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Mr A AbuMattar

MRCOphth
Wycombe Hospital
BUCKINGHAMHIRE NHS TRUST
 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

AbuMattar / Departmental Audit 16/9/2009 2


 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.

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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.

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 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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 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.

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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.

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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.

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 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.

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 0 – No spasm  0 – No spasm
 1 - Slight disability present  1 – slightly increased
only with external stimuli, frequency of blinking
no functional impairment.  2- eyelid fluttering
 2 – Mild disability, lasting < 1 second
noticeable, possibly  3– eyelid spasm lasting >
embarrassing but no 1 second but eyes open
functional impairment. > 50% of waking times
 3 - Moderate disability,  4 – functionally blind due
mild functional impairment to persistent closure
 4 – Severe incapacitating (blepharospasm) more
spasm of eyelids. than 50% of waking time
Severity of dystonia Frequency

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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”.

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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%)

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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)

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 Blepharospasm Disability Index
◦ Most patients express some form of disability
ranging between mild to severe.

Blepharospasm Disability Percentage of patients


Index BSDI (number of patients)
Mild disability 57.9% (11/19)
10 or less
Moderate disability 10.5% (2/19)
Between 10 and 20
Severe disability 15.8% (3/19)
More than 20
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.

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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)

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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.
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 Mean Duration of Number of
Global Latency to Response in patients
Rating Response in weeks
(range) days (range)
(10)
(range) (10) (10)
(10)

2.8 4 9.86 19
Blepharospasm (0-4) (0-14) (4-30)
(3.4) (4.2) (12.4) (90)

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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
Bus driver Caterer hotelier Receptionist
Semiretired consultant Payroll manager Premises manager in
mechanical engineer Clerk secondary school
Office work Checkout operator Sales admin and
Hair dresser Office work publishing
Company director Housewife

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 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™.

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Diagnosis Complication % of Patients with the
complication(11)193 patients
Local
Ptosis† 57.9% 58% mild +6.7% marked
Diplopia 5.3% 25.4%
Local lid swelling 10.53% 3.1%
Watery eye 26.3% 2.1%
Blepharospasm Allergic reaction 0.00% 1.5%
Corneal abrasion 5.3%
Systemic
Headaches/Light
headedness 5.3%
† Ptosis appeared 1-5 days post
injection and disappeared 1-2
weeks later

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Make it worse Make it better No effect
Bright light 42.1% 36.84%
Tiredness 42.1% 5.26% 31.57
Awareness someone 52.63% 42.1%
watching you doing
something
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 5.3% 21.05% 47.36%
you do

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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

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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.

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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.1735%
 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%

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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.

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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.

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“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

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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

AbuMattar / Departmental Audit 16/9/2009 35


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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