Bringing laughing, crying spells under control

As far back as 1872, British naturalist Charles Darwin observed that people with brain injuries or illnesses were sometimes stricken with uncontrollable, and often inappropriate, outbursts of anger, laughter or grief. “Certain brain diseases ... have a special tendency to induce weeping,” he wrote.

Even in modern times, there have been no specific treatments for the mysterious problem, now called “pseudobulbar affect.”

The condition occurs in people who have had strokes or brain injuries or who suffer from neurodegenerative diseases such as multiple sclerosis, Alzheimer’s disease, Lou Gehrig’s disease and Parkinson’s disease. The causes remain unknown, but more than 130 years after the condition’s discovery, sufferers finally may have a medication to treat it.

A new drug, Neurodex, has been shown to reduce emotional outbursts in people with Lou Gehrig’s disease (amyotrophic lateral sclerosis).

It’s now being tested on MS patients at 53 centers nationwide and on people with brain injuries and neurodegenerative disorders, says the drug’s manufacturer, Avanir Pharmaceuticals of San Diego.

“It usually takes a long time for a drug to get from the lab to patients,” said Dr. Richard Alan Smith, director of the Scripps Center for Neurologic Study in La Jolla. “But this has moved along at a pretty rapid pace.”

The drug’s effects on emotional disturbances was discovered by accident about 10 years ago.

Smith was studying Lou Gehrig’s disease, which attacks nerve cells in the brain and spinal cord, and had begun working with dextromethorphan, the active ingredient in cough syrups. He thought dextromethorphan might help stop the attacks on nerve cells by blocking a chemical in the brain called glutamate.

After altering the drug so that it would remain in the body longer, Smith gave it to patients. The drug didn’t stop the disease process, but patients quickly reported feeling more emotional stability while on the medication.

They have long needed such help. The uncontrollable outbursts of laughing or crying that are hallmarks of the disorder -- as well as displays of anger and frustration -- can make family relationships difficult, sidetracking rehabilitation efforts.

“Pseudobulbar affect can be very debilitating for patients going back out into the community,” said Dr. Richard Zorowitz, an associate professor of physical medicine and rehabilitation at the University of Pennsylvania. “Patients find themselves in very embarrassing situations, and it might cause them to be more withdrawn.”

Emotional stability sometimes can make a difference in whether a patient is cared for at home or in a nursing facility, Smith said.

“If your loved one is taking care of you and you’re crying with the slightest provocation, it is really tough for caretakers,” he said. “Family members often think they’re not doing a good job taking care of the patient. They think the patient is depressed. We’ve been sweeping this condition under the rug for a long time and not appreciating its consequences fully.”

Doctors don’t know why the drug works or even what is happening in the brains of people with pseudobulbar affect. Although emotional control originates in the higher centers of the brain, a disruption in brain function may be occurring in lower regions of the brain, such as the cerebellum, which is primarily responsible for motor control.

“The lower centers of the brain are involved in regulating traffic to the brain,” Smith said. “It looks like the same part of the brain that is controlling motor control is coordinating some of what is happening in the higher centers of the brain.”

Neurodex appears to have few side effects. So far, dizziness and fatigue are the most common complaints about the drug.

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Pseudobulbar affect

* Characterized by uncontrollable emotional outbursts, such as laughing or crying, that may be inappropriate or unrelated to a situation. Such outbursts are not a true indication of the person’s emotional state.

* Once diagnosed, usually a lifelong disorder.

* Diagnosed in about 1 million Americans.

* Occurs in some people with brain injuries or neurological disorders, including about 50% of those with amyotrophic lateral sclerosis, 40% of those with Alzheimer’s disease, 11% of people within one year of a stroke and 10% of those with multiple sclerosis.

Source: Avanir Pharmaceuticals