dr. jeremy raiter - ophthalmic surgeon & physician …a normal blink •frequency 15 times per...

Dr. Jeremy Raiter - Ophthalmic Surgeon & Physician M.B.B.S (WA) F.R.A.N.Z.C.O

Initial consultation 2014


Benign Essential Blepharospasm (BEB)

• Uncommon chronic condition

• Characterised initially by excessive blinking

• Increased sensitivity to light.

• Leads to uncontrollable eyelid closure

• Rendering the patient “functionally” blind.

• 3/12 injections allows patient to live relatively normal lives


Benign Essential Blepharospasm (BEB)

• Widely understood amongst ophthalmologists and neurologists

• Less familiar to Front-line professions

• GPs, optometrists and pharmacists.

• Traditional diagnosis is invariably dry eye which the patient may well have, whilst the underlying condition of BEB is not recognised.

• Historically a patient can struggle with BEB for years before they stumble across somebody who identifies BEB and refers them for effective treatment.

• In the meantime, the patient's life is so severely affected they can't work, drive or live a normal life leading to a severe level of depression

Dr. Jeremy Raiter - Ophthalmic Surgeon & Physician

M.B.B.S (WA) F.R.A.N.Z.C.O

Blepharospasm Australia Inc

• Support group - operates from Perth

• Registered charity - endeavours to raise the awareness amongst this front-line group and does effective work.

• Web site acts as a hub for clinical articles from around the world as they liaise with their peers in the USA, UK and Canada.

• Having BEB in the curriculum for medical students both at university level and in ongoing professional development would make a difference in shortening the diagnosis period.

• Website address: https://blepharospasmaustralia.org.au


https://blepharospasmaustralia.org.au/

The Eyelids - Anatomy

• Skin – thinnest of the whole body

• Subcutaneous tissue

• Skeletal muscle – orbicularis oculi

• Tarsal plate – attaches medial /lateral palpebral lig and orbital septum

• Conjunctiva



Carmine Clemente 1975


Pre-tarsal - Riolan’s muscle

Mackie Eye 2000;14:347

The Eyelids - Function

One of the most important elements in the protective system of the eye

(1) The screening and sensing action of the cilia

(2) The secretions of the glands of the eyelids

(3) The movements of the lids

- Elevation (a) Spontaneous (central)

- Closure - Blinking (b) Reflex (tactile, auditory, visual)

- Winking

- Blepharospasm


A Normal Blink

• Frequency 15 times per minute

• Duration 0.3-0.4 secs (Vision must be in abeyance)

• Average period between blinks 2.8 secs(M) < 4.0 secs (F)

• Slow motion photography demonstrates a zippering closure of the eye lids from temporal to nasal

• Forces the tears towards the punctae situated medially on the upper and lower lid margin

• Results in cleaning and wetting of the corneal surface

• Does not produce discontinuity visual sensation (similar to saccade)


Abnormal Blink

• Benign Essential Blepharospasm (BEB)

• Hemifacial spasm

• Meige’s Syndrome (Oro-mandibular involvement)

• Myokymia

• Facial tics

• Parkinson’s disease


Benign Essential Blepharospasm

• Definition

• Incidence

• Prevalence

• Male:Female ratio

• Age of onset

• Symptoms


BEB : Definition

• Is a term used to describe progressive, involuntary contraction of the eyelid muscles that leads to frequent , uncontrollable blinking and in severe cases “functional blindness”.

• Focal dystonia

• Exact cause is unknown. ? Inappropriate signal within Basal Ganglia

• 75% of patients are female F:M 3:1 ratio

• Average age of onset – 56 years

• Affects about 32 per 100,000 people


Benign Essential Blepharospasm

• Primary, essential, idiopathic, benign BS

• Secondary: dopamine antagonists (neuroleptics), PD, PSP,

DBS

• Basal ganglia circuitry

• Reduced dopamine receptor binding (age)

• Sensory afferents (photophobia) blepharitis, dry eye

• Genetics>environment


Dystonia : Definition

• Syndrome of sustained, involuntary muscular contractions, often causing twisting or repetitive movements or abnormal postures1

1. Fahn S. Dystonia. In: Jankovic J, Hallet M, eds. Therapy With Botulinum Toxin. New York, NY: Marcel Dekker;1994:173-189. 2. National Institute of Neurological Disorders and Stroke, National Institutes of Health, US Department of Health and Human Services. Dystonias fact sheet. National Institute of Neurological Disorders and Stroke Web site. http://www.ninds.nih.gov/disorders/dystonias/detail_dystonias.htm. Published December 2003. Updated April 24, 2009. Accessed June 11, 2009. 3. Langlois M, Richer F, Chouinard S. New perspectives on dystonia. Can J Neurol Sci. 2003;30(suppl 1):34S-44S. 4. Hallett M. Blepharospasm: recent advances. Neurology. 2002;59(9):1306-1312. 5. Benign Essential Blepharospasm Research Foundation (BEBRF). BEBRF blepharospasm pages. Benign Essential Blepharospasm Research Foundation (BEBRF) Web site. http://www.blepharospasm.org. Accessed June 11, 2009. 6. BOTOX® [package insert]. Irvine, CA: Allergan, Inc.; 2010.

Blepharospasm : Definition

• Blepharospasm is the second most common focal dystonia.2 It is a chronic disorder characterized by excessive involuntary closure of the eyelids generally due to spasms of the orbicularis oculi muscles3,4

• The word blepharospasm applies to the wide range of abnormal blinking, eyelid tics, or twitching resulting from any cause.3,5

• Therefore, the term benign essential blepharospasm (BEB) is used to distinguish it from the less serious secondary blinking disorders5


Blepharospasm Musculature1-7

Muscles Role

Orbicularis oculi

(pretarsal part)1,2,6,7,a Closes, blinks, and squints eyes4,5

Corrugator supercilii2,7,b

Draws the eyebrow downward and

inward, with vertical wrinkles in

forehead; the “frowning muscle”4,5

Procerus2,7,b

Pulls the inner angle of the

eyebrows downward and produces

transverse nasal wrinkles4,5

1. BOTOX® [package insert]. Irvine, CA: Allergan, Inc.; 2010. 2. Jankovic J, Kenney C, Grafe S, Goertelmeyer R, Comes G. Relationship between various clinical outcome assessments in patients with blepharospasm. Mov Disord. 2009;24(3):407-413. 3. Netter FH. Atlas of Human Anatomy. 4th ed. Philadelphia, PA: Saunders Elsevier; 2006. 4. Kendall FP, McCreary EK, Provance PG, Rodgers MM, Romani WA. Muscles: Testing and Function with Posture and Pain. 5th ed. Baltimore, MD: Lippincott Williams & Wilkins; 2005. 5. Sommer B, Lowe N. Cosmetic indications. In: Moore P, Naumann M, eds. Handbook of Botulinum Toxin Treatment. 2nd ed. Malden, MA: Blackwell Science; 2003:422-434. 6. Hallett M, ed. Botulinum neurotoxins in the management of blepharospasm. The Neurotoxin Institute Web site. http://www.neurotoxininstitute.org/ chapter_blepharospasm.asp. Accessed June 11, 2009. 7. Seiff SR, Zwick OM. Botulinum toxin management of upper facial rhytidosis and blepharospasm. Otolaryngol Clin North Am. 2005;38(5):887-902.


Procerus muscle

Pulls down skin between eyebrows

Produces horizontal wrinkles over bridge of

the nose

G Espinoza 2011 Relevant anatomy in treating BEB


Corrugator supercilii muscle

Pulls eyebrow downward and medially

Vertical wrinkles in glabellar region



Levator labii superioris alaeque nasi


BEB : Symptoms

• Early stages - irritation and discomfort of the eyelids

- increased blinking associated with light /glare

- may be unilateral or predominantly unilateral

- feeling of dryness or sensation of grittiness

• Progresses to - bilateral involvement over time

- more sustained eyelid closure that interferes with ADL

- difficulty reading, walking , socialising but variable degree

- functional blindness in 12% with sustained closure

- Social recluse


BEB : Aggravated by…

• Bright , dazzling or flickering light

• Stress

• Fatigue

• Driving

• Reading

• Dusty or smoky environments

• High speed travel by train or car


BEB : Relieved by…

• Sleep

• Relaxation

• Walking

• Talking


BEB : Behavioural techniques

• Humming , singing , whistling

• Yawning

• Coughing

• Chewing, eating

• Mouth opening

• Nose pinching

• Rubbing eyelids , or applying pressure to other areas

• Tapping temporal region or covering one eye

• Talking continuously


BEB : Therapy Modalities

• Biofeedback

• Acupuncture

• Hypnosis

• Faith healing

• Herbal therapies

• Relaxation therapy – all with limited relief

• Pharmacotherapy

• Botulinum toxin injections

• Surgery



(Essential) Blepharospasm

• Excessive blinking/eyelid irritation

• Involuntary eye closure/inability to open eyes at

will

• Blepharoclonus

• Initially may appear unilateral

• Sensory geste

• Not suppressible

• Usually progressive, remission rare

• Functionally disabling but treatable

BEB : Surgical Treatment

• Myectomy

• Protractor myectomy has proved most successful

• Complete removal of - Orbicularis oculi

-Corrugator superciliaris

-Procerus

-Extended temporal raphe region

• Full upper myectomy

• Limited



Botulinum toxin : Action

• Interferes with Acetylcholine release from nerve terminals

• Results in temporary paralysis of injected muscles

• Multiple sites as innervation zone is diffuse

• Sub-cutaneous injections 1-10 units using 31 gauge needle

• Represents best treatment available


Botulinum toxin : Local Side effects

• Pain

• Bruising

• Swelling

• Ptosis

• Diplopia

• Lagophthalmos

• Exposure keratitis

• Tearing



BoNT- pitfalls and complications

post-

Dry eyes

Reduced blink rate

treatment

Incomplete eye closure

(nocturnal)

Dry eyes exacerbate

blepharospasm

Drops (and ointment)

especially first 2 weeks

Conditions treated with BOTOX®

Hyperhidrosis

Achalasia

Migraine Strabismus

Blepharospasm

Cerebral Palsy

Cervical Dystonia

Spasticity


Neurologic effects

first noted from

sausage ingestion

1822

Bacteria

identified as

cause of

botulism

1895 1940s

BTX-A

isolated,

purified

1960s

Studies of

BTX-A in

animal muscle

HISTORY OF BOTULINUM NEUROTOXIN TYPE A

DEVELOPMENT - AUSTRALIA

1970s

1993

Hemifacial

spasm,

Blepharospasm

JCP

First tested in

strabismus

patients

First TGA

approval for

BOTOX®

1998 2001

Glabellar

lines

1980s and 1990s

Studied for treatment of

dystonias, spasticity,

selected other conditions

1999

Hyperhidrosis of

the axillaeCervical

Dystonia

2002 2003 2005 2007 2008

Focal

spasticity

in adults

Spasmodic

dysphonia,

strabismus

Upper

facial

Rhytides

Focal

spasticity

in children

≥ 2 yrs


BOTOX® Mechanism of Action1,2 Botulinum toxin type A

1. BOTOX® Approved Product Information Allergan Australia Pty Ltd. 2. Arnon S. et al. JAMA 2001;285(8):1059-1070


RE-ESTABLISHMENT OF NEUROMUSCULAR TRANSMISSION (2)

• Acetylcholine release at new neuromuscular junction with muscle re-stimulated (with lower potential)1

• Sprouts retract and original neuromuscular junction usually returns to functionality within ~3 months2

• Repeated BOTOX® injections required to maintain therapeutic effects2

Functioning

neuromuscular

junction

1. Edgar TS. Journal of Child Neurology 2001;16:37-46 2. BOTOX® Approved Product Information


Differential Diagnosis

• Certain disorders may trigger blepharospasm1

• Blepharitis

• Meibomian gland dysfunction

• Chronic dry eyes

• Apraxia of eyelid opening1

• Primarily due to failure to activate the levator palpebrae muscle

• Motor tics1

• Frequently present as increased blink rate or forceful blinking

• Ocular myasthenia gravis2

• Psychogenic blepharospasm1

1. Brin MF, Danisi F, Blitzer A. Blepharospasm, oromandibular dystonia, Meige’s syndrome and hemifacial spasm. In: Moore A, Naumann M, eds. In: Handbook of Botulinum Toxin Treatment. 2nd ed. Malden MA:Blackwell Science LTD.; 2003:119-141. 2. Elston JS. Botulinum toxin for blepharospasm. In: Jankovic J, Hallett M, eds. Therapy With Botulinum Toxin. New York, NY: 1994:191-197.

Please note: BOTOX® prescribing information states that individuals with neuromuscular junction disorders, such as myasthenia gravis, should be monitored particularly closely when given BOTOX®.

Common Blepharospasm Injection Sites of BOTOX® Neurotoxin

1. BOTOX® [package insert]. Irvine, CA: Allergan, Inc.; 2010. 2. Netter FH. Atlas of Human Anatomy. 4th ed. Philadelphia, PA: Saunders Elsevier; 2006. 3. Kendall FP, McCreary EK, Provance PG, Rodgers MM, Romani WA. Muscles: Testing and Function with Posture and Pain. 5th ed. Baltimore, MD: Lippincott Williams & Wilkins; 2005. 4. Sommer B, Lowe N. Cosmetic indications. In: Moore P, Naumann M, eds. Handbook of Botulinum Toxin Treatment. 2nd ed. Malden, MA: Blackwell Science; 2003:422-434. 5. Hallett M, ed. Botulinum neurotoxins in the management of blepharospasm. The Neurotoxin Institute Web site. http://www.neurotoxininstitute.org/chapter_blepharospasm.asp. Accessed June 11, 2009.

Medial and lateral in upper lid

Lateral in lower lid


Common Injection Sites in the Orbicularis Oculi Muscle

Muscle Site Total Dosage

Medial pretarsal orbicularis oculi of the upper lid 1.25 Units to 2.5 Units

Lateral pretarsal orbicularis oculi of the upper lid 1.25 Units to 2.5 Units

Lateral pretarsal orbicularis oculi of the lower lid 1.25 Units to 2.5 Units

BOTOX® Prescribing Information, November 2011.



Adverse Events with Botulinum Toxin

1. Those due to the expected effects of the neurotoxin on targeted muscles

• e.g., excessive focal muscle weakening1

2. Those due to migration of the toxin to adjacent, non-injected muscles

• e.g., ptosis following peri-orbital injections2

• e.g., dysphagia following neck muscle injections3

3. Those due to systemic distribution of the neurotoxin

• e.g., generalized muscle weakness or autonomic effects following treatment of the extremities4,5

Clinically, adverse events observed with botulinum toxins

generally fall into one of three categories

Dry mouth*

Dysphagia

Nearby

muscle

weakness

Into vessels leading to systemic distribution

*May be a systemic effect

Ptosis

Dry mouth*

Dysphagia

Nearby

muscle

weakness

Into vessels leading to systemic distribution

*May be a systemic effect

Ptosis

1Naumann and Jankovic. Curr Med Res Opin 2004;20:981-990; 2Taylor et al. Canadian J Ophthalmol 1991;26:133-38; 3Mejia et al.

Mov Disord 2005;20:592-597; 4Bakheit et al. J Neurol Neurosurg 1997;62:198; 5Baumann et al. Dermatol Surg 2005;31:263-70.

Treatment

• Allergan Botox Botulinum toxin A

• Ipsen Dysport Botulinum toxin B

• Merz Xeomin Botulinum toxin C

• Company Myobloc Botulinum toxin D


Case Report : Mr P.W.

• 53 year old Indigenous male • Truck driver/Mining operator North West FMG • 12 hour shifts, 2 weeks on, 1 week off. • Lives alone Karratha • 4/12 increased blinking noted initially whilst watching TV • Initially not affecting his driving • Worried he had been put under an Aboriginal spell • Tried salt water washes with cotton buds • Tried wiping wet flannel over eyelids • No eye drops`



Case Study : Mr P.W.

• PMHx – Nil sig

• Alcohol 8-10 beers/day when not working . 1-2 /day when working

• Cigarettes 20/day



• Referred by GP to Neurologist (R.E)

• Unable to continue work as a truck driver

• Frequent blinking/spasms resulting in a tendency for eyes to close

• Forced to extend head backwards and manually open eyes

• Botulinum toxin injections (20u) initially worked well

• Excess blinking but more comfortable

• Referred to Ophthalmologist 18/7/14


Case Study : Mr P.W. Examination

• VA : 6/4 OU

• VF : Full

• Blepharospasm with frequent voluntary blinking

• Forced eye closure -> spasm O.O. narrowing P.A.

• Full eye movement , normal power forced closure

• Lids : Mild blepharitis

• Anterior segments : Cornea ,A/C unremarkable

• Posterior segments : Discs 0.3 , Maculae - healthy




• Effect lasted 3/12

• 18 July 2014 11 Nov 2014 13 Feb 2015

• Reduce the rate of blinking and able to work

• Getting less confident as time goes by.


LEGAL ASPECTS

• Currently in State legislation there is NO mandatory requirement to report people who do not achieve the statutory standards for driving license

• What is our responsibility to the general public?

• What is our responsibility to the Employer?

• What is our responsibility to the patient considering the doctor –patient relationship in as far as confidentiality is concerned?

• How could the condition be monitored or policed objectively?

• Is there a conditional license available to these patients?



• Highlights the interaction between individual professionals such as medical specialists and allied health professionals

• Treatment results in improvements in the workplace

• Reducing potential hazards

• Demonstrating benefits for the workplace

• Demonstrating benefits for workers wellbeing


Learning Objectives

• To recognise a medical condition that affects the health and fitness of a worker in relation to vision

• BEB is diagnosed from the history and examination

• Transitory blindness as seen in BEB can be managed well with specialist treatment

• Input from Ophthalmologists, Neurologists, Psychiatrists, Psychologists, Optometrists, and Support Groups (BEB Australia) allows patients to lead full lives and be a productive part of the workforce despite having a chronic disorder


dr. jeremy raiter - ophthalmic surgeon & physician …a normal blink •frequency 15 times per...

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