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ALS: Diagnosis, Medical Treatment, and Nutritional Support


Amyotrophic lateral sclerosis (ALS) is difficult to diagnose early because it can mimic other neurological conditions such as Myasthenia gravis. Tests are used to rule out other diseases. Besides medications, an essential treatment for ALS is nutritional support because muscles in the mouth and throat are wasting, preparing meals and self-feeding becomes difficult, and weight loss may accelerate the progression.

ALS diagnosis

ALS, often called Lou Gehrig's disease, was first interpreted by excluding other conditions the baseball player was diagnosed with.

As no single test provides a definitive diagnosis, the patient’s physician needs to observe the patient’s symptoms, perform a physical examination and take a detailed history. A neurologic exam should be done regularly to monitor symptoms such as muscle weakness, wasting, and spasticity and whether they worsen progressively over time.

Tests may include:

  • Electromyography or electromyogram (EMG) – A needle electrode is inserted into various muscles in the patient’s body to evaluate the electrical activity of their muscle fibers when they contract and rest. Abnormalities may be a clue as to the diagnosis of ALS.
  • Nerve conduction study (NCS) – An electrical current stimulates the patient’s peripheral nerve to measure and record the nerve’s ability to send an impulse or signal along the nerve to a muscle. It can determine damage to specific nerves or muscles or nerve disease.
  • Magnetic resonance imaging (MRI) is a noninvasive procedure that uses radio waves and a powerful magnetic field to create spinal cord and brain images. It can reveal herniated discs in the patient’s neck, multiple sclerosis, head injury, and other conditions that can cause the symptoms of ALS.
  • Blood and urine tests – Analyzing blood and urine samples may be performed based on the patient’s symptoms, test results, and physical examination to eliminate other causes of the patient’s symptoms.
  • Spinal tap (lumbar puncture) – A needle is inserted between two vertebrae in the patient’s lower back to remove some fluid. The fluid will be examined for abnormal cells.
  • Muscle biopsy – While under local anesthesia, a sample of muscle tissue is removed from the patient if their physician believes their patient has a muscle disease rather than ALS.

ALS treatments

There is no cure for ALS and the damage it causes to motor neurons. The only treatments are supportive care to control symptoms, alleviate complications, provide the best quality of life possible, maximize function and increase the ease of living with the progressive, life-shortening disease.

A multidisciplinary approach consists of physicians, occupational, physical, speech, and respiratory therapists; pharmacists; nutritionists; social workers; clinical psychologists; initially home care nurses and, toward the end, hospice nurses. A specific care plan is designed for the patient considering their strengths, needs, and goals.

Medications available

Five medications are currently approved by the U.S. Food and Drug Administration (FDA) for the treatment of ALS:

  1. Rilutek (Riluzole) comes in oral form, a tablet or liquid administered through a syringe into a feeding tube. It prolongs survival by a few months, particularly in the bulbar form of ALS. It is believed to reduce damage to motor nerves by decreasing levels of glutamate. It has been on the market for more than 20 years.
  2. Radicava (Edaravone) was initially approved in 2017 as an intravenous infusion form of medication. As of 2022, it became available in an oral form. It slows the decline in functioning.
  3. ALS causes paralysis and weakness in the muscles of the throat and face. Swallowing difficulty is evident in 80% of people with ALS. Tiglutik (thickened Riluzole) was approved in 2018. It is designed to avoid the issue of crushing pills. Those with swallowing difficulty prefer the thickened form.
  4. Exservan (Riluzole oral film) was approved in 2019. It was developed for patients with extreme swallowing difficulty. It dissolves on the tongue and bypasses the need to swallow a pill or tablet.
  5. Nuedexta (a combination of two ingredients: Dextromethorphan and Quinidine). This medication may help reduce pseudobulbar bulbar affect (PBA), a medical condition that causes involuntary, sudden, and habitual outbursts of inappropriate and uncontrollable crying and laughing. It also improves swallowing and speech as well as saliva control.

Medications may be provided for depression, stiffness, extra saliva and phlegm, pain, cramps, constipation, and sleep issues.

Nutritional support

Nutritional support is key to success in living with ALS. People with Lou Gehrig’s disease require more calories to meet their daily needs than people without. Even the lack of exercise does not contribute to this need. Because muscles in the mouth and throat are wasting, eating may be difficult. Preparing meals and self-feeding and experiencing constipation, fatigue, early satiety, and decreased appetite may be challenging. Weight loss may accelerate the progression of ALS.

Five to six small meals throughout the day are recommended that contain enough calories, fluid, and fiber. Nutritionists can guide the patient to foods that are easy to consume and should be avoided. Avoid foods that crumble in the mouth, have seeds or tough skins, or are sticky. Example foods include nuts, pineapple, peanut butter, rice, corn/dry muffins, and popcorn. Other difficult foods are of mixed consistency, like cold cereal with milk, chunky soups, and salads with dressing.

The need for protein and protein supplements should be emphasized. Recommendations include drinking high-protein milkshakes, adding instant breakfast to pudding, mixing dry milk into mashed potatoes, and having meat salad with crackers. Boost calories by adding butter to a casserole, adding gravies to meat (also moistens food), adding extra mayonnaise to tuna salad, and blending milk with one cup of ice cream.

Drink liquids like thick fruit nectars if thin liquids go down the windpipe. They include milkshakes, eggnog, and yogurt drinks. The patient can also thicken thin liquids with a home (arrowroot, instant potato flakes, gelatin, and others) or commercially available thickeners.

If the food takes longer than 30 minutes to consume, then it needs to be prepared differently, changing the consistency so it is easier to eat. Use a blender to modify the consistency of food. Foods that are difficult to chew and swallow should be pureed into a pleasingly thickened liquid by blending with water, almond milk, soy milk, plain milk, or soup. Add a healthy range of fish, meat, vegetables, and fruits. Blend a broth soup with vegetables or fruit with milk.

Seek the advice of a nutritionist when considering these guidelines.

Conclusion

No single test provides a definitive diagnosis of Lou Gehrig’s disease. Various tests are available to rule out myasthenia gravis, Lyme disease, and poliomyelitis, to name a few.

Once diagnosed, there are five medications to treat ALS and others to treat the depression, stiffness, sleep issues, and more that accompany ALS. Nutritional support is critical, as patients with ALS tend to lose weight. Therefore they need to seek the services of a nutritionist to recommend safe and nutritious foods to eat.

Key takeaways

ALS is a disease diagnosed by excluding other conditions.

There is no cure for ALS, but there are five medications to treat it.

Patients with ALS lose weight for several reasons and need the guidance of a nutritionist to choose safe and nutritious foods.

Resources:

NINDS. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet.

ALS Association. Understanding ALS.

ALS Clinic. Nutrition Considerations for People with ALS.

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