Apraxia of Eyelid Opening

This is the article that Dr John Burns and I wrote for the Benign Essential Blepharospasm Research Foundation newsletter (2005:24;9-10)

Mixed Benign Essential Blepharospasm and Apraxia of Eyelid Opening.

The New Missed Diagnosis

In the 16th Century, Flemish artist, Brueghel painted “De Gaper” which depicted a subject with markedly distorted facial features and eyelid spasms.  This is generally considered to be the first recorded case of benign essential blepharospasm and is in fact what we would now sub categorize as Meige syndrome.  From that time until the mid 1980’s, benign essential blepharospasm and Meige syndrome was a relatively unknown facial disease rarely diagnosed correctly by physicians and even less frequently treated appropriately.

In 1981, the beloved founder of BEBFR Society, Maddie Lou Koster decided that she would bring the disease out into the open and make the diagnosis and its signs and symptoms as familiar to the medical community as the common cold.  All neurologists, ophthalmologists, and neurosurgeons would certainly concur that she was immensely successful.

In 1990, Dr. Rick Anderson with the support of the BEBRF mailed out 4,000 questionnaires to patients who registered prior to 1988 with the Benign Essential Blepharospasm Foundation, of which 1,653 were returned.  One of the questions was, “how many physicians did you see prior to the establishment of a correct diagnosis?”  At that time, the average response was four physicians with the range between 1 and 75.  The cost of misdiagnosis, unnecessary studies, and unsuccessful therapy was staggering and in one percent of patients exceeded $50,000.  It is my personal experience that in the past few years virtually all patients referred to my office for treatment of benign essential blepharospasm and Meige syndrome have been correctly diagnosed by either the first or second doctor consulted for their symptoms.  Furthermore, most patients are seen within six months of the onset of their symptoms as opposed to my experience in the late 1980’s when most patients had had their disease for many years and were in the advanced stages of the syndrome.  It is apparent that the past two decades have seen a marked improvement in the efficiency of diagnosing and managing benign essential blepharospasm and Meige syndrome.

Conversely, mixed benign essential blepharospasm with apraxia of lid opening is in my experience rarely initially diagnosed by the examining physician.  Moreover it is even missed by those physicians specialized in the management of this condition, including ophthalmologists and neurologists.  Apraxia of lid opening was first described by Goldstein and Cogan in 1965 as “a non-paralytic motor abnormality characterized by the patient’s difficulty in initiating the act of lid elevation.”  Apraxia is generally defined as the inability to carry out purposeful movements in the absence of motor paralysis, sensory loss, or ataxia.  To paraphrase the definition of apraxia of lid opening, it is a patient who reports, “I can’t will my eyes to open.”

Just as blepharospasm and Meige syndrome were only gradually recognized to be relatively common diseases, mixed benign essential blepharospasm and apraxia of lid opening has also been statistically found to be a relatively common variant blepharospasm.  In 1990, David R. Jordan, Rachel Anderson, Katherine B. Digre, evaluated patients who were referred to them with the diagnosis of benign essential blepharospasm or Meige syndrome.  As they followed these patients from diagnosis, initial treatment, and in some cases surgical management, it became apparent that 7 patients (7%) of this population actually had apraxia of lid opening in addition to their referring diagnosis.  None of these seven patients were correctly diagnosed at the time of the initial visit despite these physicians’ sophisticated attempts to diagnose the mixed condition.  Between 1988 and 1992, Dr. P. Krack and M.H. Marion in the Department of Neurology, University of Giessen, Germany evaluated 195 patients with benign essential blepharospasm or Meige syndrome and of that number, 20 were later diagnosed as also having apraxia of lid opening.  These twenty patients represent a comparable 10% incidence of mixed disease among their blepharospasm patients. 

In the classical presentation, benign essential blepharospasm occurs in a woman with an average age of 55 who initially notices increased blinking and sensitivity to light.  This is followed by increasingly frequent intermittent forceful eyelid closure. 

( The spasms of the corrugator supraciliaris muscle and procerus muscles draw the brows medially and inferiorly from their normal position. )

In Meige syndrome, she would have also developed spasms of the mid and lower face.  Both of these syndromes are frequently associated with varying degrees of dry eye syndrome.    

Apraxia of lid opening can occur in association with blepharospasm, but may occur rarely in isolation.  In the “pure” form, the patient experiences sustained periods of time when she is unable to will her eyes open.  During these episodes, the eyes are fully closed.  In contrast to benign essential blepharospasm and Meige syndrome however, there is no evidence of orbicularis activity and the brows are markedly elevated by the frontalis muscles, as would be expected in someone straining to open her eyes.   In the fully developed clinical picture of mixed blepharospasm and apraxia of lid opening, the patient presents with a combined pattern beginning with sustained spasms of the procerus and corrugator muscles lowering the brow as well as the orbicularis muscles squeezing the eyes shut.  The patient eventually has relaxation of those muscles resulting in an elevation of the brows, but still lacks the ability to open the eyes.  This sequence of events is difficult to observe unless the physician has a very high index of suspicion.

The diagnosis of apraxia of lid opening is established by the use of the most common and effective treatment for benign essential blepharospasm, Botox.  The patient should initially be treated utilizing the protocol each physician has found most successful for their benign essential blepharospasm patients.  Most patients return in three months for a reevaluation and adjustment of therapy.  The typical patient with mixed disease however will return reporting that they have not appreciated improvement from the Botox treatment.  In this situation, re-treatment is indicated followed by an evaluation two to four weeks later, at which time the maximal Botox effect should be apparent.  Adequate paresis is achieved when the patient demonstrates an inability to constrict the upper and lower lids against the resistance of the physician’s fingers.  In the patient with apraxia of lid opening, there are periods of persistent involuntary lid closure despite adequate Botox treatment of the orbicularis and brow depressor muscles.  The patient will usually demonstrate brow elevation in a futile attempt to open the lids.  Patients often report that gently elevating the lids open with their fingers or touching the skin on their temple can sometimes break the attacks.  When the episode resolves, there is no evidence of ptosis or levator muscle weakness. 

It has been uniformly observed by physicians with an interest in mixed blepharospasms that traditional blepharospasm treatments are ineffective in improving the apraxia of lid opening.  Specifically, Botox, adjunct oral medications, and both limited and complete myectomies do not improve apraxia of lid opening.  Apraxia of lid opening, however, can be successfully managed after control of the patient’s blepharospasm with surgical frontalis suspension.  This is a commonly performed and successful eyelid elevating operation in appropriately chosen patients.  The tethering of the brow to the lid with this procedure is believed to bring the relief achieved by patients who break the attacks with their fingers.  In patients with mixed disease, it can be combined with a limited myectomy procedure that removes orbicularis muscle from the lids to provide improved control of both conditions.

In conclusion, mixed blepharospasm and apraxia of lid opening is a relatively common cause of the inability to maintain open and functional eyelids and is probably present in at least 7% of patients with blepharospasm.  It is also likely the reason for a significant number of treatment failures.  Observation of a patient’s eyebrows throughout the complete cycle of eyelid closure, attempted reopening, and then full normal opening, are the key to early diagnosis and appropriate treatment.

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22 responses to “Apraxia of Eyelid Opening

  1. Dr. Chen,
    I read with interest, your article about apraxia of eyelid opening as I am searching the internet for information to explain this symptom. I am a surgical Physician Assistant and had a severe case of bilateral bacterial conjunctivitis this past Christmas. It responded to antibiotic eye gtts, but ever since, I have difficulty initiating the act of lid elevation upon waking from sleep either in the morning or after a nap. I have no trouble keeping them open or blinking once they are open. I have seen my O.D. who told me I have borderline “dry eyes” and gave me optifast drops with no improvement. Would you recommend that I see a neurologist or opthalomologist? I live in the city of San Francisco , am 51 years old and in otherwise good health.
    Thank you,
    Robin Wearley, PA-C

    • Hi, I would just like to add that I have the exact same symptoms. i just had severe bacterial conjunctivitis and it has cleared but I have the same problem with one of my eyes when I wake up occasionally. I find it hard to believe it to be a coincidence but no-one has anyone answers for the cause. Also the fact that it follows conjunctivitis must mean something?

  2. I too am having a problem opening my eyes upon waking. I have seen a neurologist and no diagnosis. I am now off to a opthalomologist. I am really frustrated. Any suggestions??

  3. Hi L Fox,
    I have since learned that my symptoms may be simply, sleep paralysis. Apparently it is self-limiting. One opthalomologist I corresponded with said he had another patient with similar symptoms. It lasted two years and resolved uneventfully.
    Robin

  4. gina mclellan

    I have been treated for blepharospasm with Botox for over 3 years with marginal effectiveness. My condition is more to the apraxia side than true blepharospasm as the eyes simply begin to slowly close. My brows have “fallen” significantly since this condition began and I get more frustrated overall each day. I have asked my opthalmologist about surgical procedures that might help and have been referred to a specialist to consult with on that question. As a result of the Botox, I am also now experiencing ptosis of the right eyelid. Might surgery be an option? I am a very healthy and active 59 year old woman.

  5. Hi Dr. Chen:
    I’m 45 years old, diagnosed with hemi blepharospasm 10 years ago and had been using botox as treatment since then. I had botox every 6 months and it’s not working anymore. I started with 20 units of botox and now not even 40 units is working. It usually works within a week and severe eye & facial twitching would recur within less than a month. My right eye , cheek and lip twitch most of the time and even in my sleep and eyelid muscle grew bigger and heavier to some point shutting my right eye. I can’t get any answers other than botox from my opthalmologist. Will surgery of the eyelid help lessen the twitching and shutting of the eye? Hope you can help me.
    Thank you.

  6. poonsri netcharu

    i need a good doctor to give me a treatment apraxia eye lid open after i had done surgery under my eye and i need some help

    thanks

  7. I have a friend who, after back surgery couldn’t open his eyes but it went away in a week. 4 years later because of Parkinsons he had an operation, DBS. It is 5 months and he is still having trouble opening his eyes in the morning, during naps and it seems that everytime he lowers his head in a sitting position, his eyes automatically close. He has no other symptoms. Please advise. Although the Parkinson symptoms are gone the patient is now unable to live alone. Please help

  8. Please notify me of follow-up comments

  9. I recently had frontalis suspension for eyelid apraxia and it has just progressed. When I first got the surgery about 3 months ago it seemed to be a little better but from there it has gotten worse. Please if you will, advise me on other treatment since I feel I am at my ropes end, or is ther nothing else I can do? Thank you for any help you may offer. Ben

    • Adriano Bonafede

      Hi Ben, I have the same problem (apraxia) and I would like having the same surgery you had. But by reading your experience now I am a little worried. Was your surgery really ineffective?
      Adriano

  10. Only two weeks ago I began to awaken and could not open my eyes. It is now consistently a problem. It takes 5 seconds to get them open. Once open, I need to blink a couple of times to get them to stay open.
    This only occurs after REM sleep.
    I had a mild conjunctivitis that went away. That’s when it started. For several days I did have red eyes and crust.
    Also, exactly two weeks ago, I changed my contact lenses to another prescription/brand.
    I am female, 44 years old. Otherwise no motor function loss, healthy, nutrition-minded and active.

  11. Hello. Congratulations on your great post. Looks good to me. Thanks for helping to bring awareness to this issue. I will thank the person who told me to visit your blog. Zachary

  12. Hah am I literally the first comment to this awesome writing!

  13. You have done it once again! Great post!

  14. AWWWWWWW I feel so much better knowing it isn’t a sign of ms or something. I’m a healthy 62 year old woman….and I need my eyes to continue my work. Very helpful….Thank You

  15. Adriano Bonafede

    Dear Dr. Chen, I am an Italian journalist and live in Rome. I am 59 years old and I have been suffering from a kind of apraxia eyelid opening since 3 years. Botox has almost no effects and I have to keep my eyes open by using my fingers. I have read the article you wrote with Dr. Burns and now I am looking for a surgery. Could you help me to find a doctor? Could you make this surgery? How much would it cost to me, approximately?Thank you for your help in advance.

  16. Dear Dr. Chen,
    I am 55 years old and in good health. I have been diagosed with apraxia of the lid opening and dry eyes. Do you know of a specialist in the Buffalo, NY area who has experience and could help me.
    Thank you

  17. I have been treated twice with botox. It did not help. I told my eye Dr. that i have apraxia of the eye lid opening. He told me apraxia of the lid openings just happens after a stroke. Don’t know where to go from here.

  18. hi.,,.i,m reading all these posts and find that i,m having the same problem,.,.my right eye lid is hard to open after 2/3 seconds .,,.it,s not every morning but still.,.,.,.,.i have a friend who is suffering with bfs and is now having surgery in may 2012 which she,s not looking forward to.,,..does anyone know how successful the surgery is please.,..,thankyou .,..,we both live in calgary alberta

  19. My problem starts at around 10 pm with difficulty holding my lids open. They feel like lead weights. When I wake up in the am I have difficulty opening my eyes. Usually, I just take my finger and open them. No problems during the day. I did have an eye infection about 6 months ago. I am a healthy 60 y/o woman.

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