysphagia.
  • Choking.
  • Malnutrition.
  • Dehydration.
  • Significant weight loss.
  • Communication loss.
  • Contractures.
  • Pressure injuries.
  • Constipation.
  • Pseudobulbar affect.
  • Cognitive or behavioral impairment.
  • Anxiety or depression.
  • Increasing caregiver burden.

General Health and Lifestyle Guidance:

  • Maintain regular ALS-specialist/neurology follow-up.
  • Take medications exactly as prescribed.
  • Report any new or changed neurological symptom to the neurologist.
  • Attend respiratory testing as recommended.
  • Use prescribed noninvasive ventilation consistently.
  • Use cough-assist or suction equipment according to respiratory-team instructions.
  • Keep respiratory equipment readily accessible.
  • Report difficulty tolerating respiratory equipment rather than simply discontinuing it.
  • Follow speech-language pathology recommendations for swallowing.
  • Report coughing or choking with food or liquids.
  • Monitor body weight.
  • Report unexplained weight loss or significantly decreased intake.
  • Follow individualized nutritional recommendations.
  • Discuss feeding-tube options before swallowing or respiratory function becomes severely impaired.
  • Use prescribed walker, wheelchair, brace, transfer equipment, or other adaptive equipment.
  • Avoid unnecessary falls by requesting assistance when transfers or walking become unsafe.
  • Conserve energy by spacing demanding activities throughout the day.
  • Maintain appropriate range-of-motion exercises according to PT/OT recommendations.
  • Encourage early planning for communication devices.
  • Review home accessibility and caregiver needs as function changes.
  • Discuss advance-care planning and respiratory-support preferences while the patient can actively participate in decisions.
  • Encourage caregivers to seek assistance before care needs become unmanageable.

Suggested Questions to Ask Patients:

Strength and Function

  • Have you noticed any change in your strength since our last call?
  • Are your arms, hands, legs, or neck becoming weaker?
  • Are you dropping things more often?
  • Are you having more trouble getting out of a chair or bed?
  • Are transfers becoming more difficult?
  • Are you having difficulty climbing stairs?
  • Has your walking changed?
  • Have you fallen or almost fallen?
  • Are you using your prescribed walker, wheelchair, brace, or other equipment?
  • Do you feel your current equipment still meets your needs?

Breathing

  • Have you developed any new or increased shortness of breath?
  • Are you short of breath while talking?
  • Are you having more difficulty breathing when lying flat?
  • Are you sleeping with more pillows or sleeping upright?
  • Are you waking frequently at night?
  • Are you waking with headaches or feeling unusually groggy or confused?
  • Has your cough become weaker?
  • Are you having difficulty clearing mucus or saliva?
  • Are you using noninvasive ventilation?
  • Are you able to use it as prescribed?
  • Do you have a cough-assist device or suction machine?
  • Are you having any problems with your respiratory equipment?

Swallowing and Nutrition

  • Are you having difficulty chewing?
  • Are meals taking longer than they used to?
  • Are meals exhausting?
  • Are you coughing or choking when eating or drinking?
  • Does food or liquid feel like it is going down the wrong way?
  • Are you having difficulty swallowing medications?
  • Has your appetite decreased?
  • Have you lost weight?
  • Are you able to drink enough fluids?
  • Have you met with a dietitian or speech-language pathologist recently?
  • Have you discussed feeding-tube options with your ALS team?

Speech and Communication

  • Has your speech become softer, slower, slurred, or more difficult to understand?
  • Does speaking make you tired?
  • Are family members having more trouble understanding you?
  • Do you have a communication device or alternative communication method?
  • Have you worked with speech therapy on future communication needs?

Saliva and Secretions

  • Are you having difficulty managing saliva?
  • Are you drooling more?
  • Are your secretions becoming thick or difficult to clear?
  • Do you have suction equipment?
  • Is your current treatment adequately controlling the problem?

Muscle Symptoms

  • Are you having muscle cramps?
  • Are you having muscle twitching?
  • Has stiffness or spasticity increased?
  • Is pain interfering with sleep or daily activities?

Cognition and Behavior

When appropriate, ask the caregiver:

  • Have you noticed any change in judgment or decision-making?
  • Any significant personality or behavioral changes?
  • Increased apathy or loss of motivation?
  • Repetitive or unusual behaviors?
  • Is the patient still able to manage medications and medical decisions safely?

Pseudobulbar Affect

  • Have you had episodes of laughing or crying that you cannot control?
  • Do these episodes seem out of proportion to how you actually feel?
  • Are they interfering with social activities or communication?

Caregiver and Home Support

  • Who is helping you at home?
  • Has the amount of help you need increased?
  • Is the caregiver able to safely assist with transfers?
  • Does the caregiver feel overwhelmed or physically unable to provide needed care?
  • Do you need additional home-health, equipment, respite, or community resources?

⚠️ Standard Neurology Symptom-Change Guidance

For any new neurologic symptom or change/worsening of an existing neurologic symptom, advise the patient or caregiver to contact the patient's neurologist/ALS team to report the change.

Examples include new or worsening:

  • Weakness.
  • Falls.
  • Difficulty walking or transferring.
  • Muscle stiffness or spasms.
  • Speech changes.
  • Swallowing difficulty.
  • Difficulty managing saliva.
  • Respiratory symptoms.
  • Weak cough.
  • Cognitive or behavioral change.
  • Other neurological or functional decline.

Care coordinators should not independently determine that a new or worsening symptom is simply expected progression of ALS.

If symptoms meet criteria for urgent or emergency evaluation, do not delay care while waiting for the neurologist to respond. Follow organizational escalation policies and direct the patient to the appropriate level of urgent or emergency care.

⚠️ Telephone Escalation Guidance

Emergency — Follow 911/Emergency Protocol

Follow organizational emergency protocol for:

  • Severe or rapidly worsening difficulty breathing.
  • Patient unable to speak because of breathlessness.
  • Severe choking or airway obstruction.
  • Inability to clear airway secretions with usual prescribed equipment.
  • Blue/gray discoloration or other signs of severe respiratory compromise.
  • Loss of consciousness or inability to awaken normally.
  • Sudden facial droop, one-sided weakness, or acute speech change suspicious for stroke.
  • Severe allergic reaction.
  • Serious fall with head injury, loss of consciousness, major injury, or inability to safely get up.

Do not assume acute symptoms are simply progression of ALS.

Prompt/Same-Day Clinical Escalation

Notify the appropriate nurse/provider/ALS team according to organizational protocol for:

  • New or noticeably worsening shortness of breath.
  • New difficulty breathing when lying flat.
  • New morning headaches or significant morning confusion.
  • Noticeably weaker cough.
  • Increasing difficulty clearing secretions.
  • New or worsening coughing/choking during meals.
  • New swallowing difficulty.
  • Difficulty swallowing medications.
  • Significant decrease in food or fluid intake.
  • Unexplained or ongoing weight loss.
  • New falls or rapid decline in mobility.
  • New inability to safely transfer.
  • Significant new weakness.
  • New communication difficulty.
  • Rapid increase in caregiver needs.
  • Respiratory equipment no longer working adequately or unable to be tolerated.
  • New cognitive or behavioral changes that affect safety or medical decision-making.

Respiratory weakness can progress before severe daytime symptoms appear, so new respiratory complaints in a patient with ALS should be reported promptly to the ALS/neurology team.

Suggested Talking Points:

Explaining ALS

"ALS affects the nerve cells that tell your muscles what to do. As those nerve cells become damaged, the muscles gradually become weaker."

Discussing new symptoms

"Because ALS can affect several different muscle groups over time, your neurologist needs to know about any new or worsening symptom so the care plan and equipment can be adjusted."

Discussing breathing

"ALS can weaken the muscles used for breathing. Things like difficulty lying flat, morning headaches, a weaker cough, or increasing shortness of breath can be early signs that your breathing muscles need more support."

Discussing swallowing

"If you're coughing or choking while eating or drinking, meals are taking much longer, or you're losing weight, your ALS team should know. There are treatments and strategies that can make eating safer and help maintain nutrition."

Discussing feeding tubes

"A feeding tube can be used to supplement nutrition, fluids, and medications. It doesn't automatically mean you can never eat by mouth again. The best time to discuss it is before eating and breathing become severely difficult."

Discussing respiratory equipment

"Using breathing or cough-assist equipment doesn't mean your ALS suddenly became severe. These devices are tools that can reduce the work your muscles have to do and help keep your airway clear."

Discussing communication

"If speaking is becoming harder, it's helpful to involve speech therapy early. Communication devices are easier to plan for before speech becomes severely limited."

Discussing pseudobulbar affect

"ALS can sometimes cause uncontrollable laughing or crying that doesn't match how you actually feel. That's a neurological symptom called pseudobulbar affect, and treatment is available."

Discussing caregivers

"As strength changes, the safest way to transfer, bathe, dress, or move around may change too. We want to adjust support and equipment before either you or your caregiver gets hurt."

Suggested SMART Goal Examples

1. Neurology Follow-Up
"I will attend my scheduled ALS/neurology appointment on ___ and report any changes in strength, breathing, swallowing, or speech."

2. Respiratory Equipment Adherence
"I will use my prescribed noninvasive ventilation according to my respiratory team's instructions every night for the next 30 days."

3. Cough-Assist Use
"I will use my cough-assist device according to my respiratory therapist's prescribed schedule for the next 30 days."

4. Weight Monitoring
"I or my caregiver will check and record my weight once weekly for the next 4 weeks and report continued weight loss to my ALS team."

5. Swallowing Safety
"I will follow the eating and swallowing strategies recommended by my speech-language pathologist at every meal for the next 2 weeks."

6. Nutrition Follow-Up
"I will schedule my recommended dietitian appointment within the next ___ days because eating has become more difficult."

7. Fall Prevention
"I will use my prescribed walker or wheelchair for the activities recommended by my therapy team for the next 30 days."

8. Home Safety
"I will remove loose rugs and clutter from my main walking and transfer areas within the next 7 days."

9. Energy Conservation
"I will schedule rest periods between bathing, dressing, meals, and other demanding activities each day for the next 2 weeks."

10. Communication Planning
"I will meet with speech therapy within the next ___ days to discuss communication options before my speech becomes more difficult."

11. Feeding-Tube Discussion
When clinically appropriate:
"I will discuss the benefits, risks, and timing of a feeding tube with my ALS team at my next appointment."

12. Caregiver Support
"My caregiver and I will identify at least one additional home-care, respite, or ALS support resource within the next 30 days."

13. Advance-Care Planning
"I will discuss my preferences for future respiratory support and identify my healthcare decision-maker with my healthcare team within the next 30 days."

14. Medication Adherence
"I will take my ALS medication exactly as prescribed for the next 30 days and report side effects or medication-access concerns to my healthcare team."

Sources:

⚠️ Medical Disclaimer

This resource is provided for educational and informational purposes only and is not intended to replace professional medical advice, diagnosis, or treatment. The information presented is general in nature and may not apply to every individual or health situation.

Individuals should consult their physician or other qualified healthcare professional for personalized medical advice, diagnosis, or treatment recommendations related to their specific health conditions and should not begin any new exercise program or change their diet or medications without consulting their healthcare professional.

Call 911 if you are experiencing a medical emergency.