Navigating the Labyrinth of Traumatic Brain Injury to Discover a Secondary Neurological Condition

By Jonathan Fellus, MD

Working with individuals with traumatic brain injury (TBI) every day, I see the debilitating effects and long-term struggles firsthand. TBIs are complicated and can cause a multitude of other neurological and medical issues, but with appropriate and individualized care, patients can recover and ease back into daily life—slowly but surely.

TBI Defined

According to the Brain Injury Association of America, 1.7 million people sustain a TBI each year. A TBI is caused by a significant force to the head or a penetrating head injury that disrupts normal brain functions. Diagnostically, the severity of a TBI can range from “mild” with a brief change in mental status or consciousness to “severe,” an extended period of unconsciousness or amnesia. However, the consequence of any given TBI depends on many different factors ranging from the individual's genetic susceptibility to the unique areas damaged relative to the person's particular pattern of pre-injury strengths and weaknesses.

Effects of a TBI

As we all know, the brain controls important functions we rely on daily, coordinating our body’s systems, such as breathing, heart rate, body temperature, and metabolism; thought processing; body movements; personality; behavior; and the senses, such as vision, hearing, taste, smell, and touch. When a brain injury occurs, the neurons and nerve tracts that carry messages through the brain can be affected, thereby disrupting and disabling these important daily functions.

The effects of a TBI are dependent on where in the brain and how widespread the injury occurs. The brain is divided into main functional sections, called lobes, including the Frontal, Temporal, Parietal, and Occipital Lobes, along with the Cerebellum and the Brain Stem. Each has unique functions, so depending on which area of the brain is affected, a TBI can change the way a person thinks, acts, feels and moves the body. A TBI may alter other essential internal functions, such as blood pressure, hormonal, or bowel and bladder control. Depending on the severity of the injury, these changes can be temporary or permanent.

Treatment

It’s important to remember the effects of a TBI are complex and vary greatly from person to person, depending on the cause, location and severity. When treating a TBI, the goal is to resolve acute issues relating to basic functional loss, such as respiratory management, sleep/wake cycle, bowel/bladder incontinence, communication and mobility before addressing other secondary issues that may arise. Treating the initial chaos of TBI sequelae or consequences, reminds me of a kaleidoscope—trying to decipher the picture emerging from a complex array of fragmented, moving targets.

As TBI patients evolve through the various stages of recovery, they become more active participants with regards to treatment, often engaging in goal setting with caregivers to enhance quality of life. With greater communication from the patients, clinicians can better detect less obvious facets of the disease and maximize potential for functional recovery. While the brain continues to heal, patients often revert to childlike tendencies and have to relearn how to resist certain impulses and maladaptive physical or interpersonal behaviors.

On the flipside, with this increased participation during later stages of recovery also comes a greater sense of the difficulties that lie ahead. This is a classic time for patients to react impulsively and often inappropriately with an increased risk of depression and suicide. There is a dynamic interaction between cognition, mood and behavior that can be a struggle for patients, caregivers and the interdisciplinary treatment team. Therefore, it’s important for all parties—physical, occupational, speech/language and cognitive therapists, along with neuropsychology-- to work together to help mitigate the complex disability that follows TBI. Brain rehabilitation doctors coordinate these activities, setting priorities and goals while using medications to facilitate recovery or reduce medical and neurological complications that emerge or persist.

A wife of one of my patients compares a TBI to the layers of an onion. As you peel away the different sections and treat each issue, there’s a greater hope of recovery and a sense of “normalcy.”

Neurological Ramifications

Although there are certainly physical components to the puzzle, it’s often the cognitive, behavioral and emotional repercussions that are the most incapacitating, preventing TBI patients from resuming roles in the community over the long term. As the dust settles and immediate medical or neurological issues are addressed, more subtle neurological problems may emerge as patients have a greater ability to discuss how they’re feeling. One such issue comes in the form of a distressing condition called pseudobulbar affect (PBA).

Causes of PBA

PBA is a neurological, not psychiatric, syndrome caused by a TBI and certain other underlying neurologic diseases, such as MS, ALS, Alzheimer’s or Parkinson’s disease and stroke. PBA occurs when the primary neurological condition damages the areas of the brain that control normal emotional expression. This disrupts brain signaling and causes 'short circuits’ in the form of unpredictable episodes of crying or laughing that can be frequent and severe, and may interfere with everyday life. These outbursts are often incongruent with the patient’s current emotional state, leaving them to laugh or cry when they don’t find things funny or sad. Due to the unpredictable nature of PBA, episodes often cause embarrassment for those living with the condition, particularly in social settings. These crying or laughing episodes are often so embarrassing they can interfere with routine activities or cause patients to avoid certain situations altogether. Moreover, PBA sufferers often acknowledge that they spend significant time and energy trying to hold back or simply control their emotions. Finally, PBA also has a negative impact on loved ones and caregivers as they struggle to adjust to these outbursts.

Suspecting and Diagnosing PBA

Due to minimal awareness and knowledge of PBA in the medical community, PBA is often misdiagnosed as depression or part of the primary neurological disease—when in fact, it’s a separate, treatable condition. Nearly two million people may have PBA, but many go undiagnosed. It’s important for physicians to understand the differences between PBA and depression, so patients can receive proper treatment. With a greater understanding among doctors and patients, PBA can be diagnosed more accurately and treated appropriately.

A patient suffering from PBA may show the following tell-tale signs:

• Crying or laughing for no apparent reason
• Laughs or cries at inappropriate times or appears to constantly smile
• Experiences outbursts of crying and/or laughing that are exaggerated or inappropriate for the situation
• Can’t control their laughter or tears, even when they try to, often spending time and energy “holding back”
• Socially isolates themselves out of frustration, fear and humiliation of having an episode in public

Coping with PBA

In addition to talking with a physician, patients can keep track of their episodes in a diary to better understand what triggers PBA symptoms, employ appropriate coping techniques and help the doctor treat the condition appropriately. By taking a more active role in recording these emotional episodes, patients ensure better patient-doctor communication and a more accurate diagnosis.

Here are some general tips to help cope with PBA episodes:

• Be open about it. Let people know that you cannot always control your emotions.
• Distract yourself. If you feel an episode coming on, try to focus on something unrelated.
• Change your body position. Note the posture you take when having an episode. When you think you are about to cry or laugh, change your position.
• Breathe. Inhale and exhale slowly until you are in control.
• Relax. Release the tension in your forehead, shoulders, and other muscle groups that tense up during an emotional episode.

Treating PBA

When PBA persists, interferes with daily activities or fails to respond to the behavioral techniques above, it may be time to seek medical treatment. In October 2010, the U.S. Food & Drug Administration approved the first and only medication to treat PBA called NUEDEXTA® (dextromethorphan hydrobromide and quinidine sulfate) 20mg/10mg capsules, which is thought to act on the areas of the brain responsible for emotional expression. NUEDEXTA is a combination of two well-characterized medicines and is clinically proven to help reduce or even eliminate unpredictable crying or laughing episodes associated with PBA.

Individual results may vary, and it’s important that patients tell their doctor if their PBA episodes continue or they experience bothersome side effects. Patients should periodically reassess the need for treatment as PBA symptoms may sometimes improve on their own. NUEDEXTA is not approved to treat other types of emotional lability. I personally have prescribed NUEDEXTA to many of my TBI patients experiencing PBA symptoms and have seen gratifying results.

Inspiring Patient Story

I work with many TBI patients every day, but one in particular, Richard Anderson, has an inspiring story of recovery and triumph. Richard was at the Jersey shore on family vacation during the summer of 2004 when he was hit by a drunk driver as his family was walking back to their hotel. His wife, Rose, was in the line of fire, but Richard instinctively pulled her out of the way and took the brunt of the collision, leaving him with a TBI and other serious injuries.

Richard’s recovery from his TBI has been a long, arduous journey. Severe physical injuries, coupled with a multitude of additional effects of the accident—like the inability to taste and smell— left Richard unable to continue his job as a New York City public servant and resume his parenting responsibilities for his two daughters. These tasks fell primarily to Rose, and she became the stabilizing anchor of the family. After determining his unpredictable emotional symptoms were separate from depression and anger, I diagnosed Richard with PBA.

Richard’s TBI coupled with PBA has had a tremendous impact on the Anderson family dynamic. After his TBI and onset of PBA symptoms, he has had difficulty maintaining his pre-injury activities and social contacts.

Since Richard started taking NUEDEXTA, his whole family has seen a dramatic decrease in his crying episodes. We were also able to reevaluate his overall treatment regimen for controlling his PBA outbursts. He is still under my care and strives daily to reach his maximum functional potential, one layer of the onion at a time.

Jonathan Fellus, MD, is the Director of Rehabilitation at Meadowlands Hospital Medical Center in Secaucus, New Jersey. Dr. Fellus is a paid consultant to Avanir Pharmaceuticals, Inc.

About Dr Fellus

Dr. Jonathan Fellus, Director of Rehabilitation at Meadowlands Hospital Medical Center, has served on the Board of Directors for the Brain Injury Association of New Jersey, the Physician's Advisory Board of the Epilepsy Foundation of New Jersey, and the American Society of Neurorehabilitation. He has also been appointed to the Governor's panel on brain injury research.

Dr. Fellus specializes in the neurorehabilitation of acquired Brain Injuries, including traumatic and non-traumatic causes. He graduated from Robert Wood Johnson Medical School and completed his neurology residency at Pennsylvania Hospital in Philadelphia, PA. He then completed a Neurorehabilitation Fellowship at University of Maryland Medical System's Kernan Rehabilitation Hospital, with a focus on brain injury rehabilitation.

Voted a Top Doctor in the New York Metro Area by his peers, Dr. Fellus lectures regularly and is considered a media expert in the field. He appears frequently on radio and television shows to offer his expert comments on brain injury cases, many of them high-profile, including Congresswoman Gifford’s recovery. Additionally, he has contributed to publications on neurorehabilitation and brain injury rehabilitation. He supports research in these areas with a particular interest in neuropsychopharmacological management of posttraumatic cognitive, behavioral and mood/emotional disorders as well as promoting motor and functional recovery through the use of pharmacologic agents and other forms of stimulation.

Dr. Fellus has served on the Board of Directors for the Brain Injury Association of New Jersey, the Physician's Advisory Board of the Epilepsy Foundation of New Jersey, and the American Society of Neurorehabilitation. He has also been appointed to the Governor's panel on brain injury research and is a Scientific Advisory Board member of the International Brain Research Foundation.

The Neuroscience Center will have a modern 4-bed Neuro ICU and a 30-bed acute neurorehabilitation unit with outpatient services for stroke and traumatic brain injury, and a new Neuromagnetism Unit will offer the most technologically advanced equipment available, including the Elekta Truix MEG Scanner (one of three in the world), a new 3T GE 750 MRI, and a Hitachi 1.2T open MRI.

NUEDEXTA Important Safety Information

NUEDEXTA is indicated for the treatment of pseudobulbar affect (PBA). PBA occurs secondary to a variety of otherwise unrelated neurological conditions, and is characterized by involuntary, sudden, and frequent episodes of laughing and/or crying. PBA episodes typically occur out of proportion or incongruent to the underlying emotional state.

Studies to support the effectiveness of NUEDEXTA were performed in patients with amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS). NUEDEXTA has not been shown to be safe and effective in other types of emotional lability that can commonly occur, for example, in Alzheimer’s disease and other dementias.

NUEDEXTA (dextromethorphan hydrobromide and quinidine sulfate) 20/10 mg capsules can interact with other medications causing significant changes in blood levels of those medications and/or NUEDEXTA which may lead to serious side effects. Adjust dose or use alternate treatment of the other medication when clinically indicated.

NUEDEXTA is contraindicated in patients concomitantly taking: QT-prolonging drugs metabolized by CYP2D6 (eg, thioridazine and pimozide); monoamine oxidase inhibitors (MAOIs) within the preceding or following 14 days; other drugs containing quinidine, quinine, or mefloquine and in patients with a known hypersensitivity to these drugs or any of NUEDEXTA’s components.

Discontinue use of NUEDEXTA if hepatitis, thrombocytopenia, serotonin syndrome or a hypersensitivity reaction occurs.

NUEDEXTA is contraindicated in patients with certain risk factors for arrhythmia: Prolonged QT interval; congenital long QT syndrome, history suggestive of torsades de pointes; heart failure; complete atrioventricular (AV) block or risk of AV block without an implanted pacemaker.

NUEDEXTA causes dose-dependent QTc prolongation.  When initiating NUEDEXTA in patients at risk for QT prolongation and torsades de pointes, electrocardiographic (ECG) evaluation should be conducted at baseline and 3-4 hours after the first dose. Risk factors include left ventricular hypertrophy or dystrophy or concomitant use of drugs that prolong QT interval or certain CYP3A4 inhibitors.

The most common adverse reactions are diarrhea, dizziness, cough, vomiting, asthenia, peripheral edema, urinary tract infection, influenza, increased gamma-glutamyltransferase, and flatulence. NUEDEXTA may cause dizziness.  Precautions to reduce the risk of falls should be taken, particularly for patients with motor impairment affecting gait or a history of falls.

These are not all the risks from use of NUEDEXTA.  For more information, please click here for the Full Prescribing Information.