Facial Pain - A 21st Century Guide - For People with Trigeminal Neuropathic Pain

World experts in the diagnosis, treatment, and management of Trigeminal Neuralgia contribute their expertise to cover the full breadth of knowledge in the field, including the following topics:

The dental dilemma
Do I have the right diagnosis?
How to find the best doctor
What is the best imaging study to get?
What medication is best?
The surgical solutions
What to do when nothing seems to help

This guidebook also covers complementary and alternative medicine considerations, including:
Weather and its effects on pain
Acupuncture
Medical marijuana
Upper cervical chiropractic

Also included is the latest information on the future of care for Trigeminal Neuropathic pain.

If you are newly diagnosed, misdiagnosed, in need of another solution, a friend or family member, young or old, or doing well but wish to know more about why, then this book has been written for you.

• Language: English
• Publisher: Facial Pain Association
• Release date: Oct 5, 2020
• ISBN: 9781953596031

Book preview

Biographies

Foreword

Jeffrey A. Brown

Chair of the Medical Advisory Board of the FPA

I can help you.

My dedication to that simple phrase over a career lifetime has led to the publication of this guidebook. When I speak those four words aloud to a new patient, I know what to expect—tears of relief.

This is because the road to good health in this disease called tic douloureux is too often long and tortuous.

It was more than 300 years ago that Nicolas André gave that catchy name to the electric shocks of trigeminal neuropathic pain (now known today and referred to in this book as trigeminal neuralgia or TN). But, nearly 200 years later, French neurosurgeon Henri Marie René Leriche was still writing of tic douloureux that it is one of the most terrible maladies that we have to deal with … not because it is a fatal disease … It is because it persists for so long. He told the tale of a nun who had suffered for 15 years with the tic. She had said to him, If you had refused to operate on me, I would have thrown myself into the Rhone immediately after I left you.

This small book is our effort to toss only the thought of such a thing as far into the wide river as one’s strength will allow.

This guidebook is a collection of articles, written in recent years, for the Facial Pain Association (FPA) Quarterly by members of our Medical Advisory Board (MAB), the Board of Directors, the Young Patients Committee (YPC), and invited experts in the psychology of chronic pain. It is not definitive, as this field is continually in flux, but it is our best effort at an updated compendium of essential knowledge regarding facial pain directed towards patients and their caregivers. By using a publish-on-demand model of distribution, it is possible to continually improve on what is provided, much in the way your phone software updates. The publishing model of the past is truly outdated, as is the information contained in these hardbound dinosaurs that may be elegantly typeset, beautifully illustrated, and printed on vellum pages designed to survive decades—perhaps centuries—of turning. The speed of scientific revolution is in turbo drive and can no longer await this format any more than can the daily publication of newspapers by the sliding of lead blocks of type onto a Linotype machine.

We have made an effort to make this volume into something that communicates and does not obfuscate. Too much of medicine is incomprehensible to laypeople because of a tradition of a kind of Latinization of terms. The words anesthesia dolorosa, for example, are catchy, but also indefinably confusing. They make for interesting history, but difficult modern medicine. Thus, we are flooded with recently generated descriptives such as TN1, TN2, atypical TN, atypical facial pain, secondary TN, SUNCT, posttraumatic trigeminal neuropathy, and cephalalgia. It will not be possible to sort one’s way through all these terms because they describe overlapping entities.

Our compendium is an effort to provide you with the knowledge to choose the best path towards healing. That choice needs to be guided by rational thought that is ideally supported by scientific evidence. For many choices, however, there is insufficient evidence to solidify an answer. There are different paths towards similar results. The choice between them can only be made by means of a dialog between a physician knowledgeable and experienced with the intricacies of the field and a patient who has been appropriately educated. This collection is not intended as a substitute for that dialog. It is intended as a contribution towards the appropriate education of you, the patient suffering from facial pain.

We focus on different elements of the problem. Some essays are concerned with diagnosis and the many terms that are used. Some diagnostic terms are broadly inclusive, others not. There is still expert disagreement on the terminology. Do not become bogged down in this discussion. There is not yet resolution. Understand that there is not universal understanding of what constitutes trigeminal neuropathic pain, as I would prefer to call it.

Other articles focus on descriptions of the differing surgical treatments available. Approaches such as radiofrequency, glycerol, and balloon compression rhizotomy are reviewed. Is one best? No. There is no one size fits all. There are also other ways to injure the trigeminal nerve and stop the pain. There is Gamma Knife and CyberKnife radiosurgery and, of course, Dr. Jannetta’s discovery of microvascular decompression (MVD). These approaches are described, risks specific to each reviewed, and benefits for one or the other compared.

Some chapters deal with the psychologic implications of chronic pain—coping mechanisms for the patient and support members—others on whether one is truly young or truly elderly (though these terms themselves are indefinable).

Finally, we ask what could be locked in the crystal ball containing the secrets of the future for the treatment of this entity. If only we could get just one brief glimpse into that orb.

And, should these words be read many years from now by one doing as I have done (because I’d been told by my mentor, Peter Jannetta, to begin my study by looking backward) in a time when the struggle to understand tic douloureux is long past and all the answers to its conundrums are well known, let me quote the words of the poet Wallace Stevens:

Children picking up our bones

Will never know that these were once

As quick as foxes on the hill

I dedicate this tome to that time in the undefined future when a cure for what ails each of you reading this now will be so obvious as to relegate the hard-fought knowledge quoted herein to well-deserved obscurity.

P

reface

Anne B. Ciemnecki

Secretary of the Board of Directors of the FPA

Most people with TN have a distinct memory of their first encounter with the pain. One woman told me that her first shock was in the shower. She was so astounded by the jolt from out of the blue that she called an electrician to be sure all the electrical lines in their bathroom were grounded properly. Others can clearly recall who they were with or what they were eating when they had their first shock. I have a clear memory of my first encounter too, but it is probably not what you are thinking.

I was about 13 years old, and I was sitting in the backseat of the family car. Yes, there was only one car in most families in 1965. Ours was a bright white Chevrolet Impala with red interior. The fins over the trunk were large enough to land a jet plane. It was dark and cold, and I was curled up feeling bad for my mother. She had just had her second tooth extraction in a few days. She initially went to the dentist in pain, and he removed what he thought was the offending tooth. When the pain continued, he pulled another, assuming he extracted the wrong one or that there were two bad teeth. In the car, her head wrapped in a kerchief, my mother began to cry. It was at that moment she realized that she did not have a toothache. She had the same condition that afflicted her mother.

We got home, and my grandmother cried, too. I did not understand what was wrong, but I knew it was bad. Anything that would have my mother, PTA Mom, Girl Scout Cookie Mom, and bastion of strength for my family, in tears must be devastating. And devastating it was. The pain was unbearable, and many days she retreated to bed. She weighed less than 100 pounds when healthy, and discomfort eating caused her to drop more weight. She never explained what was going on. Frankly, I am not sure she had a clear picture. And she tended to shield her children from the unpleasant.

It was 20 years after the tooth extractions before she was referred to neurosurgeon Allan L. Gardner, MD, who sat with my family for hours explaining what was causing this scourge of pain and options to treat it. He was kind, compassionate, and knowledgeable. He recommended a rhizotomy (not one of those new-fangled microvascular decompressions from a doctor in Pittsburgh). She had a rhizotomy and woke up pain free with manageable numbness. When the pain moved to the other side of her face, she had a second procedure with equally good results. Years later, when the pain returned to the right side in a different area of her face, he managed her medications, all the while reassuring me that TN was not hereditary and that I had nothing to worry about. That was the only thing he got wrong.

In 1993, I had tooth pain and the dentist said the root was infected. Not suspecting that anything else was wrong, I had a root canal procedure. But I now recognize that the pain that led me to the root canal may have been the dawning of my TN. Two MVDs, one Gamma Knife surgery, myriad medications, and 20 years later, I found my way to the FPA Board of Directors. Today, I have the honor to edit this book with Jeffrey Brown, MD. It is an opportunity for me to be what Dr. Gardner represented to my family.

Enough about me. Now about you and this book. This book offers the most up-to-date information available about TN and other trigeminal neuropathic pain. The FPA undertook a major effort to assemble chapters by a broad range of experts in diagnosing and treating TN pain so that you have in this book, not just one point of view, but advice from experts in many aspects of your life with TN. In addition to chapters about medicines and surgeries, you will find here chapters on medical marijuana, complementary and alternative medicine, and managing your life and relationships outside of a doctor’s office. The digital production of this book enables the FPA to update it when new information becomes available so that this book continues to be your go-to source of information.

Think of the book as a Hop-On Hop-Off tour bus in a city you are touring. You have an all-day pass, and you can start at the beginning of the route or any place you want along the way. You can ride around and look at everything superficially, or get off, explore, and hop on again. You do not have to see attractions or read chapters in a particular order.

The chapters are arranged in the order that a person first experiencing trigeminal nerve–related facial pain might read them, from finding a diagnosis; to medical, surgical, complementary, and alternative treatments; to living well with neuropathic facial pain. If you are an experienced patient who needs an MRI, start at Chapter 1.4. If you have wondered about medical marijuana, read Section 6. If you are a spouse or caregiver, check out Chapter 9.3. We hope you find everything you need. If you do not, contact the FPA. This is a dynamic work, and we plan to add new information regularly.

The editors have tried to adhere to a few linguistic principles without changing the words of the chapters’ authors. We hope when you speak of your pain, you follow them too:

Put people before pain. You are not a facial pain patient; you are a person with facial pain.

Use patient only when speaking of a relationship with a doctor. You are so much more than a patient.

Use the word pain and any of its synonyms, but do not use the word suffer or call someone with facial pain a sufferer or victim. Positive language is supportive.

Do not classify pain into Type 1, Type 2, etc. Labels like that may not matter and can hinder treatment. It is all neuropathic facial pain or trigeminal neuropathic facial pain.

Avoid the term suicide disease. It is not helpful, and it is not true. The FPA and this book are dedicated to helping you live a fulfilling and happy life even while you manage your pain.

This book was made possible by the generosity of people who volunteer their time and treasure to the FPA. If this book is your guide, the FPA is your tour director. The FPA is a nonprofit, 501(c)(3) volunteer organization, founded in 1990 by a team of people who were profoundly grateful for recovery from years of disabling trigeminal neuropathic pain. The organization now assists thousands of others around the world. The FPA has emerged as the world’s leading resource for information and health care guidance for all people affected by neuropathic facial pain. That includes patients, their loved ones, and healthcare professionals’ efforts.

To see all that the FPA offers, visit https://fpa-support.org. Can you volunteer your time by talking to someone else who is where you once were? Do you have a skill you can contribute? Can you donate dollars? No amount is too big or too small. Your donations enable the FPA to fulfill its mission.

Read the book in good health, and accept my sincere wishes for love, peace, and no pain.

Chapter 1.0

What is This Pain? Get an

Accurate Diagnosis

The Office of Rare Diseases (ORD) of the National Institutes of Health (NIH) considers trigeminal neuralgia (TN) a rare disease (https://www.rightdiagnosis.com/medical/rare_disease.htm). This means that TN affects fewer than 200,000 people in the United States at any time. (This is known as prevalence.) The incidence of TN, or how many people are newly diagnosed each year, is thought to be 4.3 people per 100,000.

Practically, this means that many people, including doctors, are not familiar with it. The American Medical Association (AMA) estimates that primary care physicians might expect to encounter TN two to four times over the course of a 35-year career. Doctors and dentists have limited medical training on the topic. If you think your doctor does not know much about neuropathic facial pain, you are right. How do you find a healthcare provider who is familiar with TN and other trigeminal neuropathic facial pain?

Begin by looking at doctors who are on the Medical Advisory Board of the Facial Pain Association (FPA). If they are too far away, they may be able to perform a telemedicine consult or recommend someone closer to you.

Check the FPA (https://fpa-support.org/) list of doctors. The doctors on the list have shown interest in the FPA through donations or service such as conference presentations or visits to support groups.

Join one of the TN support groups on Facebook and ask members which doctors they see.

Call a support group leader or peer mentor, also listed on the FPA website, for a recommendation.

Search the provider list on the American Academy of Orofacial Pain (AAOP) website (https://aaop.org/). The AAOP is a subspecialty of dentists who focus on causes of orofacial pain, neurovascular pain disorders, and headache. (Note that four of the seven authors in Section 1 are orofacial pain dentists.)

Contact a university or tertiary medical center in your area. These centers tend to see patients with complex conditions and have the latest technology and treatments.

You may need to travel to find the expert you need.

Answering the question, What is this pain? can be frustrating for patients and doctors alike. Your expectations need to be realistic. A proper diagnosis could take a while. It is time well spent. You cannot treat the condition until you know what it is.

Donald R. Nixdorf, DDS and Flavia P. Kapos, DDS open Section 1 with a chapter that describes a comprehensive orofacial pain examination from the point of view of an orofacial pain dentist. They assert that you must, Find the target before shooting it. In Chapter 1.2, Cynthia Ezell writes about how to find the perfect doctor and the importance of a second opinion. In Chapter 1.3, Steven Graff-Radford, DDS and Bahareh Safaie, DDS present a comprehensive guide to the diagnosis, evaluation, and management of facial neuropathic pain and discuss nonneuropathic conditions that must be considered in a differential diagnosis, such as myofascial pain, temporomandibular joint disorders (TMDs), dental diseases, cracked teeth (with exposed nerve roots), infections such as sinusitis, and migraine headache. Most facial pain is not neuropathic, so a systematic approach to its diagnosis is essential.

In Chapter 1.4, Jeffrey A. Brown, MD discusses the importance of an appropriate magnetic resonance imaging (MRI) scan in diagnosis and treatment and the recommended MRI protocols. Mark E. Linskey, MD concludes Section 1 with myths that can confound your diagnosis in Chapter 1.5.

1.1

How to Prepare When Seeking a Diagnosis

Donald R. Nixdorf, DDS and Flavia P. Kapos, DDS

Editors’ note: This chapter has been written to outline the components of a comprehensive orofacial pain evaluation; to empower you as a patient (and your loved ones) to participate in this diagnostic process more adeptly. Please keep in mind that different healthcare specialties have different scopes of practice and may define a complete evaluation differently.

Importance of a good diagnosis: Find the target before shooting it. The underlying reason for someone’s pain is best described by his or her diagnosis, which is a name given to a certain disorder or disease process. Ideally, the diagnosis should give us information about what is going on, which would consequently derive a conclusion on how to address it. It is important to keep in mind, though, that there are often multiple factors playing a role in a single condition. Elements that serve to bring on the problem, called initiating factors, may not stay and therefore may not be observable over time. The illness, however, may persist due to other influences, called perpetuating factors, or even worsen due to aggravating factors. Note that these might have been present before the onset of the problem without major issues, making it hard for the person to identify them as potentially negative, but they could be harmful in this new context of dysfunction.

A simple example is walking on a sprained ankle. A different event may have caused the injury, and walking may have never been a struggle, but under those circumstances, the affected joint may need to be guarded from the activity, or otherwise the lesion could be made worse or be prolonged. This complex puzzle needs to be carefully studied so the pieces fit together and hopefully lead to a resolution or adequate management. To make the investigation even more challenging, pain conditions in the trigeminal innervation territory often present referral patterns, meaning that the location where the pain is felt is not the actual origin of the painful symptoms. Thus, treating the site of the referral will not address the true source of the problem and will likely be ineffective.

A key part of the diagnostic process is to unveil these potentially misleading clues and identify proper targets for treatment. For example, TN pain may be perceived as a toothache (since the innervation of the teeth is part of the trigeminal distribution), but dental care will not be helpful and may even make things worse. When there is a classic presentation of TN as well as a concomitant continuous background pain, it would be hasty to assume that the latter is caused by TN without first considering a range of differential diagnoses, especially after a good response to treatment by the classic component of the pain. As an analogy, when a person has a sprained ankle, they may develop a limp to avoid putting weight on the injured limb when walking. If this becomes a habit, the other structures being overloaded in that process, such as back, hip, and knees, may become painful because of the imbalanced use.

The cause for these pains is separate and different, though related. Making a parallel back to TN, patients with intense pain in their face frequently develop regional muscle guarding, tightening the muscles in the affected area as a natural response and making them sore as a result. Treatment directed at the TN, in the presence of an uncontrolled daily constant jaw muscle pain, unfortunately may not change the muscular component if the behavior related to its development has not changed. Even though multiple symptoms are most likely explained by a single process, there is no rule that there must be only one problem occurring at a time. Furthermore, misapplied procedures increase the risk of the condition becoming chronic, because the pain remains unsatisfactorily managed longer, and negative thoughts such as worry, fear, and anger set in, becoming new contributing factors. An orofacial pain evaluation should consist of the following steps, in order of importance:

1. History taking

2. Physical examination

3. Obtaining diagnostic testing, such as imaging or laboratory tests, as needed

History: Getting to a differential diagnosis. The most important step in the diagnostic process is the history taking, which leads the clinician to develop an initial differential diagnosis. In other words, by talking to the patient and collecting the right information, the provider narrows down the list of all the possible conditions to a few more likely ones. For example, knowing the patient’s age and sex already provide a good pointer toward conditions that may be frequent in that specific demographic group. Signs and symptoms teach us about the underlying condition and its nature. When preparing for a medical appointment, be ready to answer many questions about the characteristics of the pain and the context in which it presents. Your answers probably hold the most valuable diagnostic information. An astute clinician is not only interested in what you are saying, but also how you are saying it. For patients seeking care for pain, the pain itself will be called the chief complaint, and the details of its history are often referred to by providers as history of present illness, including:

Location: Where is the pain felt? Is it always in the same place? Is it in a small spot or does it affect a broader region? Is there a spreading or movement component to it? Is it easily localized, or is it more diffuse? Does it feel superficial, on the surface of the skin, or deep, in the bone?

Onset: When did it first start, and under what circumstances? Were there any initiating factors? Was it a sudden or gradual onset?

Progression: Has it changed since the onset? Is it getting better, getting worse, or staying the same? Is it always the same when it occurs, or does it change?

Frequency: Is it constant or intermittent? How often does it occur?

Duration: How long does it stay when it is there?

Timing: Is there a time of the day when it is usually worse? Does it present on the clock? Are there periods of remission, times when you feel nothing?

Quality: What does the pain feel like? Which words could be used to describe it (e.g., sharp, dull, throbbing, shooting, electric shock–like, burning, etc.)? Is there an emotional component?

Intensity: Is it mild, moderate, or severe? How would it be rated on a scale of 0 to 10, with 0 being no pain and 10 the worst pain possible?

Interference: Does it disrupt sleep? Does it affect the ability to perform daily activities?

Aggravating/alleviating factors: What makes it better, and what makes it worse? Is it affected by physical activity, light, noises, jaw movements, body position, temperature, touch, sneezing/coughing, etc.? Does it respond to over-the-counter analgesics or other medications?

Associated features: Are there other signs or symptoms present before, during, or after the pain? Are there any appearance changes, such as redness, swelling, tearing, or sweating? Is it predictable? Can you feel or sense it coming on? Are there other changes in vision, movements, or sensation?

Prior treatments/tests: Were other providers consulted? What therapies have been tried, and what were the results? Is there any prior imaging, labs, etc., and what were the findings? What medications have you taken, at what dose, for how long, and what were the effects?

The pain itself is where most of the patient’s attention will tend to focus; however, it is important to think of the whole person and the conditions in which the problem is presenting. The history can also uncover contributing factors that can help to explain the origin of the pain as well as to guide the therapy. Fundamental pieces of the puzzle are also frequently found thinking outside of the box:

Medical history: Are there any other medical conditions? How are they being addressed? Are there any medications, vitamins, or supplements being taken? At what doses and for how long have they been taken? Has there been any trauma? Are there any other pains throughout the body?

Family history: Are there other cases of similar problems in the family? Are there any cases of autoimmune disorders, cancer, or pain disorders?

Habits: General exercise, diet, water, tobacco, caffeine?

Oral parafunctional habits: Teeth clenching and grinding; biting objects, fingernails, lips, cheeks; gum chewing, etc.?

Sleep: What is the typical sleeping routine? How long? Is it restorative? Is there difficulty falling or staying asleep? What is the sleeping position?

Psychosocial history: What is the patient’s occupation, marital status, family dynamics? What is the level of psychosocial stress? Does the patient have a support system, coping strategies? Are there diagnoses of anxiety, depression, or other mood disorders, and how are they being addressed? Have there been traumatic life events? Is there ongoing litigation?

Physical exam: Refining the list. After the history taking, the clinician should have generated a mental list of the possible conditions that could be going on. The physical examination will serve to confirm or refute such hypotheses and guide the process of diagnosis. The trained professional may be able to gather further information beyond what is volunteered by the patient, starting from general appearance, affect, posture, gait, speech, and nonverbal communication. One of the major goals of the physical exam is to duplicate the chief complaint to better understand its origin and response to stimuli. For instance, dental pain of pulpal origin is expected to be elicited or changed in a predictable manner by application of cold to the surface of the tooth; therefore, a dentist uses a cold test and is trained to interpret the response as within normal limits or altered.

To further illustrate this concept, in order to render the diagnosis of temporomandibular disorders (TMDs), there should be a positive finding of familiar tenderness to palpation or range of motion of the involved muscles and/or temporomandibular joints (TMJs) on examination. Since pain is a very personal and subjective experience, even during the exam, part of the findings will be subjective and dependent on patients’ reports. If, at any point during the exam, pain is provoked, it is of crucial importance to discuss how the provoked pain is the same or different than what is typically experienced. The regional head and neck exam should include visual inspection and palpation of the masticatory and cervical muscles, TMJs, face, thyroid gland, lymph nodes, teeth, mouth, and oropharyngeal mucosa. Changes in symmetry, shape, size, consistency, color, and texture should be noted. Range of motion of the head and neck should be evaluated for limitations and coordination, as well as associated pain or noises. Cranial nerve screening evaluation is also valuable to evaluate motor and sensory functions of the major nerves supplying the face and neck. The teeth and supporting tissues should also be inspected