Amyotrophic lateral sclerosis — more commonly known as ALS or Lou Gehrig’s disease — is undeniably cruel. It typically strikes adults in the prime of their lives, when they’re raising families or watching their kids’ families grow, working hard at their careers and contributing actively to their communities.

ALS is typically swift ly progressive, a neurodegenerative disease in which motor neurons die, leading to loss of voluntary muscle movement and potentially complete paralysis, according to the ALS Association. There is no cure, and treatments to date have proven to extend lifespans only by months.

It might seem natural, then, to think that ALS patients who become forgetful or start speaking bluntly are simply feeling overwhelmed by their diagnosis. In reality, their actions may be directly infl uenced by the disease itself.

How ALS Affects Cognition

While it’s most commonly discussed in relation to patients’ mobility and muscle control, ALS can also affect cognition and behavior, says Edward Kasarskis, M.D., professor of neurology, University of Kentucky Neurosciences Institute in Lexington, Ky. Kasarskis also works with the ALS Association (alsa.org).

“This is still an evolving issue to know exactly how many patients with ALS are affected and how severely they’re affected,” says Kasarskis. “I think it is for sure that some fraction of the universe of ALS patients have cognitive behavioral planning problems.”

Kasarskis adds that while some studies suggest large percentages of ALS patients experience cognitive impairment, the number severely impacted is much smaller.

“I personally think the ones that are functionally impaired with this probably are three to five percent of all the ALS patients,” Kasarskis says. “I know the literature says that depending on what testing you do, you might find a measurable impairment of some sort on some quantitative test that might (affect) 40 or 50 percent of the ALS patients. That’s one extreme. But it’s the same argument when you say somebody’s got a weakness, but they’re still ambulatory without any assistive devices, as opposed to someone with weakness who’s in a wheelchair. So that comes in a spectrum, and I think the same with this cognitive deal.”

Relationships have been drawn between ALS and Alzheimer’s disease — for instance, last year researchers at the Northwestern University Feinberg School of Medicine identified what they say is the cause of all forms of ALS, and were hopeful that the discovery would be helpful in understanding other neurodegenerative diseases, including Alzheimer’s.

But Kasarskis says when ALS patients are cognitively affected, it “is not a funds of knowledge whack like someone with Alzheimer’s would have.” Instead, ALS clients may experience “more of a frontal lobe behavioral problem. So a lot of patients who have this significant problem will have problems with planning and foresight and will come across as being very impulsive in their actions. In the extreme, these are people who are very impaired.”

Kasarskis gave an example of what such a cognitive impairment could look like: “For one of our patients that came to our clinic — believe it or not, the weakness from ALS was a minor problem. The behavioral problems were the major problem for this woman. For instance, she wanted to go for a walk, which is a perfectly logical thing to do. So she walked on the yellow line on a U.S. highway. That’s what I’m talking about: a lack of foresight, doing impulsive things.”

Presentations from a Seating & Mobility Perspective

How might that impulsivity present itself from a seating & mobility perspective?

Amy Morgan, PT, ATP, is Permobil’s pediatric & standing specialist.

“The frontal lobe is your language filter,” she says. “It allows you to think before you speak. Without that frontal lobe, you speak exactly what you’re thinking without filtering that information.”

Clients experiencing the effects of ALS on their frontal lobes, Morgan added, may therefore come across as being angry or difficult to work with, when in reality, they may lack the ability to speak in more polite, socially acceptable ways.

“Being aware of that helps you not to get upset and frustrated with the situation,” Morgan says.

Kasarskis says ALS patients with cognitive impairment might perseverate — i.e., repeat behaviors for no apparent reason. They can also lack the ability to understand the consequences of their actions.

“This ends up with impaired decision making, which is part and parcel of that,” he says. “Of course, if you don’t see the consequences of your actions, you’re not going to analyze the what-ifs of the situation, and you’re not going to analyze recommendations for medical interventions. You’re not going to weigh the consequences and make an informed decision.”

Patients might also have difficulty following series of instructions, such as those required by a pulmonary function test, which, Kasarskis points out, requires patients to put on nose clips, take a deep breath, empty their lungs, etc.

“They can’t follow through with that,” Kasarskis says. “They can’t sustain their actions when they’re supposed to be breathing out to maximum capacity. So we see that breakdown with the pulmonary function studies. I would think from the other therapists’ standpoint, you would see noncompliance, not following through with recommendations and perhaps not being able to utilize assistive technology that would be there for them to have the benefits.”

During the seating & mobility assessment and in subsequent meetings with clinicians and providers, Morgan says, ALS clients with cognitive impairment may have difficulty remembering the sorts of details necessary to fully take advantage of their complex rehab equipment.

Communication may also be a challenge.

“They honestly and truly believe they told you something, when they never told you,” Morgan says as an example. “And then there’s a big distrust.”

ALS also impacts the client’s ability to speak clearly enough to be understood — another source of potential frustration.

She suggests “making sure that caregivers are around as you’re giving information, sharing information, and receiving information — for accuracy, but also just to make sure that the message is getting across the way you intended it to.”

Finding Seating & Mobility Solutions

Morgan emphasizes that as with any complex rehab technology client, each ALS client presents differently and has highly individualized needs. Therefore, providers and clinicians working with them might find that keeping their options open is a good strategy.

“If the person is having trouble remembering how to make their tilt work versus their recline, that’s where the use of icons — very clear images, not just the words — can help,” Morgan says. “Possibly using auditory cues: Many of the communications speech devices may be able to do that as well.”

Clients with ALS typically find it more and more difficult to operate their power chair and powered seat functions. A compact light-throw joystick, Morgan points out, “still gives them proportional control, but it’s not as difficult to move. It’s got a shorter throw and requires less force.” Such a driving option can also be helpful for clients who would otherwise have to learn a new driving system once they could no longer use a regular joystick. “The beauty of that is you can start with (the light-throw joystick) at their hand when they’re strong. A lot of times, clients with ALS retain the capability to use their feet longer, so you can move the joystick to the footplate very easily and allow it to be a foot proportional control without having to buy anything else — maybe just a different mount.”

As the ALS progresses, Morgan adds, “They can use their chin with the same joystick, just with a different mounting system. I think it’s really nice to have options not only within your electronics and your programming, but to be able to utilize the same control in different areas as they progress and change.”

In fact, the adjustability and positioning prowess of a complex rehab power chair can be a huge benefit to ALS clients as they progress.

“Most clients that I’ve experienced, at a certain point in the diagnosis, prefer to sleep in their chair for that reason,” Morgan says. “Another thing is they can’t tolerate being in a more supine position; they want to be more upright. So because they’re able to control their chair, they end up sleeping in their chair as opposed to being in their bed.”

The Power of Good Communications

It’s critical during the entire process, Morgan says, for providers and clinicians to get as clear a picture as possible of what the ALS client is currently experiencing…which can be a challenge, given how swiftly the disease can progress.

“Once a client with ALS figures (something) out, chances are they’re going to have to figure something new out,” Morgan acknowledges. During assessments and follow-up visits, she speaks to the clients, but is also mindful of how their caregivers respond.

“I’m always aware of the non-verbals of the caregivers,” she says. “I usually ask the individual: ‘Are you having any issues paying attention? Are you finding that you’re getting distracted?’ And (the caregiver) will be shaking her head yes, even though she’s not saying anything. I like to direct it to the consumer and then allow the family to interject. Because one thing that can be off ensive to people who use chairs especially is when you talk to their family and not to them. So I’m a big advocate of addressing them, but letting anyone else chime in.”

While ALS currently has no cure, Kasarskis says some medications can help with cognitive-impairment symptoms.

“We very often will use some of the dementia medications: Aricept and that group of drugs. Sometimes, impulsive behaviors might be modulated with Seroquel. Sometimes patients will have accompanying pseudobulbar laughing and crying behaviors, and there’s an effective medication for that. I think it is hit or miss with the types of therapies that are put to these types of patients in the sense that if they work, that’s great. If it doesn’t work, you move onto other things.

A lot of these patients are in their late 60s or 70s, and they might have intolerance to some of the medications, for example.”

Both Kasarskis and Morgan focused on the reality that ALS presentations are unique to each client — and so are any interventions, whether pharmaceutical or technological in nature.

“It is individualizing the therapy, hoping something will work,” Kasarskis says. “But there’s not a coherent pathway that is almost guaranteed to work. You take the problem as it presents itself, and see if you can work through a solution. Sometimes you’re successful, and sometimes it’s a stand-off and you’re not.”

“You should be always considering the social, emotional, cognitive piece of each person that you’re treating and how they’re responding to their diagnosis and their disease or their injury,” Morgan says. “There’s always this screening, if you will, of their cognitive/emotional state. The whole picture has to be explored to provide the best equipment for that person.

“I think it’s a really holistic approach, just trying to be sympathetic, putting yourself in their shoes about what they’re just starting to learn about themselves and what’s going to happen to them. Different people are going to respond to that very differently. So you have to be aware of how this person is taking all of this in.”