Pain in ALS: What the Research Shows

by Amy Madsen on Mon, 2013-07-01 09:00

ALS researcher Adriano Chiò discusses what’s been learned about why ALS hurts

Article Highlights:
  • New research shows that pain is common in all stages of ALS, but that it often goes unreported and untreated.
  • More studies are needed to help scientists and clinicians understand pain in ALS and to determine the best ways to treat it.
  • This article is part of a package that focuses on pain in ALS. Be sure to read the second part of the series titled 11 Tips for Dealing with Pain in ALS, which features ALS nurse Mary Beth Geise's strategies for preventing and relieving pain.

It used to be said that pain isn't associated with amyotrophic lateral sclerosis (ALS).

Used to.

Although typically not considered a primary consequence of ALS, researchers and clinicians are realizing that pain does play a role for people with the disease.

In fact, in a pair of recent studies evaluating pain in amyotrophic lateral sclerosis (ALS), researchers found that:

  • pain is common in ALS, occurring significantly more frequently in people with ALS than in the general population;
  • pain is frequent in all stages of ALS;
  • the intensity of pain in ALS is negatively correlated with functional status (greater pain is associated with less functional ability);
  • pain in ALS often is under-recognized and undertreated; and
  • studies are needed to clarify the mechanisms of pain in ALS and determine the most effective treatment strategies.
Adriano Chiò, associate professor of neurology at the University of Turin in Italy

In June 2013, MDA spoke with Adriano Chiò, an associate professor of neurology at the University of Turin in Italy who has studied pain in ALS.

Q: Why was there such a long-standing misperception that pain isn't associated with ALS?

A: Historically, Jean-Martin Charcot, who was the first to describe amyotrophic lateral sclerosis, stated that patients with ALS had no pain or sensory symptoms. This statement created a sort of blindness toward pain that lasted for at least one century. Only in more recent years has the awareness of the presence of pain in ALS increased, up to the inclusion of pain treatment in more recent guidelines from the American Academy of Neurology and the European Federation of Neurological Societies.

Q: Is pain in ALS ever a primary consequence of the disease, or is it always a secondary symptom?

A: Pain is usually a secondary symptom, related to reduced mobility, abnormal stresses on the musculoskeletal system caused by weak musculature, or difficulties in the range of motion of a joint — also known as an articular block. However, pain related to spasticity and muscle spasms can be considered a primary consequence of ALS.  

Q: What would you estimate is the percentage of people with ALS who experience pain?

A: Probably not less than 50 percent, with a progressive increase in frequency with disease progression.

Q: What are some of the common types and locations of pain that people with ALS experience?

A: There are several types of pain in ALS. Cramps are a typical type of pain, sometimes very severe and localized at the lower extremities, and less frequently at the hands, and the abdomen, and even at the tongue. Articular pain is more often localized at the shoulders. Musculoskeletal pain is also quite common, and occurs in the back, legs, arms, shoulder and neck. It is related to damage to bones, tendons, ligaments and joints. Pain related to spasticity, although less common than in other diseases such as multiple sclerosis, can be present in lower limbs, particularly during walking.

Q: How is pain assessed or measured?

A: A person's experience of pain is subjective and cannot be directly measured. One way of assessing it is with a visual analog scale (VAS), which is used in questionnaires such as those that measure pain intensity. When responding to a VAS item, respondents specify their level of agreement to a statement by indicating a position along a continuous line between two endpoints — an example of one such statement may be, "This is a scale indicating the intensity of your pain from 0 to 100. Please show me on the scale how bad your pain is."

MDA provides services and support through 120 local offices in the United States and Puerto Rico.

MDA's medical clinics nationwide, including specialized MDA/ALS research and clinical care centers, are staffed by top health professionals skilled in the diagnosis and medical management of muscle disease, including ALS. Services may include occupational, physical and respiratory therapy; diagnostic tests; genetic counseling; and more.

Local MDA support groups offer emotional and practical support to individuals affected by ALS, their families and caregivers. MDA also accepts donations of gently used durable medical equipment (such as wheelchairs, communication devices, patient lifts, hospital beds and more), repairs these items as needed, and provides them to individuals with muscle disease at no charge to enhance mobility, independence and quality of life.

Visit the MDA website to learn more about the MDA office in your area, as well as the nearest MDA/ALS research and clinical center.

Q: Is pain in ALS associated only with late-stage disease, or do some people experience it in the early or middle stages?

A: With the exception of muscle cramps, or fasciculations, which occur early in the ALS disease course, pain is related to more advanced stages of the disease.

Q: Is pain in ALS associated with a particular type of onset (bulbar or limb)?

A: Pain is more common in patients with limb involvement; its occurrence is usually less frequent and intense in patients with bulbar onset.

Q: Is pain in ALS associated with gender or age?

A: There are no indications that pain is related to gender in ALS. It is also likely that it is not related to age, when the presence of pain related to other disorders is excluded.

Q: Are there any known associations between pain and ALS caused by particular mutations (for example, SOD1, TDP43, FUS or C9ORF72)?

A: There have been some cases that suggest carrying an SOD1 mutation may be characterized by prominent pain, but there are no indications that pain is specifically associated with other ALS-related mutations.

Q: Is pain associated more or less with ALS-FTD, than it is with ALS alone?

A: There are no indications that pain is more frequent in patients with ALS-FTD versus pure motor ALS.

Q: Are there things people with ALS can do on their own to try to prevent or relieve pain?

A: Pain related to joint problems can be prevented with active and passive physical therapy. Massages can be useful for the treatment of cramps and pain related to spasticity. Maintaining good posture is very import both in pre-empting and relieving pain.

Q: What treatments are available for people with ALS who are experiencing pain?

A: Treatment of pain in ALS is not different from the treatment of pain in other disorders, and is based on physical therapy, NSAIDs and opioid-based drugs. However, it should be highlighted that there have been no studies specifically devoted to the assessment of different pain therapies in ALS. In my opinion, such studies are urgently needed.

A: Should someone with ALS who is experiencing pain consult with their neurologist, or should they seek help instead from a pain specialist, physical therapist or other medical professional?

Q: Usually neurologists have the knowledge and skills to effectively treat pain in ALS patients. In people with refractory pain, also known as "intractable pain" or pain that is resistant to conventional pain therapies, a consultation with a pain specialist is mandatory.

The first step in pain treatment is to identify the problem. This means asking patients and caregivers about the presence of pain. In those who cannot speak, questions should be asked using augmentative, alternative communication devices. The site, the timing and the intensity of the pain should be investigated, using drawings showing the body and pain diaries containing a record of the time, type and quality of pain that is experienced.

Q: Are there negative consequences associated with ignoring pain?

A: The consequences of ignoring pain are extremely negative for the quality of life of patients. They can include depression, anxiety and negative attitudes toward prolonging life. Pain reduces the capacity of the patient to participate in the family and social life.

More information about pain in ALS

To learn more, read:

Neurologist Teepu SiddiqueNeurologist Teepu Siddique, director of the Division of Neuromuscular Medicine at Northwestern University Feinberg School of Medicine in Chicago and founding director of the MDA clinic at that institution, stresses that the most important concern about pain in ALS is a lack of awareness. Physicians need to ask their patients about it, he says, and patients need to ask their doctors.

Siddique, a recipient of multiple MDA research grants, notes that ALS is not defined by pain — that is, pain is not a defining feature of the disease — which can cause it to be easily overlooked. In addition, people with ALS often are "stoic," he says, unwilling to talk about their pain to others.

But the experience of pain "comes out in questioning," he says. That's why the initial assessment at every doctor visit should include not only the recording of weight, blood pressure, current medications and symptoms, but also patient and caregiver responses to questions designed to determine whether pain is a factor.

For those experiencing it, ignoring pain should never be an option as doing so can have profound negative effects on a person's physical, mental and emotional state. People with ALS aren't immune to other conditions, Siddique notes, so although a particular pain may seem benign or tolerable and no cause for worry, it's important to determine what's causing it and what can be done to alleviate it. Doing so can have a huge positive impact on a person's quality of life.

Amy Madsen
Your rating: None Average: 4.7 (12 votes)
MDA cannot respond to questions asked in the comments field. For help with questions, contact your local MDA office or clinic or email See comment policy