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 Table of Contents  
ORIGINAL ARTICLE
Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 263-267

Functional blindness: Blepharospasm with apraxia of eyelid opening


Eye Clinic, Kartal Research and Training Hospital, Istanbul, Turkey

Date of Submission10-Apr-2020
Date of Decision25-Apr-2020
Date of Acceptance15-Jun-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Titap Yazicioglu
Konaklar mh. Sebboy Sok. Petekler Sit. G/B D: 18, Yenilevent, Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_42_20

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  Abstract 


Purpose: The purpose was to evaluate the effectiveness of protractor myectomy surgery in patients with severe blepharospasm whose daily life is severely impaired. Materials and Methods: Records of fifty patients with benign, essential blepharospasm were examined retrospectively and seven were included in the study. All patients underwent a complete ophthalmological and neurological examination. The severity and frequency of spasm was determined by Jankovic Rating Scale, ranging from 0 to 4, and six daily activities were determined by Blepharospasm Disability Index. The primary indication for protractor myectomy was poor response to serial botulinum toxin injections. The main outcome measure was based on whether the spasms were fully healed or the need for additional botox application. Results: Fourteen eyes of seven patients with significant functional disability were included in the study. Excessive blinking and visual disability were the most common symptoms seen in all patients, and all had previously used one or more medications. Myectomy surgery was performed in all patients who were resistant to serial botulinum toxin injections. Of the 14 eyelids, 85.7% showed resolution of spasms and no longer required botulinum toxin treatment following surgery. One patient with keratitis sequelae and dry eye was given botulinum 1 month after the surgery. One patient with residual blepharospasm in the lower eyelid underwent lower eyelid myectomy and blepharoplasty. Transient periorbital edema, hematoma, and echymosis developed following surgery. The patients were followed up for 2 years, and excellent cosmetic result with no contracture was observed in all patients. Conclusion: Protractor myectomy is an effective treatment modality in cases of blepharospasm with severe functional disability.

Keywords: Apraxia, blepharospasm, botulinum toxin, myectomy


How to cite this article:
Yazicioglu T. Functional blindness: Blepharospasm with apraxia of eyelid opening. Kerala J Ophthalmol 2020;32:263-7

How to cite this URL:
Yazicioglu T. Functional blindness: Blepharospasm with apraxia of eyelid opening. Kerala J Ophthalmol [serial online] 2020 [cited 2021 Jan 16];32:263-7. Available from: http://www.kjophthal.com/text.asp?2020/32/3/263/304555




  Introduction Top


Benign essential blepharospasm (BEB) is a type of focal dystonia characterized by excessive, involuntary, repetitive contractions of the protractor muscles (orbicularis oculi, procerus, and corrugator muscles).[1],[2] The spasms have a significant impact on the daily activities of patients by causing visual disturbance or functional blindness, anxiety, and depression.[3],[4]

The exact cause of BEB is unknown. It is thought to be multifactorial, such as interaction of certain genetic and environmental factors.[4],[5] As it is known, the blink reflex is controlled by the basal ganglion (extrapyramidal dopaminergic circuit), and any abnormalities in the basal ganglion and dopaminergic system create BEB. It is also known that progressive loss of dopamine in the substantia nigra with aging increases the trigeminal blink reflex. This relationship between them explains why BEB is common in older age.[4]

The involuntary eyelid closure gradually worsens and results in apraxia of eyelid opening (AEO).[6] It is a nonparalytic motor abnormality and characterized by difficulty in opening the eyelids in the absence of visible contractions of the orbicularis oculi muscle.[6],[7] It is more common in women, and the peak age of onset is in the sixth and seventh decades of life.[8]

The most effective treatment option for BEB is botulinum neurotoxin Type A. It is well tolerated, but sometimes, the disease can be severe and resistant to botulinum toxin treatment.[9],[10] Such patients are candidates for surgical treatment.[5],[9],[11] Many surgical techniques, such as peripheral facial neurectomy by alcohol injections, surgical sectioning or percutaneous thermolysis, and selective peripheral facial nerve avulsion, have been used to interrupt the pathway from the motor cortex to the orbicularis oculi muscles.[1],[12] Among them, upper eyelid myectomy procedure, that involves removal of the orbital, preseptal, and pretarsal orbicularis muscles, as well as correction of the associated eyelid deformities, has been shown to be a very effective treatment in refractory cases.[10],[13]

Restricted daily activities of patients with eyelid apraxia may cause anxiety and depression. In this study, we intended to show the positive effects of surgery on their severely restricted life.


  Materials and Methods Top


In this retrospective study, fifty consecutive patients with blepharospasm treated with botulinum toxin were evaluated, and only seven of them who were refractory to botulinum toxin were included in the study. All procedures adhered to the tenets of the Declaration of Helsinki, and local approval form was received from the ethical committee. Written informed consent was obtained from every patient who participates in the study.

All patients had a full neurological examination to rule out other underlying neurological conditions, and a complete ophthalmological examination was conducted.

Patients with previous eyelid surgery or eyelid retraction were excluded from the study. Demographic features of the disease, duration and severity of spasm, presence of depression and anxiety, ocular irritation, burning sensation, watering, photophobia, sensory tricks, treatment received prior to surgery, number and type of surgery, duration of relief from spasms after surgery, complications or secondary effects of surgery, and further treatment data to obtain relief from blepharospasm after surgery were all observed and are summarized in [Table 1].
Table 1: General characteristics of the patients

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While evaluating the objective signs of spasms, we used Jankovic Rating Scale – a 5-point scale ranging from 0 to 4, where 0 indicates no symptoms and 4 indicates the most severe and frequent symptoms. We also used the Blepharospasm Disability Index in evaluating each patient's ability to drive, read, watch television, perform household tasks, and walk and were categorized as mild, moderate, or marked.

The primary indication for protractor myectomy was poor response to serial botulinum A toxin injections. The surgery was done under general anesthesia. Orbicularis fibers (preseptal, pretarsal, and orbital) and brow protractor muscles (corrugator, supraciliaris, and procerus) was carried out through a brow and lidcrease skin incision. Care was taken to the supraorbital nerve. In case of lower eyelid blepharospasm, we performed lower eyelid myectomy. The lower eyelid skin was dissected from the orbicularis muscle, and all visualized portions of the orbicularis muscle were removed. Additional eyelid deformities, such as dermatochalasis, were also taken care simultaneously in the necessary cases. The skin was closed with 6/0 Vicryl suture, and pressure bandage was applied to the lid.


  Results Top


A total of 14 eyes of 7 patients, 5 women and 2 men, with a mean age of 62.1 ± 6.4 years (range, 55–72 years), were enrolled in the study. The mean duration of illness was 21.4 ± 12.7 months (range, 12–36 months), and the mean follow-up period was 24.2 ± 5.7 months (range, 18–36 months). All the patients had tried one or more medications before. Excessive blinking and visual disability were the most common symptoms in all patients (100%), and the severity and frequency of blepharospasm was evaluated as Grade 4 [Figure 1]. All patients had anxiety-related disease and severe functional disability. There was only one patient who had keratitis sequelae and dry eye as the triggering factor and received botulinum toxin treatment due to blepharospasm.
Figure 1: Preoperative with blepharospasm and apraxia of lids demonstrating eyelid spasms

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All patients underwent protractor myectomy surgery [Figure 2]. Of the 14 eyelids, 85.7% showed resolution of spasms and no longer required botulinum toxin treatment following surgery. Only one patient (14.3%) with corneal disease and dry eye required further botulinum toxin injection. The average time for botulinum injection was 30 days. One patient (14.3%) had residual lower eyelid blepharospasm and treated with lower eyelid myectomy combined with lower eyelid blepharoplasty. There was no major morbidity or visual loss and no postoperative superfacial punctate keratitis, and none of the patients experienced marked forehead numbness. All patients had transient periorbital edema, hematoma, and echymosis following surgery. All eyelids had excellent cosmetic results without signs of contracture. The functional disability score was increased to Grade 0 in all patients, and adequate lid opening was achieved 3 months after the surgery [Figure 3]. In the patient with keratitis sequelae and dry eye, the spasms and corneal irritation signs were completely resolved, but due to low vision, he had some difficulty in performing the daily activities.
Figure 2: The corrugator superciliaris and procerus muscles between the brows and the base of the nose and also orbicularis muscles are removed

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Figure 3: Adequate lid opening was achieved 3 months after the surgery

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  Discussion Top


Intermittent involuntary closure of the eyelids may occur in the presence of spasmodic contractions of the orbicularis oculi or in the failure of levator palpebrae contraction.[3],[4] This failure is called AEO.[4]

Treatment of AEO is difficult.[3] Although botulinum toxin provides the most effective treatment, it is difficult to determine the proper dose, and also the effects of botulinum toxin are reversible and temporary, lasting approximately 3 months.[9],[11],[14]

It is very important for physicians and patients to be aware of AEO, when botulinum toxin fails to improve spasms despite the production of eyelid weakness.[15] While deciding the myectomy surgery, some conditions must be taken into consideration, such as AEO associated with BEB, blepharospasm-associated deformities, patients who are truly unresponsive to botulinum toxin, and patients who cannot afford or who refuse botulinum toxin injections.[7],[15],[16]

In a long-term study of patients with botulinum toxin-resistant BEB, it has been stated that subtotal myectomy provides subjective improvement and decreases the long-term need for injections in over 50% of patients.[11],[17] In another study, it has been said that among patients who received upper eyelid myectomy or upper and lower eyelid myectomy, there was an improvement in 88% of patients, with only 38% requiring injections after full myectomy.[11],[18] Ortisi et al. reported that protractor myectomy provides an improvement of the symptoms in 64% of patients and 89% of patients require continued injections of toxin.[10] Rana and Shah described two patients with apraxia of lid opening and blepharospasm, who responded to an initial treatment with botulinum toxin but later stopped responding satisfactorily despite increased doses.[15] Studies have also shown that patients who need botulinum toxin after myectomy surgery often require fewer injections which provide long-term relief.[10]

In our study, excessive blinking and visual disability were the most common symptoms seen in all patients (100%). While evaluating the spasms of patients, we used objective and subjective rating scales, as it is mentioned in the literature that the objective measures may not be sensitive and subjective rating scales may depend too much on the patient's memory expectations and psychological state at the time of rating.[14] According to this evaluation, the frequency and severity of blepharospasm of all patients in our study was Grade 4, and all had severe anxiety and functional disability.

It has been stated that there is a significant association between blepharospasm and anterior ocular segment diseases such as blepharitis and keratoconjunctivitis.[19] Dry eye has been reported frequently in patients with blepharospasm. It is difficult to determine whether dry eye causes blepharospasm, because the blink film increases to compensate for deficiency, or blepharospasm can cause dry eyes, because the periodic blinking effect of the eyelids is very important for the maintenance and renewal of the precorneal tear film.[20] We had one patient who was followed up due to keratitis sequelae and dry eye and received botulinum toxin for blepharospasm which resolved completely. Because of this result, we agree with the idea that these two mechanisms act together.

In our study, the preferred treatment in botulinum toxin-resistant cases was protractor myectomy which was successfully performed in 14 eyelids of seven patients. Nearly 85.7% of the patients showed resolution of spasm and no longer required botulinum toxin treatment following myectomy. One patient (14.3%) had residual lower eyelid blepharospasm and was treated with lower eyelid myectomy combined with lower eyelid blepharoplasty.

Although myectomy surgery is the first option in the treatment of AEO, there may be some surgery-related complications such as considerable swelling and ecchymosis, numbness of the forehead due to supraorbital nerve damage, chronic periorbital lymphedema, exposure keratitis, and ectropion.[1],[7] In our study, except transient periorbital edema, hematoma, and echymosis on the eyelids, we observed no major morbidity or visual loss related to surgery. All eyelids had excellent cosmetic results without signs of contracture, and adequate lid opening was achieved 3 months after the surgery.


  Conclusion Top


Blepharospasm with AEO is a troublesome condition that severely restricts daily activities. As the resolution of symptoms from botulinum toxin is transient and requires repeated injections, protractor myectomy surgery is a good option in the treatment of severe cases. Although the small number of patients and retrospective nature of the present study did not allow us to explain the longterm effectiveness of the surgery, we conclude that myectomy surgery has a positive effect on the recovery of the patient's social life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bates AK, Halliday BL, Bailey CS. Surgical management of essential blepharospasm. Br J Ophthalmol 1991;75:487-90.  Back to cited text no. 1
    
2.
Yoon DK. Surgical treatment of essential blepharospasm. Kor J Ophthalmol 1988;2:90-4.  Back to cited text no. 2
    
3.
Hallett M. Blepharospasm: Recent advances. Neurology 2002;59:1306-12.  Back to cited text no. 3
    
4.
Sameera I. Minimal orbicularis myectomy: Does it relieve spasms in benign essential blepharospasm? Am J Cosmetic Surg 2015;32:1-10.  Back to cited text no. 4
    
5.
Hallett M, Evinger C, Jankovic J, Stacy M; BEBRF International Workshop. Update on blepharospasm: Report from the BEBRF International Workshop. Neurology 2008;71:1275-82.  Back to cited text no. 5
    
6.
Yoon WT, Chung EJ, Lee SH, Kim BJ, Lee WY. Clinical analysis of blepharospasm and apraxia of eyelid opening in patients with Parkinsonism. J Clin Neurol 2005;1:159-65.  Back to cited text no. 6
    
7.
Jivraj I, Meredith B, Shriver E. “I can't open my eyes”: A case of blepharospasm and apraxia of eyelid opening. Ophthalmic Visual Sci 2015;23:1-7.  Back to cited text no. 7
    
8.
Defazio G, Livrea P, Lamberti P, De Salvia R, Laddomada G, Giorelli M, et al. Isolated so-called apraxia of eyelid opening: Report of 10 cases and a review of the literature. Eur Neurol 1998;39:204-10.  Back to cited text no. 8
    
9.
Jordan DR, Patrrinely JR, Anderson RL, Thiese SM. Essential blepharospasm and related dystonia. Survey Ophthalmol 1989;34:123-31.  Back to cited text no. 9
    
10.
Ortisi E, Henderson HW, Bunce C, Xing W, Collin JR. Blepharospasm and hemifacial spasm: A protocol for titration of botulinum toxin dose to the individual patient and for the management of refractory cases. Eye (Lond) 2006;20:916-22.  Back to cited text no. 10
    
11.
Clark J, Randolph J, Sokol JA, Moore NA, Lee HB, Nunery WR. Surgical approach to limiting skin contracture following protractor myectomy for essential blepharospasm. Digit J Ophthalmol 2017;23:8-12.  Back to cited text no. 11
    
12.
Waller RR, Kennedy RH, Henderson JW, Kenneth RK. Management of blepharospasm. Trans Am Ophthalmol Soc 1985;83:367-85.  Back to cited text no. 12
    
13.
Georgescu D, Vagefi MR, McMullan TF. Upper eyelid myectomy in blepharospasm with associated of lid opening. Am J Ophthalmol 2008;145:541-7.  Back to cited text no. 13
    
14.
Wabbels B, Jost WH, Roggenkämper P. Difficulties with differentiating botulinum toxin treatment effects in essential blepharospasm. J Neural Transm (Vienna) 2011;118:925-43.  Back to cited text no. 14
    
15.
Rana AQ, Shah R. Combination of blepharospasm and apraxia of eyelid opening: A condition resistant to treatment. Acta Neurol Belg 2012;112:95-6.  Back to cited text no. 15
    
16.
Pariseau B, Worley MW, Anderson RL. Myectomy for blepharospasm. Curr Opin Ophthalmol 2013;24:488-93.  Back to cited text no. 16
    
17.
Chapman KL, Bartley GB, Waller RR, Hodge DO. Follow-up of patients with essential blepharospasm who underwent eyelid protractor myectomy at the Mayo Clinic from 1980 through 1995. Ophthalmic Plast Reconstr Surg 1999;15:106-10.  Back to cited text no. 17
    
18.
Anderson RL, Patel BC, Holds JB, Jordan DR. Blepharospasm: Past, present, and future. Ophthalmic Plast Reconstr Surg 1998;14:305-17.  Back to cited text no. 18
    
19.
Sun Y, Tsai PJ, Chu CL, Huang WC, Bee YS. Epidemiology. PLoS One 2018;26:1-14.  Back to cited text no. 19
    
20.
Horwath-Winter J, Bergloeff J, Floegel I, Haller-Schober EM, Schmut O. Botulinum toxin A treatment in patients suffering from blepharospasm and dry eye. Br J Ophthalmol 2003;87:54-6.  Back to cited text no. 20
    


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