Hemifacial Spasm and treatments

Dr Jaydev Panchwagh: This disease is actually called Hemifacial Spasm. I call it the ‘winking disease’. That surgery is possible to cure hemifacial spasm is not widely known.

Interviewer

: You described this disease as ‘winking disease’, but it seems to be a rather serious one.

Dr Jaydev Panchwagh

: Yes! It is a serious disease, but in a different sense. I will tell you why. Let us first understand what the disease is, then we will get an idea why it is serious.

These are some patient experiences of hemifacial spasm:

Patient 1:

 I am free of Hemifacial spasms after the MVD operation, and I am no longer conscious of my hemifacial spasm, after the operation.

Five years after surgery, my life has changed completely.

Patient 2

:  I have had this disease for 5 to 7 years now. I was told that there is no treatment for it. But when I came in contact with Dr Jaydev Panchwagh,I immediately took the decision and got operated by Dr Jaydev Panchwagh. Now I am completely cured and confident. I would certainly say that this is like a new lease of life for me!

Interviewer: Hello and welcome to the public awareness programme on Brain and Spine(neurosurgery) related diseases and problems. Some diseases affect us with internal changes, while some others manifest their symptoms externally. One such disease that is very visible externally is Hemifacial spasm. The main symptom or sign of this disease is uncontrolled winking of one eye or cheek or one half of the face. It may look funny to the beholder, but for the person who suffers from this disease, it is painful, embarrassing and irritating.

What is Hemifacial spasm and what are the symptoms of this disease? How do we diagnose this, what are the available treatments and how do we choose the right one? All these questions will be answered today by renowned Neurosurgeon Dr Jaydev Panchwagh from Pune, Maharashtra, India.

Namaste Doctor!

Dr Jaydev Panchwagh: Hello

Interviewer: Dr Panchwagh, recently you had written about Hemifacial Spasm in a newspaper and there was a lot of discussion among the readers and public about it.

Dr Jaydev Panchwagh: True! Because I had given it the title of ‘winking disease’ which created a lot of curiosity and was discussed a lot! Actually this disease is called Hemifacial Spasm, this sounds fanciful and rare. If you call it the ‘winking disease’, people immediately understand it and know what I am talking about.

I decided to call this so, because one of the most frequent complaints from those who suffer from it is involuntary winking. This causes them great distress when in public and has occasionally got them in trouble.

Interviewer: That’s true. ‘Winking Disease’ may be a simple name, but it seems to have serious socio-psychological implications.

Dr Jaydev Panchwagh: Yes, of course. It is a serious condition, but in a different way. Let us first understand what the disease is all about.

Let me explain what the word Hemifacial spasm means. Hemi means half, facial means related to the face, and spasm means repeated and uncontrolled contraction of the muscles. In this disease, patients suffer from contraction of one side of the face, which they are not able to control. So when such afflicted persons begin a conversation, contraction of one side of the face occurs, which results in winking of the eye on that side of the face. It may appear to the observer that the person is winking their eye! Initially, the eye and only a part of the face contracts involuntarily, and as the disease progresses the entire half of the face may get pulled to one side. Generally, these symptoms initially begin when the patient is stressed or is in a conversation. Later, it occurs randomly, at any time.

For example, a student who has gone for an interview and is under stress to perform well could manifest these symptoms of Hemifacial spasm. Or a secretary in a company dealing with a new client could have enhanced stress levels which trigger the hemifacial spasm.

In fact, any occasion which causes stress to a person where they become conscious of their facial expressions like social interaction could trigger Hemifacial spasms.

This is a socially troublesome disease. The patient feels very awkward talking to others and tries to avoid face-to-face interaction. They start avoiding social contacts and their professional life may also suffer. Others may not understand why the patient speaks peculiarly. Over a period of time, the patient becomes self-conscious and starts avoiding social interactions. This may affect his personality as well, and there is a danger of losing one’s job due to this.

Let me tell you about a patient who came to me recently. She was a secretary in a Mumbai-based company. and she suffered from Hemifacial Spasms. This lady had a great sense of humour. She had learned to live with the disease without much frustration. After her surgery, she said to me, “Doctor, let me tell you…just imagine….I am a secretary, I am sitting at my table, and  a well-dressed visitor comes up to me and asks that he wants to meet Mr Roy…..and in reply if I winked at him while pointing in the direction of Mr Roy’s cabin, it would create such a wrong impression of me! The guest would be puzzled why I was winking at him while taking him to see Mr Roy” She covered her frustration with a smile.

So this is how embarrassing it could get. As the disease progresses and the frequency of spasms also increase, it no longer holds just a cosmetic/social significance. The patient is unable to speak well because of the twitches, sometimes they are unable to read books or watch TV or work on the computer.

Interviewer: Doctor, what causes this disease?

Dr Jaydev Panchwagh:  The facial nerve controls the movements of our facial muscles as well as our facial expressions. If the facial nerve gets irritated due to some reason, or is compressed either continuously or in a pulsatile manner, then it fires repeatedly sending signals to the muscles to contract. After all, what is a nerve? It can be compared to an electrical wire which carries the current of command/ control signals from the brain to the muscles. So, when the facial nerve conducts information from the brain, it makes the muscles of the face contract. If the facial nerve is provoked to fire repeatedly and unwarrantedly, due to whatever reason, the facial muscles on the same side begin to twitch on their own and the patient has no control over it.

In a tense or stressful situation, or during a social interaction, the nerve triggers signals and the facial twitching gets exacerbated. Though the main cause is likely to be a blood vessel that compresses the nerve, occasionally there could be a tumour which causes compression and symptoms of hemifacial spasm. This is not common…I would say about 3 or 4 cases out of 100. Different studies would give different numbers, but this is a generalisation. 96-97 % of Hemifacial spasm is likely to be caused by a blood vessel compressing the facial nerve at its origin, near the brain stem.

If you want to know why this disease Hemifacial Spasm is a potentially serious disease, I can show you a patient interview:

In this you can see why and how the spasms become a nuisance during speech. You can also see from the second clip that an extrovert faces intense problems because of being home-bound. This patient was a villager who loved talking to people and being extremely social. When she became afflicted with Hemifacial spasm, she could not bear to meet people and talk to them, she preferred remaining indoors and not interact with relatives or friends. For her, life became unbearable and lost all meaning. This is the same patient one month after surgery. She says that she has stopped having the twitching, and is cured of her hemifacial spasm, and that she has regained her confidence. In another interview that she gave five years after her Hemifacial spasm surgery, she describes her pre-operative ordeal “My face would get pulled to one side, I used to feel heavy in the head and I could not hear well. I consulted a lot of doctors over the years, but no one offered me a permanent cure. I was overcome by frustration…I could not go to social gatherings or functions, I could not meet guests at home….I had to hide the left side of my face with my hand so that nobody could see the twitches. These episodes occurred very frequently, every 1 or 2 minutes. I had winking of the eye, my face and cheek were pulled to the left, and inability to hear well. I was so depressed that I had decided to end my life. As a last resort, I met Dr Jayadev Panchawagh and asked him if he could offer me a permanent cure from Hemifacial spasm. He said ‘Yes, of course!’. I underwent an MRI and was operated in February 2010, ten years after I began suffering from this horrible disease. I have become my happy old self, I can mingle with my friends and relatives, participate in social functions, I can hear well, I can speak well. It is now five years after my operation and I have absolutely no problems.

Dr Jaydev Panchwagh: Very few people, including doctors are aware of the fact that this disease called hemifacial spasm can be cured by a surgery called Microvascular Decompression or MVD. This disease becomes obvious to people very soon both to the patient and to the observer.

There was a pujari (one who offers prayers in the temple, a priest) who suffered from Hemifacial spasm. He asked me ‘ Can you imagine what I must be going through when I get these spasms while chanting prayers in front of devotees?’

Interviewer: Doctor, what do patients normally do when the disease manifests itself?

Dr Jaydev Panchwagh: Initially patients fail to understand what’s happening to them, some think there’s a skin problem, some think that they have a dry eye or have a foreign body in their eye….there is lack of awareness of this disease among the public and even the General Practitioners…that it is called Hemifacial Spasm, that it can lead to serious repercussions and that it has a cure by Microvascular Decompression surgery. So, people suffer and bear this trauma for many years. Patients can lose out on the prime years of their life by suffering unnecessarily. Opting for surgery is a personal choice, once they are offered a solution. I think creating public awareness about this disease and its surgical treatment is of utmost importance.

Initially the spasms are less intense and more focused around the eye muscles and are less frequent. As the compression on the facial nerve progresses, the nerves ‘weaken’ because of constant pulsation. Going back to the analogy of a nerve being like an electrical wire or cable, the nerve fibres are very close to each other like wires in an electric cable. Individual wires are separated from each other by insulation. The equivalent of insulation in the nerve is called myelin sheath. In hemifacial spasm, when there is compression by the blood vessel, the myelin sheath is lost or damaged. This causes the individual wires or nerve fibres to talk to each other, called ‘cross-talk’. This is like a short-circuit in the electric wire caused by lack of insulation. The longer the compression exists, the greater is the loss of myelin and greater is the cross-talk. The symptoms worsen, the disease progresses and the recovery become more unpredictable. Typically it takes about 6 to 7 years for the symptoms to worsen and involve one half of the face.

Interviewer: How do you diagnose Hemifacial Spasm? What investigations need to be done?

Dr Jaydev Panchwagh: For diagnosis of Hemifacial spasms, an MRI is required.  In neurosurgery, the symptoms are distant from the organ of disease, unlike other organs of the body. For example: The pain in the back of thigh caused by sciatica is actually a symptom of a problem of compression of nerve roots at the level of the spinal cord, A symptom of imbalance while walking could be because of a problem in the cervical spine (neck), there is contraction of the facial muscles in hemifacial spasm…and the cause is a problem in the brain. This is one of the reasons that patients do not readily co-relate their symptoms with the organ that is diseased. Whereas in the pain of a heart attack, the diagnosis may easily be recognized by the patient or their relative.

Many a time, when patient is told that that they have Hemifacial spasm or Trigeminal neuralgia, they feel that they need to get an MRI of the face done! Instead, a brain MRI is actually necessary. This actually helps in studying the facial nerve in very small MRI slices to find out in great detail about the nerve and its relation to surrounding blood vessels. This will help to determine whether one or more blood vessels is compressing it. It also helps to diagnose if there is a tumour causing the compression etc. Special sequences in the MRI give us all the information we want, and getting the MRI done in specialised centres that give us this specific information is of utmost importance. This is necessary before we plan surgery.

Interviewer: After the diagnosis and investigations, we need to discuss treatment.

Dr Jaydev Panchwagh: If the patient desires cure from Hemifacial spasm, then surgery is the only option. A Micro-Vascular Decompression or MVD surgery is the surgery for Hemifacial spasm.

Interviewer: How is the surgery done? Which part of the brain is actually operated upon?

Dr Jaydev Panchwagh: Medically speaking, the facial nerve lies in an area called the cerebello-pontine angle after it exits from the brain. This area is approached by a key-hole opening in the skull behind the ear of the affected side. This surgery, like the one for Trigeminal Neuralgia is also called Micro Vascular Decompression or MVD. The nerve that is decompressed here is the facial nerve, whereas in Trigeminal neuralgia, it is the Trigeminal Nerve. Micro refers to the microscope being used during surgery, vascular refers to the blood vessel causing the compression and decompression refers to removal of that compression. So for Trigeminal Neuralgia, MVD is done on the Trigeminal nerve and for Hemifacial spasm, it is done on the Facial nerve.

Let me show you a surgical video of the MVD surgery.  This is done using one of the world’s finest neuro-microscopes, which is why the nerve and vessel are seen so magnified and in such fantastic resolution and detail. This is very crucial for the success of this operation. It’s not about the magnification alone, the Xenon light source used in the microscope lights up all intricate details of the brain anatomy. You van see in this MVD video that the facial Nerve is compressed by the blood vessel from behind, and the pulsations of the artery cause the hemifacial spasm in this case. One has to be very careful while handling the vessel because they supply extremely important parts of the brain. We have now inserted a Teflon sponge between the nerve and vessel. The sponge is made of an inert material (does not react with surrounding tissues), and remains there, cushioning the nerve and protecting it from the pulsations of the blood vessel. This is the crux of the operation…operating through a key-hole and decompressing the facial nerve using an inert Teflon sponge.

Interviewer: Does the Teflon sponge move after insertion? Can it get misplaced later, after surgery?

Dr Jaydev panchwagh: No, the sponge does not move. It remains where it was inserted. There are just 4 or 5 sutures or staples on the skin. The incision is inside the hairline of the patient, so the scar of an MVD surgery is generally not visible after the operation.

Interviewer: can it happen that some patients are relieved in a day or two, and for some others it takes up to a month for the face to become normal?

Dr Jaydev Panchwagh: Though the hemifacial spasms generally disappear immediately after the operation, occasionally it takes longer, up to a month and a half after surgery. This is mentioned in international statistics and their results. Generally speaking, the results are better with early surgery. But the patient may not be receptive to the idea of surgery when the disease is in its early stage. Actually when patients initially begin suffering from this disease, if they are operated on, there is a statistically higher incidence of success.

Interviewer: Usually how soon can the patient get back to his normal life?

Dr Jaydev Panchwagh: Usually about ten to fifteen days after surgery, they are able to get back to their routine.

Interviewer: What are the risks of this operation? One is naturally afraid because it is after all, an operation on the brain…

Dr Jaydev Panchwagh: Any operation, whether on the leg or hand or abdomen… or in my specialty brain and spine, comes with some inherent unavoidable risks. I always say that it is necessary to weigh the risks of surgery against those of the disease itself. More importantly, the patient must ask himself the following questions: How experienced is the surgeon in this type of surgery? Is the hospital capable of catering to all aspects of care both during and after operation?

Interviewer: A very important question that comes to all our minds is that of expenses. Is this covered by medical insurance?

Dr Jaydev Panchwagh: Hemifacial Spasm and MVD are both covered by medical insurance if it is valid.

Interviewer: What is your message to the public about this disease?

Dr Jaydev Panchwagh:  My earnest appeal is “please take into consideration the timeline of the disease”. It is natural to postpone getting operated because the disease has just begun, ‘let me wait till it becomes a little worse, then I would decide’…is a common enough reaction. But the problem is that delay brings down the success rate of surgery. My message is “Please use the resources available to gain more knowledge of the disease, of the treatment options and surgery. Reach a well-informed decision. Do not go to faith healers and quacks for treatments and solutions… Also, don’t get scared of surgery or intimidated that it is a brain surgery. Do not wait till it is quite late.

Interviewer:  It is now clear that Hemifacial Spasm is a disease with serious implications, even though it may appear funny to the beholder. If you encounter any individual afflicted with the disease, please sympathise with them and advise them to consult a skilled and experienced neurosurgeon.

Doctor, a heartfelt ‘Thank you’ for enhancing our knowledge about Hemifacial spasm.

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