People who have pseudobulbar affect have sudden, frequent, and uncontrollable episodes of laughing or crying. In most cases, the laughing or crying is disproportionate to the humor or sadness of the situation. Sometimes the display of emotions can even seem inappropriate. In people with PBA, there is a disparity between a person’s feelings and the outward emotions they are displaying. Pseudobulbar affect occurs in association with other brain disorders, including amyotrophic lateral sclerosis (ALS), Parkinson’s disease, multiple sclerosis (MS), epilepsy, stroke, traumatic brain injury (TBI), and Alzheimer’s disease. “From the perspective of the person who has pseudobulbar affect, the condition can be stressful and embarrassing,” says Frank Longo, MD, PhD, chief of neurology at Stanford Health Care in Palo Alto, California. Having PBA can mean being misunderstood by your friends and family because your outward laughing or crying doesn’t reflect your emotions, Dr. Longo says. “It’s important to understand the disorder and that the person is not doing anything to contribute to or cause PBA,” says Longo. “People with PBA demonstrate sudden, involuntary emotional outbursts that are either incompatible to a given situation — for example, laughing when told something that is sad — or compatible with the situation at hand but of greater intensity than would be expected, such as laughing uncontrollably when others would typically only smile,” says Brian Lebowitz, PhD, director of neuropsychology training at Stony Brook University Hospital in New York, who specializes in traumatic brain injury. Release Hypothesis In patients with one of the associated neurological disorders, some of the neurons in the frontal lobe that connect to the lower brain region (medulla) that controls laughter or crying are lost or damaged. In this theory, the lesions “release” the laughter and crying center of the brain, per past research. (2) Gate Control Theory By examining the brains of people with MS and PBA, researchers believe that the disorder is a result of the disinhibition of the “gate control” mechanisms that keep our expression of emotion in check. According to this theory, neurologic damage from MS or another brain disease disrupts activity in the parts of the brain related to sensory-motor and emotional processing, noted a review published in November 2017 in U.S. Pharmacist. (3) Dysfunction of Neurotransmitters Theory According to this theory, the neurotransmitters serotonin, dopamine, glutamate, and sigma-1 are disrupted in various brain pathways and cause the emotional expressions to be altered. (3) Because it is found in many neurologic disorders, PBA is often diagnosed by neurologists, says Longo. “Geriatricians and psychiatrists might diagnose the condition as well,” he adds. “PBA is clinical diagnosis, meaning that the clinician is basing this on the history and observation of the patient, versus a test-based diagnosis, such as a blood test or a brain scan,” says Longo. As in the case with many clinical diagnoses, it can be easy to miss unless the doctor has some experience with it or is looking for it, he says. PBA may also be measured on a scale, based on answers to a series of questions. The Center for Neurologic Study-Lability Scale (CNS-LS) is a seven-question, self-administered (meaning the patient answers the questions) questionnaire that asks questions about laughter and crying. There is also a scale called the Pathological Laughter and Crying Scale (PLACS), which is made up of 18 questions and is given by a healthcare professional. (1) Because it’s not uncommon for people with ALS to have PBA, it’s standard practice for Nicholas J. Maragakis, MD, a neurology professor at Johns Hopkins and codirector of the ALS Clinic in Baltimore, to ask his patients questions that could signal a PBA diagnosis. “I ask each of my patients about emotional lability; do you experience easy laughter or easy crying? The next question I ask is, ‘Do these episodes get in your way as you go about your day? Does it get in your way socially or bother you?’ I use that information to some degree to think about potential treatment,” Dr. Maragakis says. “If patients with neurological conditions cry or get more emotional, it’s oftentimes dismissed,” says Maragakis. “PBA is easier to diagnose if the person laughs inappropriately, but in my experience, there tends to be more crying,” he says. The challenge is that patients often think that the symptoms of PBA are a reaction or part of the neurologic disorder, he says. “Someone may say, ‘I cry a lot, but I cry a lot because I have ALS,’ or they’ll say, ‘I’m depressed so I cry a lot.’” With further questioning, Maragakis sometimes uncovers pseudobulbar affect. Since it is a separate phenomenon from the ALS, it needs to be treated differently.
How Is Pseudobulbar Affect Different From Depression?
Pseudobulbar is often mistaken for depression. (4) “There can be a couple reasons PBA can be misdiagnosed,” says Longo. “One is the clinician hasn’t had experience with PBA and is not thinking about it. The nuances and the history might get missed,” he says. Even if the clinician observes some of the features of PBA in the person, they might not be fully appreciated, he adds. “A second reason it’s missed is that PBA can appear similar to depression. A little bit of tearfulness can be passed off as depression, but it was really an episode of pseudobulbar affect,” says Longo. In general, the tearful episodes do seem more common than the laughing episodes, he says. “But PBA isn’t linked to depression,” says Longo. It may often be confused for depression, but that’s not because of a link between the two conditions. One way to determine if the symptoms are PBA or depression is “the company it keeps,” says Longo. “If someone does have one of the neurological disorders that I mentioned, then I think you would be a little more likely to consider whether a person may have pseudobulbar affect,” he says. If someone has absolutely no neurological problems, then PBA would a pretty rare thing to have, he adds. Longo also notes that the length of the episodes can be another way to differentiate between depression and PBA. “A big difference that I’ve seen in my experience is that the pseudobulbar episodes are very brief, maybe a half a minute or a minute or two and then sometimes the tearfulness immediately shifts into laughing,” he says. That briefness and that immediate shift would be very atypical for depression, he says. “Typically, the tearfulness that goes with depression is more prolonged; that can go on for minutes or hours or days, and it would be very atypical to shift into laughing,” he says. “I think the timing and the pattern is different between depression and PBA.”
Prognosis of Pseudobulbar Affect
The long-term outlook for pseudobulbar affect depends on the underlying condition that the person has. Most people diagnosed with PBA have another condition such as Alzheimer’s disease, multiple sclerosis, or Parkinson’s disease. With treatment, some people can reduce the severity and frequency of their episodes of laughing or crying, notes the Cleveland Clinic. (5) “This would involve explaining what PBA is, that it can be common in a number of these neurological disorders, and how we think it works,” says Longo. “It’s not the person’s fault, and they don’t have control over it,” he says. “It’s very helpful to understand PBA, because if you observe it without having seen it before or knowing anything about it, it’s stressful to see,” says Longo. “It’s complicated, because it looks very emotional. The more people know this and understand it, the better,” he says. In certain cases, if the emotional episodes are somewhat mild and not happening a lot, that insight into the condition might be enough for the patient and caregiver to manage the PBA.
Medication Options
“If education is not enough, and the PBA is more severe or frequent, the physician has to judge, ‘Is this affecting quality of life and their relationships with their family and their social life?’ If so, then one could try a medication,” says Longo. If drug intervention is deemed appropriate, the goal of treatment of PBA is to reduce the severity and frequency of the emotional episodes. For many years, PBA was treated with antidepressant drugs, including serotonin reuptake inhibitors (SSRIs) and tricyclic antidepressants. (1) More recently, the U.S. Food and Drug Administration (FDA) approved a drug specifically for pseudobulbar affect — called Nuedexta (dextromethorphan-quinidine) — that can reduce the frequency of the episodes, says Longo. “It has a pretty low side-effect profile, and it’s great that it’s available,” he says.
Alternative and Complementary Therapies
According to the American Stroke Association, there are a few techniques that may help with coping or minimizing an episode of PBA, including the following: (7)
Try distracting yourself by occupying your mind with something unrelated to the present moment or by counting the number of objects on a nearby shelf.Breathe slowly and deeply until you feel in control.Relax your face, shoulders, and other muscle groups that tend to tense up during an episode.Change your body position. Pay attention to your posture when you’re having an episode.When you feel an episode coming on, change your position.
These suggestions aren’t a replacement for medical advice; talk with your doctor about ways to deal with PBA episodes.
Prevention of Pseudobulbar Affect
Although PBA is associated with brain injury and different preexisting neurological conditions, experts aren’t certain why some individuals develop it and others don’t; there isn’t a way to prevent or reduce the risk of developing pseudobulbar affect. There are things that could help reduce the risk of developing one of the underlying conditions of PBA, namely stroke. A person can reduce their risk of stroke by not smoking, treating heart disease or high blood pressure, keeping their blood sugar level within a healthy range, exercising regularly, and staying at a healthy weight, according to the U.S. Department of Health and Human Services. (8) There is evidence that suggests that keeping the heart healthy through exercise and diet may help protect the brain. Two diets that have positive results in many studies include the DASH (Dietary Approaches to Stop Hypertension) diet and the Mediterranean diet, notes the Alzheimer’s Association. (9) “At times, people with PBA appear confused and frustrated about their symptoms and may say that they find themselves laughing or crying ‘all the time,’ sometimes when they aren’t even sure why,” says Dr. Lebowitz. For some people who are dealing with a serious neurological illness, such as a recent stroke or ALS, PBA stands out as a particularly bothersome symptom, says Lebowitz. “At times this is due to feelings of embarrassment associated with the individual’s lack of control over his or her emotional expression. To avoid the embarrassment, some individuals with PBA avoid interactions with others, especially those that they anticipate might be heartfelt or emotional, such as a grandchild’s graduation or a reunion with an old friend,” he says. For these reasons, PBA can keep people away from work, volunteering, or other opportunities for social interaction, further adding to feelings of loneliness and alienation, he says. “The patients I see often dismiss PBA as a secondary event and ascribe it to feelings of sadness or depressed mood,” Maragakis says. A nationwide survey targeted the six neurological disorders in which PBA can develop — ALS, MS, Alzheimer’s disease, stroke, Parkinson’s disease, and traumatic brain injury — to determine the number of people with PBA. Estimates range from 1.8 to 7.1 million in the United States, depending on the diagnostic test used and the scoring scale. (4) A meta-analysis published in March 2016 in Journal of Stroke and Cerebrovascular Diseases that looked just at the prevalence of pseudobulbar affect in people who had had a stroke concluded that PBA affects approximately 1 in 5 stroke survivors soon after the stroke, and 1 in 8 survivors beyond six months after the stroke. (10)
Black and Hispanic Americans and Neurologic Care
A study published June 2017 in Neurology looked at data to identify people with any self-identified neurologic disorder, which included conditions such as Parkinson’s disease, MS, and stroke. (11) Black Americans were nearly 30 percent less likely to see an outpatient neurologist, even after adjusting for income, education, insurance, and health status differences. Hispanic Americans were 40 percent less likely to see an outpatient neurologist. Alzheimer’s Disease and Other Forms of Dementia Alzheimer’s disease is a progressive form of dementia that affects memory, thinking, and behavior. Amyotrophic Lateral Sclerosis (ALS) Also known as Lou Gehrig’s disease, after a baseball player who was diagnosed with ALS in 1939, this is a progressive nervous system disorder that results in loss of muscle control. Epilepsy Epilepsy is a central nervous system disorder in which sudden surges of electrical activity in the brain, called seizures, affect how a person appears or behaves, usually for a matter of seconds to minutes. Multiple Sclerosis (MS) In MS, the immune system attack the central nervous system, causing lesions that disrupt the flow of information between the brain and body. MS causes physical as well as cognitive and psychological symptoms. Parkinson’s Disease Parkinson’s disease is a progressive brain disorder that affects physical movement and can also affect mood, thinking and memory, sleep, and sense of smell. Symptoms include stiffness, slowing of movement, tremors, and balance problems. Stroke A stroke occurs when the blood flow to the brain becomes blocked or when an artery in the brain leaks blood or ruptures. The resulting injury to the brain can cause physical, cognitive, and psychological symptoms. Stroke can also be fatal. Traumatic Brain Injury (TBI) A TBI is when a bump, blow, or jolt to the head or a penetrating head injury disrupts the normal function of the brain. A mild TBI is known as a concussion. PBA Voices Made up of patients, healthcare providers, and advocacy organizations, this group works to help people living with PBA, their doctors, and caregivers by building awareness of the condition. Brain Injury Association of America The mission of the Brain Injury Association of America is to advance awareness, research, treatment, and education and improve the quality of life for all people affected by brain injury. The organization has chapters around the United States to provide direct support and advocacy on a local level. National Multiple Sclerosis Society The Society’s mission is to help people affected by MS live their best lives. The organization works to educate, provide support, fund research, and advocate. American Stroke Association A division of the American Heart Association, the American Stroke Association offers information on stroke warning signs, prevention, and recovery, as well as on the aftereffects of a stroke, including PBA. The ALS Association This mission of The ALS Association is to work to discover treatments and a cure for ALS as well as to advocate and empower people affected by ALS to live their lives to the fullest.
