Blepharospasm Audit

Blepharospasm in WGH Patients

Mr A AbuMattarMRCOphth

Wycombe HospitalBUCKINGHAMHIRE NHS TRUST

AbuMattar / Departmental Audit 2

The facial dystonias benign essential blepharospasm and hemifacial spasm are rare disorders1, with an incidence of 8.9 per 100,000 people18, which usually start with an increase in frequency of blinking, are often associated with dry eye syndrome, and sometimes occur with precipitating factors.2,3

With time, the phenomenon becomes more frequent until it causes difficulty with carrying out daily activities, and impairs health related quality of life, causing social embarrassment, anxiety, and depression.3,4 Rarely the spasm can cause functional blindness.4

Botulinum toxin type A has been demonstrated to improve the quality of life in OM dystonias.5

Introduction

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A retrospective noncomparative interventional study in the form of a questionnaire, to assess the subjective patient benefit of Botulinum toxin treatments for essential blepharospasm in patients attending the specialist Botulinum toxin clinic at Wycombe General Hospital and to determine the complications and impact of treatment on quality of life of essential blepharospasm patients.

Objective

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A computer search was used to identify all patients who had attended the clinic in the period between March/2008 and April/2009.

All patients were treated with the protocol to tailor the toxin dose. This subset was analysed to determine the number of visits required to titrate the dose, and the change in symptoms following treatment.

A comprehensive questionnaire was handed to patients while waiting for their treatment in the clinic and given the instruction to enquire or discus any aspect of the questionnaire.

Patients and Methods

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Questionnaire Advantages of Written Questionnaires6

◦ Are very cost effective◦ Are familiar to most people◦ Reduce bias. There is uniform question

presentation and no middle-man bias. The researcher's own opinions will not influence the respondent to answer questions in a certain manner. There are no verbal or visual clues to influence the respondent

◦ Are easy to analyze◦ Are less intrusive than telephone or face-to-face

surveys

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Questionnaire Disadvantages Of Written Questionnaires6

◦ The curse of statistical analysis in questionnaires is low response rate.

◦ Inability to investigate responses.◦ They allow little flexibility to the respondent

with respect to response format.◦ Nearly 90% of all communication is visual.

Gestures and other visual clues are not available with written questionnaires. A questionnaire requesting factual information will probably not be affected by the lack of personal contact.

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For new patients we Explain◦ The nature of the problem to the patient.◦ We don’t know the underlying cause.◦ ? Chemical imbalance in movement control centres◦ May spread to involve the lower face, jaw and neck but

will not become generalised.◦ Remote possibility of spontaneous improvement◦ We don’t know how to cure the condition but we can

usually control it. ◦ Discus the risks and benefits of injections with special

emphasis on the fact that patient will experience one or more of the side effects in the course of his/her treatment.

Management protocol WGH

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At their first clinical visit, the patient is given a standard dose of botulinum toxin.

For blepharospasm and hemifacial spasm, our standard dose is 20U of Dysport™ in 2 locations and 30U of Dysport™ in one location around the palpebral structures of each eye.

The standard dose can be altered according to clinical judgement.

Treatment is also started for any signs or symptoms of dry eye or blepharitis.


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Ten units of Dysport™ in two locations on the upper lid for those patients with evidence of pretarsal spasm.

The pretarsal injections are given as close to the lid margin as possible as recommended by Mackie7 for the control of Riolan’s muscle


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Patients with aberrant facial nerve regeneration and myokymias are treated with lower initial doses according to clinical judgement

Patients with hemifacial spasm are referred to a neurologist for further assessment and neuro-imaging (in parallel with treatment for the spasm)

The patient is then reviewed 6 week later (with clear instruction to phone for any problem), that is, visit 2. If the spasm is controlled, the patient continues with the same injections as required for the individual patient on a regular basis. If however the spasm is not fully controlled, further injections are given to any muscles that remain in spasm


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Patients who have severe spasm that cannot be controlled without producing side effects are offered surgery, that is, Blepharoplasty with limited Myectomy. The purpose is to reduce the bulk of the orbicularis muscle in the area that can be reached through a cosmetic blepharoplasty incision such that the patients can keep their eyes open spontaneously or the spasm can be controlled with toxin injections without producing intolerable side effects by using less toxin and / or less frequent injections.


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The Dysport™ vials are stored frozen until the toxin is reconstituted

A vial of 500 units freeze dried Dysport ™ toxin is reconstituted with 2.5 ml of preservative free 0.9% saline solution to yield toxin in a concentration of 20 units per 0.1 ml.

The toxin is injected subcutaneously with 27-30 gauge needle.

With the patient in a sitting position The eyelids and eyebrows are cleaned with alcohol and the toxin injected medially and laterally into the pretarsal orbicularis oculi, avoiding the superior oblique and the levator palpebrae muscles to prevent Diplopia and Ptosis respectively.

Injection technique

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Dystonia scale / Jankovic clinical grading of severity of spasm ◦ Zero to four point scale. ◦ used to assess function and degree of improvement. ◦ Zero represented no spasm and four denoted severe

incapacitating spasm. Peak effect scale

◦ Maximal benefit obtained from the injections◦ Rated on zero to four.

Global rating scale◦ Peak rating score minus one point if the injection is

associated with mild to moderate complications, and minus 2 points if associated with severe or disabling complications.

Baylor study rating scales 8

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Peak effect rating scales magnitude of subjective response

Magnitude of subjective response considering improvement in function scale (0 to 4) was assessed as follow9 : ◦ 0 - no effect. ◦ 1 - mild effect but no functional improvement.◦ 2 - moderate improvement but no change in functional

disability.◦ 3 - moderate change in both severity and function.◦ 4 - marked improvement in severity and function.

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Disease-specific self-assessment scale consisting of 6 × 5-point items assessing:◦ Vehicle driving ◦ Reading ◦ Watching TV ◦ Shopping ◦ Getting about on foot◦ Doing everyday activities

0 = no interference in these activities. 30 = severe interference.

Higher score indicates greater severity in the scale.

Blepharospasm Disability Index

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Jankovic clinical grading of severity and frequency of spasm

Severity of dystonia Frequency

0 – No spasm 1 - Slight disability present

only with external stimuli, no functional impairment.

2 – Mild disability, noticeable, possibly embarrassing but no functional impairment.

3 - Moderate disability, mild functional impairment

4 – Severe incapacitating spasm of eyelids.

0 – No spasm 1 – slightly increased

frequency of blinking 2- eyelid fluttering lasting

< 1 second 3– eyelid spasm lasting >

1 second but eyes open > 50% of waking times

4 – functionally blind due to persistent closure (blepharospasm) more than 50% of waking time

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Patients rated and recorded the degree of their spasm

◦ “Latency” was defined as the interval (in days) between the injection and the first sign of improvement following the injection.

◦ “Global rating” was used as a measure of overall response. A definite improvement with a global rating ⋝2 on a 0-4 scale.

◦ The “maximum” duration of improvement was the number of weeks during which the patients experienced “peak effect”.

Patients rating of Degree of their spasm

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A total of Forty one (41) patients attend WGH specialised clinic for a variety of oromandibular dystonias including: Blepharospasm, Hemifacial spasm, Aberrant regeneration and others.

Only Blepharospasm patients included in the study.

Twenty one(51%) returned the questionnaire of which 2 where hemifacial spasm and hence excluded leaving 19 for the study. (46%)

Results

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Jankovic clinical grading of severity of spasm of WGH patients:◦ 89.5% of patients suffer from ⋝2 severity scale in

a 0-4 scale Grade 0 10.5% (2/19) Grade 1 0.00% (0/19) Grade 2 42% (8/19) Grade 3 26.3% (5/19) Grade 4 21.1% (4/19)

Results

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Results Blepharospasm Disability Index

◦ Most patients express some form of disability ranging between mild to severe.

Blepharospasm Disability Index BSDI

Percentage of patients(number of patients)

Mild disability 10 or less

57.9% (11/19)

Moderate disability Between 10 and 20

10.5% (2/19)

Severe disabilityMore than 20

15.8% (3/19)

Undetermined 15.8% (3/19)

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The response was noted on an average of four days after the injection (“Latency interval” ranged between 1-14 days with a mean latency interval of 4 days).

The duration of “maximum” improvement ranged between 4-30 weeks with mean duration of “maximum” improvement of 9.86 weeks.

Results

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Peak effect rating scales◦ All patients appreciated improvement in their

symptoms Grade 0 – 0.00% Grade 1 – 10.53%(2/19) Grade 2 – 10.53% (2/19) Grade 3 – 26.31% (5/19) Grade 4 – 52.63% (10/19)

Results

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Global rating scale◦ 89.5% of patients demonstrated definite

improvement by scoring ⋝2 in 0-4 scale. ◦ Only 10.5% of patients failed to improve with

global rating of ⋜1 after one or more injections. ◦ Mean global rating of 2.8 indicated that botulinum

toxin significantly improved disability from blepharospasm in our patients.

Results

Global rating Percentage of patients(number of patients)

0 10.53% (2/19)

1 0.00% (0 /19)

2 31.57% (6/19)

3 42.11% (8/19)

4 15.79% (3/19)

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Mean Global Rating

(range)

(10)

Mean Latency to Response

in days(range)

(10)

Duration of Response in weeks (range)

(10)

Number of patients

(10)

Blepharospasm

2.8(0-4)

(3.4)

4 (0-14)

(4.2)

9.86(4-30)

(12.4)

19

(90)

Results

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Gender ◦ Male : Female ratio is 1: 2.16

(Epidemiological studies have shown ratios of occurrence in men to women of 1:1.4 to 1:2.2 and mean ages of onset of 39.2 years for men and 42.9 years for women. 19,20 )

Age◦ Fifth to 8th decade

Patient spectrum

WGH patients

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Bus driverSemiretired consultant mechanical engineerOffice workHair dresser

Caterer hotelierPayroll managerClerkCheckout operatorOffice workCompany director

ReceptionistPremises manager in secondary schoolSales admin and publishingHousewife


1/3rd of patients attend the clinic for more than 5 years.

1/3rd of patients have their injections every 6 weeks.

Right :Left : Both sides ratio is 1:1:1. In bilateral Right > Left. All patients receive Dysport™ but when

asked all picked Botox™.

WGH patients

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WGH patients Complications

Diagnosis Complication % of Patients with the complication(11)193 patients

Blepharospasm

Local Ptosis†

DiplopiaLocal lid swellingWatery eyeAllergic reactionCorneal abrasion

Systemic Headaches/Light headedness

57.9% 58% mild +6.7% marked

5.3% 25.4%

10.53% 3.1%

26.3% 2.1%

0.00% 1.5%

5.3%

5.3%† Ptosis appeared 1-5 days post injection and disappeared 1-2 weeks later

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WGH patients clinical features

Make it worse Make it better No effect Bright light 42.1% 36.84%

Tiredness 42.1% 5.26% 31.57

Awareness someone watching you doing

something

52.63% 42.1%

Watching television 30.57% 5.26% 42.1%

Reading 10.52% 57.89%

Dark glasses 10.52% 26.3% 36.84%

Driving 0% 5.3% 57.9%

Coffee 5.3% 52.6%

Touching forehead 5.3% 5.3% 57.89%

Talking 5.3% 5.3% 57.89%

Pulling your eyelid 10.52% 15.79% 47.36%

Yawning 5.3% 63.15%

Humming and singing 10.52% 52.53%

Concentration on what you do

5.3% 21.05% 47.36%

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The results of treatment are difficult to assess accurately in a retrospective study; however, 89.5% of patients experienced an improvement in symptoms. These figures compare favourably to other retrospective studies that report improvements in symptoms in 85–95% of patients.12,13,14,15

Patients could read, watch television, drive and function almost normally because of reduced lid and eyebrow spasms

Discussion

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Botulinum toxin type A is safe and effective treatment for blepharospasm which support findings from double blinded, placebo controlled studies.16

0% patients had complications that were considered by the patients as disabling.

Discussion

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In this study ⋝15.7% of patients needed more than one initial visit to control the spasm suggesting that there are many patients for whom the standard dose does not provide optimal control of symptoms. These figures still compare will with other specialised tertiary referral clinic.1735%

1/4th (26.3%) of patients underwent Blepharoplasty with Orbicularis de-bulking

Symptoms improved in 80% of patients. 20% stayed the same. Botulinum toxin injections continued in 100%

Discussion

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Ptosis was experienced in More than half of patients. This is important to consider when counselling patients, especially Ptosis may prevent the patient from driving.

Discussion

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90% of patients are satisfied with the doctor attitude and how he listen to them.

53% of patients are satisfied with the waiting time in the clinic.

95% of patients are satisfied with their consultation time.

90% of patients are satisfied with the explanation given in their first consultation.

Discussion

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THANK YOU

“The combination of the operation with the regular injections has given me a greatly improved quality of life . I am able to undertake some work and have also regained my driving license (after 6 years).” Edwin Smith

Semiretired consultant mechanical engineer

Dystonia Society websitehttp://www.dystonia.org.uk

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1. Bradley EA, Hodge DO, Bartley GB. Benign essential blepharospasm among residents of Olmsted County, Minnesota, 1976 to 1995: an epidemiological study. Ophthal Plast Reconstr Surg 2003; 19: 177–181.

2. Grandas F, Elston J, Quinn N, Marsden CD. Blepharospasm: review of 264 patients. J Neurol Neurosurg Psychiatry 1988; 51: 767–772.

3. Anderson RL, Patel BC, Holds JB, Jordan DR. Blepharospasm: past, present, and future. Ophthal Plast Reconstr Surg 1998; 14: 305–317.

4. Elston JS. Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injection. Br J Ophthalmol 1987; 71: 664–668.

5. Bhattacharyya N, TarsyD, Impact on quality of of life of botulinum toxin treatment for spasmodic dysphonia and oromandibular dystonia. Arch Otolaryngol Head Neck Surg 2001;127:389-392

6. http://www.statpac.com/surveys/disadvantages.htm 7. Mackie IA. Riolan’s muscle: action and indications for botulinum toxin injection.

Eye 2000; 14: 347–352. 8. Dystonia: etiology, clinical features, and treatment page 171 By Mitchell F. Brin,

Cynthia Comella, Joseph Jankovic 9. Brin MF, Jankovic J, Comella C et al : Treatment of dystonia using botulinum

toxin. In : Treatment of movement disorders. Kurlan R, J.B. (Ed.) Lippincott Company 1995; 183-230

References

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10. Journal of Neurology, Neurosurgery, and Psychiatry 1990;53:633-639 Botulinum toxin treatment of cranial-cervical dystonia, spasmodic dysphonia, other focal dystonias and hemifacial spasm . Joseph Jankovic, Kenneth Schwartz, Donald T Donovan

11. Blepharospasm and hemifacial spasm: a protocol for titration of botulinum toxin dose to the individual patient and for the management of refractory cases E Ortisi, HWA Henderson, C Bunce, W Xing and JRO Collin. Eye (2006) 20, 916–922. ©2006 Nature Publishing Group All rights reserved 0950-222X/06

12. Grandas F, Elston J, Quinn N, Marsden CD. Blepharospasm: review of 264 patients. J Neurol Neurosurg Psychiatry 1988; 51: 767–772.

13. Anderson RL, Patel BC, Holds JB, Jordan DR. Blepharospasm: past, present, and future. Ophthal Plast Reconstr Surg 1998; 14: 305–317.

14. Elston JS. Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injection. Br J Ophthalmol 1987; 71: 664–668.

15. Aramideh M, Ongerboer de Visser BW, Brans JW, Koelman JH, Speelman JD. Pretarsal application of botulinum toxin for the treatment of blepharospasm. J Neurol Neurosurg Psychiatry 1995; 59: 309–311.

16. Jankovic J, Oriman J. Botulinum A toxin for cranial-cervical dystonia: A double-blind, placebo-controlled study.Neurology 1987;37:616-23.

17. Blepharospasm and hemifacial spasm: a protocol for titration of botulinum toxin dose to the individual patient and for the management of refractory cases

18. Nutt JG, Muenter MD, Melton LJ III, et al. Epidemiology of dystonia in Rochester, Minnesota. Adv Neurol. 1988;50:361-365

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