From the Clinic

In the past few years, clinicians and researchers have come to realize that a number of patients—perhaps close to 50 percent—have changes in thinking or behavior that come with ALS. The changes don’t stem from fatigue or lowered oxygen levels common later on. They can be subtle or pretty

“You usually know quickly if patients have these problems,” says Jennifer Heidler. “You get a feel before testing starts.”

obvious. And they involve the frontotemporal parts of the brain—areas that control the so-called executive functions of planning, organizing and insight as well as language and emotion. Memory, fortunately, is spared.

Jennifer Heidler, a clinical psychologist skilled in measuring cognitive abilities, often evaluates patients from the Hopkins ALS clinic. Recently, she attended the first international workshop on the topic, held in Canada.

What’s the most common problem here?

It’s a slowing in speech, a difficulty in finding the right words.

Are there others?

People can become agitated or irritable or generally be more emotional. Sometimes appetite changes or unusual eating habits crop up, like a new-found need to suck on candy. Patients sometimes withdraw or don’t feel like doing anything, even while they’re able.

Isn’t that last bit depression?

Not always. Speakers at the workshop said major depression isn’t particularly common in ALS, but that perhaps a quarter of patients have milder forms. Some large studies, they said, show that depression isn’t causing the typical changes in thought and behavior, even though symptoms of depression and frontotemporal injury can overlap.

One piece of research—it needs duplicating—caught my eye. It suggests some ALS patients don’t register threatening situations well. Their insight is less and they’re less troubled by the illness than you’d expect. My own mother was serene when she had ALS; perhaps something like that was at work.

So if you’re a caregiver and suspect a problem?

Get patients an evaluation that includes an MRI and neuropsychological testing. And caregivers should take part. Our common inventory has them rate their patients for negative behavior or a lack of inhibition. Do they make rude jokes? Shun friends? Caregivers see things we can’t.

Does knowing brain changes are possible do any good?

We know it relieves caregivers of guilt. The pages they write on the inventory reveal there’s pent-up emotion. And knowing that mental effects typically advance slowly, behind ALS’s usual problems, may be a comfort. Meanwhile, drugs can improve frontotemporal symptoms.

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