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Entries in Pseudobulbar Affect (2)

Tuesday
Aug092011

After Brain Surgery, U.K. Gradeschooler Can't Stop Giggling

BananaStock/Thinkstock(NEW YORK) -- Seven-year-old Enna Stephens is facing a daunting 16 months of chemotherapy and radiation after doctor's removed a tumor from her brain, but thanks to a bizarre side effect of the surgery: all she can do is laugh about it.

Enna suffers from pseudobulbar affect (PBA), a neurological disorder brought on by nerve damage that makes it difficult to control one's emotional response. Some patients with PBA cry uncontrollably, others get angry, but for Enna, it has manifested as frequent bouts of the giggles.

While PBA can cause normal reactions, such as a chuckle following a joke, to become exaggerated, the emotional responses sometimes run contrary to the actual emotion the patient is feeling, or have nothing to do with it at all.

"Different patients suffer it in different ways," says Dr. Brian Greenwald, medical director of brain injury rehabilitation at the Mount Sinai Rehabilitation Center in New York City, who did not treat Enna. "Sometimes they'll be hysterically crying but not actually feel upset. Sometimes they are angry but it comes out as laughter. It can be incredibly frustrating to live with because it starts to interfere with one's social and professional life," he says.

In cases of traumatic brain injury, PBA can be a sign of damage to the brain and will often subside with time as the brain heals. For those with degenerative conditions, such as Parkinson's or Alzheimer's, however, PBA tends to worsen over time.

According to Enna's doctors, there is something to smile about: they believe the cancer was completely removed and there is an 80 percent chance it won't return, doctors told the U.K. press.

Copyright 2011 ABC News Radio

Friday
Nov052010

Newly Approved Drug May Help Patients Control Laughing, Crying Outbursts

Photo Courtesy - Getty Images(NEW YORK) -- Last Friday, after more than four years of review, the U.S. Food and Drug Administration approved the first and only therapy designed to improve symptoms of Pseudobulbar Affect (PBA), a neurological disorder that causes involuntary laughter and sudden, uncontrollable crying in patients.

The new medication, Nuedexta from Avanir Pharmaceuticals in Aliso Viejo, Calif., combines the over-the-counter cough suppressant dextromethorphan with quinidine, a generic drug used to restore normal rhythms to erratically beating hearts.

In clinical trials, Nuedexta was safe, reduced the frequency and severity of PBA episodes, and showed a significant advantage over a placebo. But in 2006, the FDA expressed concerns that higher doses of the drug combination raise the risks of dangerous cardiac rhythms. By reducing the doses of quinidine from 30 milligrams to 10 milligrams, Avanir satisfied the FDA's concerns about cardiac risks. In a Phase III clinical trial of the drug with MS patients, half the study participants who got the drug reported no PBA episodes in their last two weeks of the study.

"This is wonderful news for all the patients who suffer from PBA," said Dr. Erik Pioro, a Cleveland Clinic neurologist who specializes in ALS and related disorders. "They will now have an effective, safe, and well-tolerated treatment for this distressing and extremely isolating condition."

In the absence of something better, doctors have treated PBA with off-label prescriptions for antidepressants or levodopa, which boosts levels of the neurotransmitter dopamine. But these have a range of side effects and haven't been subjected to large studies in PBA patients.

Avanir plans to make the drug available in the first quarter of 2011, said CEO Keith Katkin, and will start by providing 30-day samples to select doctors who treat PBA.  Avanir estimates the drug will run $3,000 to $5,000 a year for patients, or about $250 a month.  Patients with limited incomes will get the drug free through a patient assistance program, Katkin said.

Copyright 2010 ABC News Radio







ABC News Radio