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  Scientists develop treatment designed to treat emotional control disturbances in ALS patients
Posted July 18, 2005 in ALS Research

Copyright 2005 National Public Radio (R)
All Rights Reserved
National Public Radio (NPR)
SHOW: Weekend All Things Considered 8:00 AM EST NPR
July 16, 2005 Saturday
LENGTH: 833 words
ANCHORS: JENNIFER LUDDEN
REPORTERS: JON HAMILTON
JENNIFER LUDDEN, host:

Laughter and tears usually reflect what someone's feeling, but that's not always the case for people whose brains have been affected by disease or stroke. They may laugh or cry uncontrollably even though they're not especially happy or sad. The problem afflicts hundreds of thousands of people in the US. Now scientists say they've developed the first treatment designed specifically for these people. NPR's Jon Hamilton has more.

JON HAMILTON reporting:

A church is an awkward place to start showing inappropriate emotions. Charla LaFountain(ph) learned this the hard way.

Ms. CHARLA LaFOUNTAIN (MS Patient): They would just start a sermon or whatever, and I would just start laughing. Why am I laughing? They're talking about, you know, serious things and I'm laughing.

HAMILTON: She would also burst into tears watching "The Brady Bunch." LaFountain lives in Lewistown, Montana. She's had multiple sclerosis for about 20 years. MS makes it hard for her to get around. But LaFountain says she was devastated when the disease began to make her laugh or cry when she wasn't feeling amused or sad. It was embarrassing and it was interfering with her career as a social worker. So LaFountain says she didn't hesitate when a neurologist asked if she wanted to try an experimental drug.

Ms. LaFOUNTAIN: And I said, yeah, sure, I would try anything, because it's kind of serious that--in my line of work, that I have the right emotions for the right things.

HAMILTON: The drug she tried is called Neurodex, and its history is as odd as the condition it treats. A researcher in San Diego named Richard Alan Smith was trying to find something that would treat ALS, also known as Lou Gehrig's disease. It's a bit like multiple sclerosis in that it attacks the brain and nervous system. Smith runs the non-profit Center for Neurologic Study. He hoped to protect nerve cells from ALS using the active ingredient in cough syrups like Robitussin. That's not as crazy as it sounds. These cough syrups act on nerve cells in the brain that trigger coughing. As it turned out, the approach didn't stop ALS, but Smith began to suspect it was doing something else for ALS patients who took it.

Mr. RICHARD ALLEN SMITH (Center for Neurologic Study): Almost immediately, patients were reporting that their problem with emotional control had completely resolved.

HAMILTON: But Smith says he had trouble believing what patients were saying.

Mr. SMITH: Well, I'm a scientist, so at first I was skeptical, because when you give any therapy for patients, with at least the diseases that I work with, you always get reports back of how much you're helping the patient, and we went back and tested it very carefully and demonstrated that this, indeed, was a legitimate claim.

HAMILTON: The drug, which is called dextromethorphan, appears to act on cells in what's known as the lower brain. That includes the brain stem and part of the cerebellum. Smith says that's surprising, because few scientists suspected this part of the brain was involved in expressing emotion.

Mr. SMITH: It's actually new understanding of how emotional behaviors are actually regulated in the brain. This is somewhat at odds with the traditional view that emotions are centered in cortical areas of the brain, which are roughly referred to as sort of higher centers of the brain.

HAMILTON: Neurodex is being developed by a small company in San Diego called Avanir. This summer, Avanir asked the FDA to approve Neurodex for people whose expressions of emotion don't reflect their real mood. FDA approval is just one hurdle, though. David Hansen of Avanir says another challenge will be to educate doctors about the problem Neurodex is designed to address.

Mr. DAVID HANSEN (Avanir): When a patient comes in and reports to a physician that they're crying and they don't know why, many, many physicians, rightfully so, will automatically assume that the patient could be depressed and they may put them on an anti-depressant.

HAMILTON: That may help, but Hansen says often doctors have missed the true diagnosis. One reason may be that doctors still aren't sure what to call uncontrollable bouts of laughing, crying or even anger caused by brain damage. They've had plenty of time to think of one. The problem first was described by Charles Darwin more than a century ago. Some doctors refer to it as emotional incontinence. Others call it emotional lability. And researchers are more likely to call it by its formal name, pseudobulbar affect.

In any case, Hansen says Neurodex won't succeed unless doctors learn to recognize the condition it treats. Charla LaFountain was one of the lucky ones. Her doctor made the right diagnosis and she says Neurodex has changed her life.

Ms. LaFOUNTAIN: It seemed like it started working right away, and then I just had more control over what I would do, you know. Then I had natural responses to things.

HAMILTON: The FDA is likely to make a decision about Neurodex by the end of the year.

Jon Hamilton, NPR News.

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