Guests were touched when Ken James burst out crying at his surprise birthday party — but grew concerned as he kept crying and crying. “I boo-hooed loud andfor the longest time,” says James, 66, via e-mail from Marshville, N.C. “I couldn’t help it. Surprises aren’t for ALS patients. [Wife] Deloris explained that crying just went with ALS.”
Uncontrolled crying and laughing, at random or out of proportion to the situation, are the hallmarks of pseudobulbar affect (PBA), a symptom that affects 15 percent to 45 percent of people with ALS, especially those with bulbar onset. PBA also occurs in multiple sclerosis, Alzheimer’s and Parkinson’s diseases, brain injury and stroke.
(The drug Nuedexta was approved to treat this symptom in 2010.)
What is PBA?
Although the reason is uncertain, PBA seems to be related to degeneration of motor neuron pathways from the upper brain to the lower (bulbar) brain (perhaps the cerebro-ponto-cerebellar pathway). These pathways, which normally modulate emotional expression to fit the situation, can become “disconnected” as ALS progresses, creating out-of-context emotional outbursts. The term pseudo (false) refers to the fact that the problem isn’t in the bulbar neurons themselves, but in their loss of connection to neurons elsewhere in the brain.
Recent research indicates the problem doesn’t arise from dysfunction in the brain’s prefrontal cortex.
|Debi Faubion (left), news anchor at WSOC-TV in Charlotte, N.C., Deloris James and Ken James at an event honoring Ken called Nine Who Care.
To distinguish PBA from depression or other causes, doctors may administer a questionnaire rating episode frequency, duration, voluntary control and appropriateness to context and inner feelings.
What sets it off?
“It can be a TV program or even a ball game that triggers it,” says James. “Anything that excites me or is sad usually gets a response.”
Other triggers include stressful situations, trying to answer difficult questions, discussions with any emotional context, and sometimes, just thinking. However, people usually don’t feel extremely emotional during a PBA episode.
James’ episodes are short, sometimes just a few seconds long. He laughs more than cries, “which is a plus, because it makes people think I’m happy but in reality both feel the same to me. Crying upsets people more.”
“PBA has a profound effect, depending on its frequency, on both patient and family. It can lead the patient to withdraw from social situations, as they have little control over these events,” says David Simpson, director of MDA clinics at Michigan State University in East Lansing and the Michigan Institute for Neurological Disorders in Farmington Hills.
Simpson usually asks ALS patients if they’ve had any “emotional roller-coastering” and finds many are relieved to know they’re not “losing it.”
In some, antidepressants may reduce symptoms. The new drug candidate Nuedexta has proved safe and effective in clinical trials at reducing episode frequency and severity, without the side effects of antidepressants.
Nuedexta contains the over-the-counter cough suppressant dextromethorphan (DM), which reduces certain neurotransmitter activity (glutamate excitotoxicity) in the brain, and quinidine sulfate, a well-established heart drug that inhibits the body’s rapid metabolism of DM, allowing it to linger in the brain.
James participated in a Nuedexta trial through his MDA/ALS Center at Carolinas Medical Center in Charlotte, N.C., and found it didn’t stop his episodes, but helped some. It also reduced his drooling, a common problem for people with ALS, so he asked for and was granted permission to continue taking the drug once the trial was complete.
Besides medication, simply learning more about PBA makes it more manageable, says Amy Young, a social worker at the MDA/ALS Center at Carolinas Medical Center.
“On follow-up visits, families will say, ‘Mom’s gotten the giggles several times lately, and we so enjoy those moments now, we all just laugh along with her.”
It can help to ignore the behavior and change the subject to something more “emotionally benign,” says Susan Woolley Levine, a neuropsychologist at the Forbes Norris MDA/ALS Center in San Francisco. “Of course, this isn’t what most people intuitively do, which is to hand over the tissues and say comforting things,” she says. Unfortunately, this can prolong episodes.
Taking a break and returning to a subject later may help. Some recommend concentrating on breathing patterns — focusing on breathing in for uncontrolled laughing and breathing out for crying.
“Just let them happen,” advises James. “Don’t let PBA or ALS control your life. Get out often. No need to try to explain PBA to everyone. Just say this goes with ALS.”
See the Avanir Pharmaceuticals Nuedexta site for more information about this drug.
Editor's note (Sept. 23, 2011): This article was updated to reflect new information about Nuedexta. Nuedexta was approved to treat PBA in 2010.