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  ALS Patients Test Emotion Drug
Posted September 4, 2003 in ALS Research

Copyright 2003 The Hearst Corporation
The Times Union (Albany, NY)
September 3, 2003 Wednesday THREE STAR EDITION
SECTION: CAPITAL REGION, Pg. B1
LENGTH: 719 words

HEADLINE: Patients hope for emotional rescue;
Albany Medication being tested locally may bring relief for sufferers of malady that causes laughing, crying jags

BYLINE: Claire Hughes; Staff Writer

BODY:
Carole Sue Kelly laughed so hard and so long during a defensive driving course that she disrupted the class and had to leave. When she had trouble disciplining her 12-year-old grandson, she started crying -- and couldn't stop.

"I'm very levelheaded," said Kelly, a 62-year-old resident of West Sand Lake. "This was very out of character for me."

At first, she thought she needed psychiatric help. Then a nurse told her that her sudden, uncontrollable laughing and crying might be a problem called pseudobulbar affect. It afflicts people like Kelly, who has amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, as well as patients with multiple sclerosis (MS), Alzheimer's disease, a history of strokes and other neurological disorders.

A month ago, Kelly began taking an experimental drug intended specifically for pseudobulbar affect. Since then, she feels more like herself, she said.

"I don't cry. I don't laugh. I'm not embarrassed," Kelly said.

Kelly was lucky that someone recognized her symptoms. Pseudobulbar affect often goes undiagnosed, doctors said. Patients may not mention fits of crying or laughing to their doctors, because they don't think the problems are associated with their physical conditions.

"Often somebody will be in our office and you'll witness it yourself," said Schenectady neurologist Dr. Richard Brooks. "And you'll say, 'Gee, how often do you do that?' "

Unfortunately, doctors don't always see an outburst. And sometimes pseudobulbar affect is mistaken for depression -- in part because patients' inappropriate behavior can cause them to withdraw socially rather than face embarrassment.

"The problem is the recognition of it," said Albany neurologist Dr. James P. Wymer of Upstate Clinical Research Center, which is testing the drug Neurodex to treat pseudobulbar affect.

More than 1.5 million Americans are diagnosed with pseudobulbar affect, according to Avanir Pharmaceuticals, which is working toward U.S. Food and Drug Administration approval of Neurodex. The drug company estimates that the problem afflicts 10 percent of multiple sclerosis patients, 50 percent of ALS patients, 40 percent of people with Alzheimer's disease and 11 percent of patients one year after a stroke.

The exact cause of pseudobulbar affect is unknown. Health professionals said it is most common in people whose illnesses involve both sides of their brain. Experts theorize the syndrome is related to injuries in pathways that control emotional expression. It goes by other names, including emotional lability, emotional incontinence and excessive emotionalism.

More than half of the 100 patients at the ALS Center at St. Peter's Hospital suffer from pseudobulbar affect, said Barbara Adams, a case manager there. ALS attacks nerves in the brain and spinal cord that control muscle movement, causing paralysis in later stages. Those patients whose symptoms indicate injury to the bulbar area of the brain -- including problems with speech, swallowing and breathing -- are likely also to suffer from fits of uncontrolled laughing and crying, Adams said.

Neurodex would be the first drug intended specifically for pseudobulbar affect. Currently, doctors do not always treat the syndrome -- especially if the problem bothers family members more than patients, said Dr. Daniel Silverman, medical director at the Center for the Disabled in Albany. When needed, doctors prescribe drugs approved for other purposes. Antidepressants sometimes reduce symptoms, but don't address the real problem -- patients with pseudobulbar affect usually do not say they feel sad when they're crying.

"Having a condition like ALS or MS is bad enough, but then to have this kind of embarrassing problem is like rubbing salt in an open wound," said Dr. Jonathan Cooper, director of St. Peter's ALS Center.


FACTS:SIGNS AND SYMPTOMS Facts about pseudobulbar affect: Characterized by outbursts of sudden, uncontrollable laughing and/or crying.Often afflicts people with amyotrophic lateral sclerosis, multiple sclerosis, Alzheimer's disease or a history of strokes.Symptoms often go undiagnosed or are mistaken for depression.Estimated to afflict more than 1.5 million Americans.Researchers suspect a link to injuries in pathways that control emotional expression.

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