Blephafrospasm Hemifacial spasm

1. Blepharospasm and Hemifacial spasm
– recent update
L J Basumatary
MBBS,MD,DM
Consultant Neurologist
Excelcare Hospital, Guwahati
Formerly
Associate professor of Neurology
AMC, Dibrugarh

2. Learning objectives
• Movement Disorders
• Focal dystonias
• Hemifacial spasm
• Differentials
• Investigations
• Management protocol

3. Outline
• Hemifacial spasm
• Blepharospasm
• Treatment modalities
• Role of Botulinum toxin

4. Disclosure Information
• In the past 12 months, I have no relevant financial
relationships with the manufacturer(s) of any commercial
product(s) and/or provider(s) of commercial services
discussed in this CME activity.
• I do not intend to discuss an unapproved/investigative use of
a commercial product/device in my presentation.

5. In 1985, Professors Stanley Fahn and David
Marsden created a new society, now known
as the International Parkinson and
Movement Disorders Society (MDS)..
This term was suggested by Professor Lewis
Rowland to Professor Fahn, to encompass
all disorders hypokinetic and hyperkinetic,
which were previously known as
extrapyramidal syndromes.
Source: Camargo CHF, Teive HAG. Drugs in Context 2019; 8: 212586. DOI: 10.7573/dic.212586 2
of 14 ISSN: 1740-4398
What is movement disorder?

6. What is dystonia?
Involuntary muscle contractions
Sustained contraction vs intermittent contraction
(tonic vs clonic)
→ abnormal postures or repetitive movements
Can affect 1 muscle, groups of muscles, or muscles throughout
the body
Thought to be caused by damage or abnormalities in basal
ganglia
Can be idiopathic, genetic, or acquired

7. Cervical dystonia

8. Generalized Dystonia

9. Blepharospasm
“When your eyes can’t stop blinking”

10. Clinical definition
• PRIMARY Blepharospasm1,2
– Benign Essential Blepharospasm (BEB). Also
known as Essential Bleharospasm
• Mostly Idiopathic
• Form of focal dystonia
– Involuntary contractions of the periocular
muscles resulting in forceful eye closure, and
impairing normal opening and closing of the
eyes
1. Cillino S, et al. Eye 2010; 24: 600–607.
2. Berardelli et al., (1985). Brain, 108(Pt 3), 593–608.

11. Clinical definition
• SECONDARY Bleharospasm1
– Ophthalmological cause
• Usually acute and obvious e.g. corneal abrasion
– Drug induced (side effect)
• Dopaminergic antagonists (e.g. neuroleptics)
– Can also occur in the setting of secondary
dystonia (e.g. Cerebral Palsy)
1. Simon GJB, et al. Int Ophtalmol Clin 2005: 49-75.

12. History
• First record of
blepharospasm is
from a 16th century
painting by a Flemish
artist Brueghel called
“De Gaper”
• These patients were
considered mentally
unstable and spasms
were regarded as
psychogenic in origin

13. Primary Blepharospasm
• Adult onset (5th to 7th decade of life) but
younger cases are also seen
• More common in females (~75%)
• Prevalence 1.2-5 cases per 100,000

14. Primary Blepharospasm
• Burden
– Interference with daily tasks
– ~12% incapacitated or functionally blind
– Can cause occupational disability and
depression
1. McCann JD, et al. Int Ophtalmol Clin 2005: 113-21.

15. Blepharospasm
neurophysiology1,2
Tonic or phasic contractions of the orbicularis oculi muscles
May also be associated with levator palpebrae muscle inhibition
Abnormal responsiveness of the blink reflex to sensory stimuli
In patients with blepharospasm, the recovery cycle of the R2
component of the blink reflex is enhanced, presumably owing to
a lack of brain stem interneuronal inhibition
1. Berardelli, A., et al. (1998). Brain, 121(Pt 7), 1195–212.
2. Jankovic, J. (2006). Lancet Neurol, 5, 864–72.

16. Blepharospasm
pathophysiology
• Mutual inhibition of protractors and retractors is
lost
• Spams of the Orbicularis oculi with or without
Corrugator supercilli, procerus and frontalis
muscles results in involuntary forceful blinking
• Muscles innervated by the temporal division of
the Facial Nerve
• Sensory tricks can temporarily relieve symptoms
– Whistling, singing, finger touch of face etc.

17. Etiology
• Cause of BEB (multifactorial) is still debated but consists
of sensory, motor, and central nervous components1
1. Simon GJB, et al. Int Ophtalmol Clin 2005: 49-75.
Genetics?
Sensory Trigger?
Reduced CNS
inhibition
Hyperactive circuit in
the cortical/ sub-
cortical areas
Motor: Periocular
muscle contractions
Hyper-active motor nerve branches of the
facial nucleus, facial nerve, orbicularis oculi,
corrugator and procerus muscles
CNS activates control
center
(Basal ganglia, mid-
brain, or brain-stem)
Sensory nerves respond to stimuli: light,
corneal/eyelid irritation, pain, emotion, stress,
or other trigeminal stimulants

18. Diagnosis
• Early symptoms of Blepharospasm include:
– Increased involuntary blink rate and Eyelid or uppr-
face spasms
– Eye irritations and perception of dry eye
– Eyelid tightness
– Light sensitivity
– Increased wrinkles around the eyes
1. Boyd B, Drews R. Highlights Ophthalmol 1995. 23(4): 39-43.

19. Diagnosis
• Diagnosis of BEB is one of exclusion
– Based on clinical exam
– Rule out secondary blepharospasm due to
ophthalmological causes
– Review history for secondary dystonias including
medication
– Rule out neurological disorders such as Parkinson’s,
Huntington’s, and motor tics
1. Simon GJB, et al. Int Ophtalmol Clin 2005: 49-75.

20. Treatment
• Conservative
• Treatment of choice: Botulinum Toxin Type A
• Oral Medications (limited efficacy)
– Benzodiazepines, anticholinergics, dopaminergic
blocking agents
• Surgery
– Myectomy
1. Simon GJB, et al. Int Ophtalmol Clin 2005: 49-75.

21. Role of BoNT-A in BEB
• Botulinum toxin type A, used in the treatment of
blepharospasm since 1983, has become the
treatment of choice, and is very successful in
controlling eyelid spasms.
• Average doses of toxin type A are 12.5--25 U per eye
for BOTOX®, injected just beneath the skin into the
orbicularis muscle.

22. • Basal ganglia dysfunction
• Overactivity of the seventh nerve
leading to simultaneous forceful
contractions of the eyelid
protractors and retractors
• Ion channelopathy
• Sensitization of the trigeminal
system by photophobia
The mechanism
underlying benign
essential
blepharospasm is
not yet clearly
understood.
Some of the
proposed
mechanisms
https://eyewiki.aao.org/Blepharospasm
Pathophysiology

23. • Orbicularis Oculi
– Preseptal
– Pretarsal
– Orbital
• Frontalis
• Procerus
• Corrugator
• Levator palpebrae
superioris
https://www.sciencedirect.com/topics/neuroscience/blepharospasm
Muscles Involved in Blepharospasm

24. • Sphincter muscle around the eye: pars orbitalis and pars palpebralis
(preseptal and pretarsal part)
• Orbital part originates in the nasal part of frontal bone, frontal process
of maxilla, lacrimal bone (medial part of the orbit), medial palpebral
ligament, runs around the eye via the upper eye cover fold and lid and
returns in the lower eyelid to the lateral palpebral ligament
• Preseptal or palpebral part originates in the medial palpebral ligament
lacrimal sac, runs above and below the eye to the lateral palpebral
ligament
• The orbital and the preseptal muscles form concentric circles around
the eye
• Pretarsal part lies just around the palpebral margin
Orbicularis oculi. Most commonly involved
Anatomy of the periocular muscles

25. Levator
palpebrae
superioris
• arises from the inferior
surface of the sphenoid
bone
• From this point, it diverges
anteriorly to insert into the
skin of the upper eyelid and
the superior tarsal plate
• elevates and retracts the
upper eyelid
Anatomy of the periocular muscles

26. • Originates in the fascia of the nasal bone and upper
nasal cartilage
• Runs through the area of the root of the nose and fans
upward to insert in the skin in the center of the
forehead between the eyebrows
• It acts to pull the skin of the center of the forehead
down, forming transverse wrinkles in the glabella region
and bridge of the nose
• It usually acts together with corrugator or orbicularis
oculi or both
Procerus
Anatomy of the periocular muscles

27. Frontalis
• A thin and quadrilateral muscle adherent to
the superficial fascia
• The frontalis muscle passes through and
inserts into the bundles of the orbicularis
oculi muscle on the superior border of the
eyebrow at the middle and medial side of
the upper eyelid
• The frontalis muscle intermixes with the
bundle of the orbicularis oculi muscle
Anatomy of the periocular muscles

29. Treatment
• Artificial tears, lid scrubs, tinted lenses
• Botulinum toxin injections
• Consider oral medications
• Consider excision of eyelid muscles
(myectomy)

31. Jan 5, 2017

32. BEB Injection Dos and Donts
• Avoid:
– Levator Palabrae Superioris (ptosis)
– Medial lower lid injections (Dilopia)
– Injection very close to lid margin
– Injury to eye (needle should point away from
pupil)
• Total initial dose of BOTOX ® should not be
more than 25 u per eye
1. Allergan Data on File.

33. Pretarsal vs. preseptal
injections
• Data suggest better outcomes with
pretarsal injections
– Aramideh M et al. 1995:
• Significantly more clinical success (81 to 95%)
• Significant decrease in ptosis
• Significant increase in duration (8.5 to 12.5 weeks)
– Cakmur R et al. 2002:
• Significantly higher response rate (97% vs 90%)
• Significantly longer duration (11.4 vs. 8.19 weeks)

34. Recommended units Site of muscle
2.5 -5 units
Volume:
0.05-0.1 ml at each site
 Medial and lateral pre tarsal orbicularis
oculi of upper lid
 Lateral pre tarsal orbicularis oculi of lower
lid
 Medial and lateral pre septal orbicularis
oculi of upper lid
 Lateral pre septal orbicularis oculi of lower
lid
 Medial and lateral pars orbitalis orbicularis
oculi of upper lid
 Lateral pars orbitalis orbicularis oculi of
lower lid
Dosage and Adverse Reactions

35. Comparison of (a) preseptal and (b) pretarsal injection points in
Blepharospasm
• Four injections are usually given in the orbital or preseptal portion of the orbicularis oculi muscle,
can also be injected into the pretarsal portion of the orbicularis oculi.
• Avoid injection into the medial part of pre septal portion to avoid ptosis
• Injection in pretarsal portion of the orbicularis oculi muscle is considered the best method for
treating involuntary eyelid closure due to contractions of this muscle
• Side effects after botulinum toxin injection, including ptosis, diplopia, dry eyes, epiphora, keratitis,
lid edema, entropion/ectropion, and facial weakness, are transient and usually mild

37. Meige Syndrome
• Blepharospasm + oromandibular dystonia
(lower facial spasm)
• BOTH sides affected
British Journal of Ophthalmology 1997;81:439–442

38. Meige Syndrome
• Population
30-70 years old, mean age 55 years old
Female > males (2:1)
5/100,000
• Can start as blepharospasm and then spread
to involve lower face
• Spread is more common early in disease

39. Meige syndrome
• Cause: dopaminergic and cholinergic
hyperactivity
• Decreased functioning of inhibitory neurons
• Abnormal sensorimotor processing
• Abnormal control of CN nuclei in brainstem by
basal ganglia
• Can be primary or secondary

40. Meige Syndrome
• Treatment:
“Sensory tricks”
• 1st line systemic medication
• 2nd line botulinum toxin
– Reserved for blepharospasm component
• Deep brain stimulation

45. Hemifacial spasm: clinical
overview
• What is it?
– A common adult-
onset non-dystonic
disorder
– Involuntary tonic or
clonic spasms of one
side of face
– Severity is variable

46. Clinical features
• Ususally unilateral but may be bilateral
– Bilateral cases have asynchronous
contractions of both sides
• Involuntary contractions start usually in
the periocular muscles but spread to
involve all muscles supplied by the 7th
nerve
• There may or may not be antecedent
history of Bell’s palsy
1. Cillino S, et al. Eye 2010; 24: 600–607.
2. Berardelli et al., (1985). Brain, 108(Pt 3), 593–608.

47. Orbicularis oculi
Procerus
Corrugator
Frontalis
Nasalis
Levator labii superioris
Zygomaticus major
Orbicularis oris
Risorius
Depressor anguli oris
Mentalis
Platysma
Muscles involved

48. Epidemiology
• Adult onset (4th to 5th decade of life)
usually in the periocular muscles
– May spread slowly to involve other muscles
over months to years
• More common in females
• Prevalence
– 14.5 cases per 100,000 in women
– 7.4 cases per 100,000 in men

49. Epidemiology
• Burden
– Interference with daily tasks
– Can cause occupational/ social disability and
depression
– Facial assymmetry
• Commoner in women but men more likely
to seek treatment
• More common in Asian than in Caucasian
populations
Kemp LW et al. Curr Treat Options Neurol 2004; 175-9.

53. Neurophysiology
• Tonic or phasic contractions of the muscles supplied by
7th nerve
• Spasms continue during sleep: sleep may be disturbed
• May also be associated with spasms of stapedius
• Sensory tricks provide temporary relief
• Worsening seen with increased stress, chewing,
speaking, bright light, and cold
1. Berardelli, A., et al. (1998). Brain, 121(Pt 7), 1195–212.
2. Jankovic, J. (2006). Lancet Neurol, 5, 864–72.

54. Neurophysiology
• Theories of causation
– Vascular compression by
aberrant loop (of a branch of
the Posterior Inferior Cerebellar
Artery, [PICA])
– Compression may even be due
to tumors, local infections, or
other vascular malformations
– Focal secondary demyelination
– Ephaptic transmission
1. Kemp LW et al. Curr Treat Options Neurol 2004; 175-9.
2. Tan NC et al. Q J Med 2002; 95: 493-500.

55. Diagnosis
• Early symptoms include:
– Increased involuntary blink rate and Eyelid or
uppr-face spasms
– Eye closure along with synchronous lower face
symptoms such as angle of mouth deviations or
latysmal contractions or clicking noise due
stapedial contractions
• In severe cases there may be sustained
contractions of the affected side of face
1. Boyd B, Drews R. Highlights Ophthalmol 1995. 23(4): 39-43.

56. Diagnosis
• Radiological
– Role of CT
– Role of MR angiography
• Electrohysiological
– EMG

57. Differential Diagnosis

59. Primary or idiopathic hemifacial spasm (presumably caused by
vascular compression of the ipsilateral facial nerve)-62%
Hereditary hemifacial spasm – 2%
Secondary causes
Bell's palsy
Facial nerve injury
Demyelination
Brain vascular insults
Hemifacial spasm mimickers
Psychogenic
Tics
Dystonia
Myoclonus
Hemimasticatory spasm

62. Treatment
• Conservative
• Treatment of choice: Botulinum Toxin Type A
• Oral Medications (limited efficacy)
– Benzodiazepines, Carbamazepine, Baclofen, Phenytoin
etc.
• Surgery
– Janetta’s operation (Microvascular decompression)
• Some patients may experience recurrence of HFS
• Complications include hearing loss and facial weakness
1. Simon GJB, et al. Int Ophtalmol Clin 2005: 49-75.

63. Role of BoNT A In HFS
• Botulinum toxin type A, used in the treatment of
blepharospasm since 1983, has become the
treatment of choice, and is very successful in
controlling eyelid spasms.
• Average doses of toxin type A are 12.5--25 U per eye
for BOTOX ®, injected just beneath the skin into the
orbicularis muscle.

67. Injection sites Average dose per
injection site
Medial and lateral pre – tarsal part of pars palpebralis
part of orbicularis oculi of upper lid
2.5 5 U
Lateral pre – tarsal part of pars palpebralis part of
orbicularis oculi of lower lid
2.5 5 U
Medial and lateral pre – septal part of pars palpebralis
part of orbicularis oculi of upper lid
2.5 5 U
Lateral pre – septal part of pars palpebralis part of
orbicularis oculi of lower lid
2.5 5 U
Medial and lateral pars orbitalis part of orbicularis oculi
of upper lid
2.5 5 U
Lateral pars orbitalis pars orbicularis part of orbicularis
oculi of lower lid
2.5 5 U
Dose and site of Botulinum toxin injection

68. Injection sites Average dose per
injection site
Procerus 1.25 – 5 U
Corrugator 1.25 – 5 U
Frontalis 1.25 – 5 U
Nasalis 1.25 – 5 U
Levator labii superioris 2.5 – 5 U
Zygomaticus major 2.5 – 12 U
Orbicularis oris 1.5 – 5 U
Risorius 1.25 – 5 U
Depressor anguli oris 2.5 – 5 U
Mentalis 2.5 – 5 U
Platysma 5 – 20 U
Dose and site of Botulinum toxin injection

71. Complications After 226 microvascular decompressions
in 194 patients with hemifacial spasm

72. Jordan DR et al. (1989). Essential blepharospasm and related dystonias. Survey of
Ophthalmology, 34(2), 123–132.

74. Key Messages
• Hemifacial spasm is a movement disorder affecting the muscles innervated by the facial nerve. The result
is involuntary tonic or clonic contractions of the facial muscles, which are almost always unilateral.
• The cause in most cases is a compression of the facial nerve in its root-exit zone from the brainstem by a
vessel with an aberrant or ectatic blood vessel.
• The diagnosis is led by the clinical features. Important in deciding treatment is the differential diagnostic
differentiation from other craniofacial movement disorders. Magnetic resonance imaging using CISS
sequence is helpful for diagnosing a possible vascular compression or other intracranial cause.
• The most important symptomatic treatment is local injection of botulinum toxin. In more than 85% of
patients this leads to a notable alleviation of symptoms. The disadvantage associated with his procedure is
the fact that repeated administration at intervals of several months is necessary.
• Microvascular decompression surgery is the only causal treatment option with a success rate of about 85%
in terms of permanent freedom of symptoms.

75. THANK YOU
drbasumatary@gmail.com