Amyotrophic Lateral Sclerosis Care Guidelines

October 2009


The American Academy of Neurology (AAN) released its new guidelines on patient care in amyotrophic lateral sclerosis (ALS) on Oct. 13, 2009. Several MDA-affiliated physicians were involved in their development.

The guidelines are based on evidence that they're effective in the care of people with ALS. The AAN used the language "strong evidence" to mean that more than one high-quality scientific study supports the proposed treatment or test. "Good evidence" means at least one high-quality scientific study or two or more lesser-quality studies support the guideline. "Weak evidence" means there are supportive studies that are deficient in their design.

Strong evidence

The AAN recommends Riluzole, the only drug approved by the U.S. Food and Drug Administration (FDA) to treat ALS. It has a modest effect on slowing the rate at which the disease worsens. Strong evidence shows riluzole can prolong survival.

Good evidence

There is good evidence that the drugs dextromethorphan and quinidine (combined) can lessen the severity and frequency of episodes of uncontrolled laughing or crying that sometimes occur in ALS. The FDA approved the drug Nuedexta in 2010 to treat pseudobulbar affect (PBA) associated with ALS. (PBA occurs when neurologic diseases damage the area of the brain that controls expression of emotion. The damage can trigger episodes of involuntary laughing or crying that are completely unrelated to the person's circumstances or are more exaggerated than the person's actual mood would cause.)

There is good evidence that drooling can be controlled using the drug botulinum toxin type B, injected into the glands near the jaw that make saliva.

Maintaining adequate nutrition is difficult when swallowing function deteriorates severely. There is good evidence that a percutaneous endoscopic gastrostomy (PEG) tube or a radiologically inserted gastrostomy (RIG) tube — commonly called a “feeding tube” — placed in the stomach, stabilizes body weight and prolongs survival.

It's important to undergo tests to detect breathing problems even before they become apparent. If a breathing problem is detected, good evidence shows that a noninvasive ventilation (NIV) device is likely to lengthen survival and slow the rate at which the ability to breathe deteriorates.

Good evidence shows that people with ALS should be screened for possible problems with thinking ability.

There is good evidence that visiting a multidisciplinary ALS clinic (one with many types of health professionals, such as an MDA/ALS center) can help people with ALS get the best possible care. Good evidence also shows that people with ALS who visit a multidisciplinary clinic live longer than those who don't.

Weak evidence

There is weak evidence that suggests that quality of life improves for people with ALS if they attend a multidisciplinary ALS clinic, and if they use a noninvasive ventilation device.

There also is weak evidence that small amounts of radiation therapy may help with drooling.

MDA's role

Neurologist Valerie Cwik, MDA's executive vice president of research and chief medical and scientific officer, was part of the AAN Practice Parameter Task Force that developed the physician guidelines. Several authors of the physician guidelines are directors of MDA/ALS centers throughout the United States and/or have received MDA research support.

For more information

The guidelines for physicians were published Oct. 13, 2009, in the journal Neurology. The physician guidelines also are available on the American Academy of Neurology website (select "Practice Guidelines"). AAN also offers a patient-directed version of these guidelines, written by Cathy Wolf.

Meaning for people with ALS

Physicians in MDA clinics and MDA/ALS centers are likely to be aware of these new AAN guidelines. People with ALS who are not attending one of these clinics or centers may wish to inform their physicians of the new guidelines.

©2009, Muscular Dystrophy Association