Amyotrophic Lateral Sclerosis (ALS): Treatment Overview

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Treatment Overview

Although there is no cure for amyotrophic lateral sclerosis (ALS), treatment can help you maintain strength and independence, manage symptoms, and avoid complications for as long as possible. Treatment also focuses on providing emotional support as your disability increases.

Physical therapists and occupational therapists can help you maintain strength and function and make the most of your remaining abilities. Speech therapists can help you maintain your ability to communicate after speaking problems begin.

Medicines may be used to relieve symptoms and make you more comfortable. These include:

• Glycopyrrolate (Robinul), benztropine (Cogentin), transdermal hyoscine (Scopolamine), atropine, trihexyphenidyl hydrochloride, or amitriptyline (Elavil) for extra saliva or drooling. These medicines may be helpful if you have trouble with the muscles that control swallowing.
• Amitriptyline (Elavil) or fluvoxamine (Luvox) to help with uncontrollable emotions and mood swings (emotional lability).
• Baclofen (Lioresal), tizanidine (Zanaflex), dantrolene (Dantrium), or benzodiazepines such as diazepam (Valium), to relieve muscle stiffness, spasms, and twitching.
• Quinine, phenytoin sodium (Dilantin), benzodiazepines, or gabapentin to relieve muscle cramps.
• Antidepressant medicines, to help with depression, sleeplessness, poor appetite, or fatigue.
• Pain relievers, to reduce muscle pain, which sometimes develops late in the course of the disease.
• Morphine, to help with breathing problems (dyspnea). Antianxiety medicines such as diazepam or lorazepam may be helpful in relieving anxiety caused by breathing problems. The American Academy of Neurology also considers acupuncture a useful option for breathing problems when used in addition to medicine or breathing devices.³

If you are having problems getting enough food or taking medicine, a tube can be inserted through the skin into the stomach (percutaneous endoscopic gastrostomy, or PEG). The tube provides an easier way to get adequate nutrition and take medicines.

Breathing devices may be needed as the muscles of the chest become affected. Initially, extra oxygen can be given through a nasal cannula, a flexible plastic tube that is placed in the nostrils. For people who have mild breathing problems, nonsurgical forms of ventilation may be used. These include bilevel positive airway pressure (BiPAP) and noninvasive positive pressure ventilation (NPPV), which deliver air or extra oxygen through a face mask. But in some cases, an opening in the neck leading to the windpipe (tracheostomy) may have to be created and oxygen delivered through a tube in the opening.

At first, breathing devices may be needed only part of the time, such as when you are sleeping. As the disease and breathing problems get worse, you may need to be on a breathing machine all the time.

Riluzole

A medicine called riluzole (Rilutek) may prolong survival by about 2 months.⁴ It is not known exactly how the medicine works, but it may slow the release of certain brain chemicals (neurotransmitters) that are believed to play a role in ALS. Riluzole is the only medicine approved for the treatment of ALS.

There are some disadvantages to taking riluzole. Although it has been shown to prolong survival for about 2 months, it does not improve symptoms or quality of life in ways that people with ALS, their caregivers, or their doctors have been able to detect. Most people tolerate riluzole very well, but it can cause side effects, including nausea, vomiting, weakness, dizziness, and coughing. Because riluzole can cause liver problems, people taking the drug need to have their liver checked on a regular basis. Treatment with riluzole is also expensive, and it may not help some people.

Scientists are testing many other medicines to see if they work for people with ALS. An updated list of clinical trials to test these drugs in people with ALS can be found on the ALS Association Web site (www.alsa.org) or at www.clinicaltrials.gov.

Last updated:	August 14, 2008
Author:	Monica Rhodes
Reviewed By:	E. Gregory Thompson, MD - Internal Medicine, Colin Chalk, MD, CM, FRCPC - Neurology
Editors:	Kathleen M. Ariss, MS, Pat Truman, MATC