Peripheral Facial Palsy – More Than Just a Flat Expression

 

Facial Palsy

You awaken one morning and discover that you cannot smile, have lost your sense of smell and/or taste. You have difficulty speaking, and your face is drooping. Perhaps you have had nonspecific flu-like symptoms, headaches, fatigue, muscle aches and fevers.

Have you had difficulty concentrating, or are you sensitive to light or sound? Do you feel more irritable than normal or do you have a stiff neck or ear pain or one or more rashes? Those symptoms may have already come and gone, perhaps multiple times, and years ago. Have you had problems with your heart such as an arrhythmia or heart block? And now you suddenly find your face is affected by “something”, or you may have vertigo, difficulty closing one eye, or reduced hearing in the ear on the side of the face that is affected.

That “something” causing a sudden, dramatic change in your face may be the most common neurological symptom associated with Lyme disease, which is peripheral facial palsy (PFP), occurring in approximately 10-30% of patients.1,2

It may also be caused by a number of other factors.

You say a routine national laboratory has tested you for Lyme disease in the past and your results were negative—so you “don’t have Lyme”? Then please test again. If you haven’t been tested before, ask your physician to test you for Lyme disease using a top laboratory such as IGeneX, or Bowen laboratories.

Lyme disease can provide false positives when patients are tested for a number of reasons. The patient’s results can be affected by a course of antibiotics taken prior to testing. Or the Borrelia burgdorferi (Bb) spirochete that causes Lyme disease may have been undetectable in that particular blood sample. Also, the antibody response to Bb is slow, so your body may simply have not made any antibodies, hence they were undetectable. Some Lyme patients never make sufficient antibodies. And last, the test that was used to detect those spirochetes may not have used regional isolates, so it was inaccurate or not sensitive enough to detect Bb.

The relationship between PFP and Lyme disease is clear. In 1998, the FDA approved a vaccine against Lyme disease called “Lymerix” (SmithKline Beecham, PA). The vaccine contained a recombinant outer surface lipoprotein A (OspA) of the Zs7 strain of Borrelia burgdorferi (Bb) spirochete, which was given in 2 double-blind studies with more than 20,000 patients. The results were published in the New England Journal of Medicine at the time.

These early OspA antibodies, which occur after infection and are not exclusive to vaccination, are not easily detectable in the early stages of Lyme disease, especially in humans, although they were detectable in animal studies. In children, PFP was felt to be caused by the direct neural invasion of Bb spirochetes. It was unclear how much impact the vaccine would have on the incidence of Bells palsy, but it was expected to prevent about three quarters of the potential cases of Lyme disease.

Studies by Ikeda et al. that were cited during vaccine studies showed that 32% of cases of Bells palsy were caused by B burgdorferi, and if correct, it was expected that roughly a quarter of the cases of Bells palsy per 100,000 population might be prevented. The vaccine was not approved for use in children under age 15, and was subsequently pulled off the market in 2002 after a flare of controversy over its safety and effectiveness, while Glaxo-Smith Kline cited “insufficient demand.”3

In acute PFP, there is not normally a rash present, but there may be. In the beginning of the acute form of the disorder, simple ear pain, (similar to a mild ear infection), may be the only symptoms present, or perhaps a mild fever or flu symptoms. Over the course of several days, the symptoms will progress into more acute stages.

Facial palsy typically occurs on one side of the face; however, it can rarely present bilaterally (both sides). The external surface of the ear on the affected side may be swollen and/or reddened in appearance. Depending upon nerve involvement, there can also be moderate to severe pain in the face, in or around the ear, and the skin on the face may be painful to touch. Pain may extend from the top surface of the patient’s head on the involved side, through the face, neck, and into the shoulder and back. Facial palsy is typically not painful, though extreme forms having inflammation can cause excruciating pain along affected nerve pathways.

There is evidence that the incidence of Borrelia-associated PFP is much higher than reported, and it is postulated that many cases of what is known as “idiopathic” facial paralysis, (Bells palsy) are actually undiagnosed Lyme borreliosis. In many people, acute PFP is the only symptom of Lyme disease, and a diagnosis of Bells palsy is automatically given by the doctor, instead of reviewing the patient’s clinical history for positive serological Lyme testing, or other hallmark Lyme symptoms, such as a tell-tale “bull’s-eye” rash, or a history of arthralgias (joint pains) and other Lyme-specific symptoms.

PFP in children can be caused by many disorders, including trauma, otis media (ear infection), tumors or growths, a congenital anomaly, or Lyme borreliosis. Viral infections can also cause facial paralysis, such as a reactivation of the varicella-zoster virus (VZV), which is the virus causing “chicken pox” and “shingles”. This virus can also cause in children, what is known as “Ramsay Hunt syndrome”, which includes facial paralysis and zoster (eruptions on a nerve path and inflammation) around the outer ear or in the back of the throat (oropharnyx), and affects the eighth cranial nerve, the peripheral nerve dictating inner ear function. VZV reactivation can also cause PFP in the absence of zoster, which is called zoster sine herpete.

In addition, HSV-1 (Herpes simplex virus type 1), EBV (Epstein-Barr virus), CMV (cytomegalovirus), mumps virus and HHV-6 (human herpes virus type 6) have all been reported to cause acute peripheral facial paralysis in children. In most patients of any age (up to 60%), the cause remains unknown and a diagnosis of “idiopathic” peripheral facial paralysis, or Bells palsy is made.4 If more patients were tested serologically for Lyme disease at the onset of PFP, the number of clinical cases of Lyme disease might well show an increase.

Most people recover from acute PFP, but in approximately 5%, recovery is incomplete and complications occur which include involuntary muscle movements (synkinesis), loss of facial function, or odd or absent sensations.

As a prime example of PFP, whether caused by Lyme borreliosis or a reactivation of the zoster virus (I have had chicken pox as a child); I would like to share with you my personal experience with this aspect of disease. In 2000, 9 weeks before my second marriage, I awoke with a terrible earache. Over the course of 5 days, and as many physicians later, I was diagnosed with an ear infection, cellulitis, tinnitus, unexplained ear pain, and shingles of the cranial nerve.

Unfortunately for me, all of the diagnoses did nothing to alleviate my pain and facial dysfunction on the left side of my face. Cranial nerves I (olfactory), III (oculomotor), V (trigeminal), VII (facial), VIII (Vestibulocochlear), IX (Glossopharyneal), X (vagus), XI (Spinal/Accessory) and XII (Hypoglossal)5 in me were affected. This translates into a very bad, very painful experience affecting sensation of taste, smell, ability to sense or move my facial muscles, shoulder, raise my arm, turn my head in certain directions and it gave me vertigo and double vision. I was unable to blink in the affected eye, and my eyelid drooped and I could not hear well out of the affected ear.

An audiologist’s test noted approximate 20% hearing loss in the affected ear. My outer ear was horribly swollen and red. I used special eye drops during the day to moisten my eye and goopy cream at night and tape to keep my eye shut so that I could sleep.

I could not feel or move anything in a little over one-half of my face. I was acutely aware that the nerves running along the side of my jaw were being slowly squeezed into dysfunction by the swelling in my jaw and cheek. My doctor told me that if these nerves are cut off or pinched long enough, they will die, and due to the severity of my symptoms, he thought I was in real danger of losing full function in my face. In fact, my physician felt that I was one of the worst cases he had seen in his 30+ years of clinical practice.

Eating and drinking became quite a challenge; and I would pinch shut my lips on the left side of my mouth, holding them tightly closed with my left hand, and tilt my head to the right and do my best to eat and drink without spilling. Chewing was no picnic either, and I certainly appreciated that ability more than one can imagine, when it finally returned.

The pain was so remarkably intense that I took my heating pad to work with me and sat at my desk for the better part of several days with the temperature set on the highest setting possible. I held the heating pad up to my face for the bulk of two weeks—anything to ward off the horrible pain I was experiencing that was unaffected by analgesics. At the end of most days I cried because I did not know if I would ever find relief from the pain and I worried also that my face might not return to normal. I did not want to be disfigured for life.

When I laughed, my face took on a hideous form that was embarrassing to me and slightly disturbing to others. Knowing this, I would cover my face whenever I had to speak with someone or whenever I was about to laugh.

“All I want to do is be able to smile at my wedding in two weeks,” I told the hair stylist. I nearly called off the wedding for fear of looking freakish. I exercised my slack facial muscles with my fingers every day, attempting to increase the circulation or nerve signal function if it was even possible, but nothing seemed to make a difference. All that I could do was ride out the inflammation and hope for the best.

I was unprepared for how the general public would receive my facial palsy, but I found out the first time I walked into a retail store. I asked the clerk a question, and as soon as she heard me speak, she made a comment about being unable to understand me, as I was slurring my words. I couldn’t form words properly with my lips and tongue, so my speech was slurred. I had to repeat what I was asking and I noticed that she, along with practically everyone else I would meet over the next 2 months, had a curious habit of averting their eyes when speaking to me. On one occasion, when I and my soon to be mother-in-law walked into a store selling bridal accessories, the clerk took one look at me when I asked her a question, and found it more comfortable to provide my mother-in-law with the answer, which I found rude.

My last physician had prescribed acyclovir and prednisone, and decided that I was suffering from shingles of the cranial nerves. I had done research and while the diagnosis didn’t quite seem to fit, I was glad that he prescribed some kind of medication that would hopefully deal with this serious problem. After 3 days on the medications, I wound up in the emergency room with a severe reaction to the medication, so it had to be halted.

Over 9 weeks, the basic facial palsy resolved, but I was left with reduced hearing ability in my left ear, tinnitus, skin hypersensitivity, and permanent nerve damage in my facial muscles to such a degree that I no longer looked, smiled, or spoke quite the same as I had before the affliction.

Photographs of me smiling are markedly different post-syndrome than prior. It takes a great amount of effort on my part to smile to a degree detectable on photographs. To me, the feeling in my face is not the same as it was before my affliction. I would smile before, and my face would show the appropriate smile. Now however, when I “smile”, little more than a smirk appears on my face, giving me a “mean”, “sad”, or “flat” appearance.

In fact, in a court trial, a social worker testified that my face had a “flat” expression. She was unaware of the fact that my facial expressions were residual effects of facial paralysis and instead, deemed them a marker of my parenting ability. It is interesting to note that psychologists will note in their records that patients with certain mental illness have a “flat” facial expression. I can’t help but wonder if those psychiatric patients are manifesting clear signs of undiagnosed neuropsychiatric Lyme disease, as I know Lyme and other tickborne illnesses have clear psychiatric manifestations.

While most people with PFP won’t experience the bulk of what I have, and while PFP can be caused by other factors, be aware that PFP is a hallmark of stage II Lyme borreliosis, and an important signal that the Lyme spirochete has migrated into the central nervous system. If Lyme has been undetected before, step up your efforts to be retested. Left untreated, borreliosis of the central nervous system manifests cardiac problems, paralysis and cognitive disorders, autonomic dysfunction, strokes, blindness, dementia and other serious problems. If you have Lyme disease, the sooner you get diagnosed and treated, the better your overall outcome.

For other causes of PFP, your physician will help you navigate the waters of this disorder which though embarrassing and perplexing, you can take comfort in the fact that PFP runs a relatively short course and is typically not painful, just annoying. Try to look on the bright side of things and realize that with every challenge there comes a greater lesson to be learned.

In my case, these symptoms were another indication in my clinical history that I indeed had Lyme disease, and should have been tested for it and treated. Though none of my doctors would do that until many years later, I did learn tolerance and patience with this experience, something that we can all use.

 

References

1. John J. Halperin, MD, Nervous System Lyme disease. Infections in Medicine. 17(8):556-560, 2000. Cliggott Publishing, Div of SCP Communications.

2. Oransky I, Saraiya A. The Lyme Disease Vaccine: Implications for Bell Palsy. Infections in Medicine. 2000;17(6):456-457

3. Silverman E. Glaxo withdraws its Lyme disease vaccine Treatment is still safe, but demand too low. 2002. E-News from Natl Vaccine Info Ctr.

4. Furuta Y, Ohtani F, Aizawa H. et al. Varicella-Zoster Virus Reactivation Is an Important Cause of Acute Peripheral Facial Paralysis in Children. Pediatric Infectious Disease Journal. 2005;24(2):97-101.

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