If you started laughing when someone told you a family member died, most people would find it bizarre. But for Dr. Jon Fellus, neurologist and chief medical officer for the International Brain Research Foundation, this is an ongoing problem for people who suffer from pseudobulbar affect (PBA). Also known as involuntary emotional expression disorder, PBA is a neurological disorder that causes random and uncontrollable episodes of crying and/or laughing.
“PBA is a mismatch between what you’re feeling on the inside and what you are showing on the outside, so if the two don’t line up we have a problem,” said Fellus.
First identified by evolutionary theorist, Charles Darwin, 130 years ago, the origins of PBA vary because only people who suffer from neurologic damage—like traumatic brain injury or Alzheimer’s disease—are susceptible to the disorder. However, Fellus believes that PBA is mainly caused by the harm of various neural circuits in the brain stem that are responsible for the brain’s emotional expressions. “It’s a network problem. Your emotions get processed and delivered to the face, but if there are too many potholes along your [neural networks] you lose the part of the brain that asks, ‘Am I really feeling inside what I’m about to show to the outside?’”
According to the Brain Injury Association of America, it's estimated over one million people deal with PBA in North America alone. Despite the growing number of cases, PBA is still under-recognized and undertreated in most medical settings. This is usually a result of misdiagnosis of PBA symptoms with mental illness disorders like depression, bipolar disorder or schizophrenia. To fight this, Fellus and several colleagues developed PRISM (Pseudobulbar Affect Registry Investigating Symptom Management), a questionnaire that identifies the likeliness of having PBA. Still, Fellus believes that pseudobulbar affect is still oft overlooked by both patients and doctors.
We talked with Fellus about the difficulties of diagnosing PBA, the “Don’t Ask, Don’t Tell"-like policies for doctors, and why people think it’s a disorder created by pharmaceutical companies.
VICE: What was your first interaction with PBA?
Dr. Jon Fellus: There was a woman from Brazil who was hit by a bus and she ended up damaging her frontal lobe, which is the front of the brain. Because of the accident she developed PBA which made her scream and cry uncontrollably.
So, the PRISM test uses different screening questions to help PBA, how does it work and what do these screening questions consist of?
Well, it’s a really simple seven question questionnaire called CNS-LS (Center of Neurologic Study Lability Scale) that asks about your control over your emotions. We then measure your score which can range from seven (normal) to 35 (extremely severe). So, if you get a score of 13 on the test, you likely have some degree of PBA. As the number gets higher, the worse your symptoms are. In my whole career, I’ve had maybe three patients that got the highest possible score of 35.
So, does the severity of the condition vary from person to person?
Absolutely. I’ve never seen two cases that were exactly the same. PBA expresses itself differently in every person. Some people only laugh; some people only cry; some people do both. I’ve had a couple patients that have laughed and cried at the same time, which has got to be painful.
It’s horrible. Think about what it’s like to express an emotion, but in the complete opposite way, and completely beyond your control. You pretty much have to take yourself out of almost any kind of social situation.
Is it possible for PBA to go away?
If it’s acquired during the early stages of a stroke or brain trauma, you generally get better and it could disappear completely in a couple of months to two years. However, if you still have it after three years, odds are you’re probably going to have it for good. For other neurological disorders like Parkinson’s, Alzheimer’s, and Multiple Sclerosis, it usually worsens over time until your brain has no more connections left to even create a smile.
Do you need a stimulus or some type of trigger to start laughing or crying?
Yes and no. PBA can be triggered by something happy that makes you cry, something sad that makes you laugh, or it can come out of nowhere. For example, a grown man is watching a puppy dog commercial and starts weeping for no reason—that doesn’t usually happen. The key is knowing that they didn’t behave this way before their disorder. Unfortunately, a lot of these people have families that sort of shrug it off and think it's depression and don’t recognize it as a separate entity. I also think doctors are caught up in this, “Don’t ask, don’t tell” scenario. They don’t ask patients, they don’t screen them and patients don’t come forward to tell their doctors.
Why do you think that’s the case? I would figure if there is an issue biologically or neurologically you would want to fix it.
People will talk to you about their physical problems like, I broke my leg, I can’t close my hands, I can’t lift my arm, but that’s something they can physically see. It’s hard for these people to remember who they used to be. They just assume that this is the way things are now and that they’re going to have to live with it. And doctors don’t get around to asking these questions because it’s a messy area. It’s like if they cry, they must be depressed and should see a psychiatrist. But a psychiatrist might say "well, it’s a neurological issue." It’s like a political football where everyone is passing it off to the other. And it takes time to ask patients what they’re feeling. If more doctors realized there was a simple questionnaire for this condition I’m sure they would be more inclined to just screen for it as we do for other things. As a doctor you have to ask your patients about their five vital signs which is their heart rate, breathing rates, body temperature, blood pressure and pain.
So, what I would like to see as the sixth vital sign that doctors ask their patient for is their emotional temperature. Simply, ask them-—and it’s a really simple question—“Do you ever find that you laugh or cry for no reason or little or no reason,” or “Do you find that you spend time and/or energy trying to control your emotions?” That question really just starts a dialogue and it’s a relief for most of the people I treat that someone is finally asking them about their emotions.
Can you have PBA and clinical depression at the same time?
Absolutely, they can be depressed about having PBA or a chronic disease like Parkinson’s and knowing they might not get better. Patients talk to me about the fact that they feel sad inside but feel like they’re crying too often for no reason. What also makes it tricky is diseases like Parkinson’s or stroke are also known to cause depression. At the same time, treatments for PBA are very effective, so you’ll know in a couple weeks whether PBA was a component of your problems if the crying goes away.
What’s the most severe case you’ve come across?
There was a woman who was injured during an accident after her car was struck from behind. Her friend ended up dying from the collision and when she would talk about her friend in the accident, she would laugh uncontrollably. The woman tried to sue the driver who hit her, but her lawyer ended up dropping the case and settling for a fraction of what it was worth because he couldn’t use her as a witness since she would laugh every time she talked about her friend dying. This woman also had a lot of trouble controlling her anger, would smile and laugh to the point of choking on her food, and laugh in church at the most serious moments. She was also one of the rare cases where she would laugh and cry at the same time.
One other case that I never had the chance to treat was a patient of mine who suffered from brain trauma in a construction work accident. He would laugh nonstop for two hours and so hard they would have to take him to the emergency room.
Is PBA treatable?
Yes, it is. Historically, people used to use antidepressants because crying is generally more common than laughter, and if someone has been crying, you would want to err on the side of caution that it might be depression.
Now, most doctors use Nuedexta, which is made up of dextromethorphan that is also the active ingredient in Robitussin-DM and other cough syrups. It doesn’t matter what the underlying neurological condition that caused PBA is, the treatment is a one size fits all medicine. We prescribe one capsule a day for the first week and then twice a day after that. That woman I described earlier who would laugh when you talked about her friend dying, I gave her a sample and half an hour later she came to me and said it was the first time in eight years that her emotions were under control. However, not everybody responds that quickly, but it’s rare to have a drug that works so well. It just sort of breaks the rule of medicine.
How many patients are you currently treating right now?
I would say I have about 120 patients, give or take. I’ve probably treated 200 patients over the years.
Between the several neurological disorders that are associated with PBA is there one that is more likely to have the condition?
Traumatic Brain Injury seems to be at the top of the list. Right now I’m treating a former NFL player for PBA. His story is already out there in the news about the many concussions he suffered and dealing with issues like crying after getting angry at his daughters because of PBA. Again, things like this happen because your brain can cumulatively hold many concussions that can scramble your circuits until they reach a threshold point where your brain doesn’t keep your emotions in check. And over half of that is from brain trauma.
The responses to the PBA commercial you and your colleagues created seems to be mixed, with some saying that PBA isn’t real, to conspiracy theorists saying it's big pharmaceutical companies making up diseases for money, what are your thoughts on this?
I think for some people the condition doesn’t seem real because it doesn’t make sense to them. They just don’t understand why anyone would want to stop laughing when it’s considered a good thing. But I mean if you look back, Charles Darwin described this condition over 130 years ago, so it’s not a new thing; there’s plenty of high quality research out there. For any doubter of PBA, all they need to do is ask someone who is tormented by it or a condition like it. I mean, I saw my grandfather cry for the first time in my life a year before he was diagnosed with Alzheimer’s Dementia and realized he also had PBA. Is that not real? Those comments are just absurd and laughable and it’s sadly by people who are either ignorant, or have an agenda to say it’s not real.
For more info on PBA check out Fellus’ website: www.pbafacts.com
Special thanks to Tonya Campbell for providing me with additional material for research.