hemifacial spasm

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Amr Hasan, M.D. Lecturer of Neurology - Cairo University

CairoNeuro 2014

Botulinum toxin in hemifacial

spasm: A real “Face Off”

Agenda

Hemifacial spasm

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• Anatomy of facial muscles

• Definition

• Aetiology

• Pathophysiology .

• Investigations

• Clinical features and differential diagnosis.

• Botulium toxin in hemifacial spasm

• Video presenations

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Anatomy of facial muscles

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Anatomy of facial muscles (circumorbital and palpebral muscles)

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Anatomy of facial muscles (Nasal Muscles)

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Anatomy of facial muscles (Buccolabial muscles)

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QUIZ

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Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Hemifacial Spasm

Characterized by:

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• Paroxysmal, involuntary

clonic and tonic

synchronous contraction

of the muscles

innervated by the facial

nerve on one side.

• The spasms are due to

brief burst of normal

motor units firing at high

frequency.

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Hemifacial Spasm

Causes:

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• Vascular compression of the

facial nerve by a dolichoectatic

(a distorted, dilated, and

elongated) vertebral artery or

tortuous AICA.

• Facial nerve compression by a

mass, brainstem lesions such as

stroke or multiple sclerosis

plaques.

Hemifacial Spasm

Causes:

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• Secondary causes

such as trauma or Bell

palsy.

• Familial

• Idiopathic

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Hemifacial Spasm

Causes:

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Hemifacial spasm

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transmission, which is electrical activity crossing from one phapticEsynapse.demyelinated neuron to another resulting in a false

root end zone facial nerve at the axonsnvolves abnormal activity of Idemyelination.secondary to compressive damage/

facial nerve or "Kindling theory" involves increased excitability of the nucleus due to feedback from a damaged facial nerve.

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Hemifacial spasm

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• Unilateral (rarely bilateral) spasm involving half of the facial

muscles, typically lasting several minutes at a time.

• Affects women more than men.

• Onset fifth and sixth decade.

• Persists during sleep.

Hemifacial spasm

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• Painless, no sensory loss.

• Involves entire side of face.

• HS most often begins insidiously in the orbicularis oculi

muscle.

Hemifacial spasm

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• Contractions gradually spread to

other muscles of the face

including the platysma

Hemifacial spasm

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Hemifacial spasm

Differential Dx:

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• Blepharospasm

• Meige’s syndrome (not to be confused with Meigs' syndrome,the triad of ascites,pleural effusion and benign ovarian tumor)

• Tourette’s syndrome (multiple compulsive muscle spasms associated with

utterances of bizarre sounds or vile words).

• Trigeminal Neuralgia (acute episodes of pain in the distribution of the V CN)

• Eyelid Myokymia (eyelid twitches; caffeine & stress)

• Tardive Dyskinesia (Orofacial dyskinesia, associated with dystonic

movements of the trunk and limbs, from long-term antipsychotic drug use)

• Eyelid apraxia

Hemifacial spasm

Blepharospasm:

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• Onset: usually in adult life (sixth & seventh decade)

• 3:1 female predominance

• Always Bilateral, episodic, involuntary contractions of the orbicularis

oculi (therefore, may not involve other facial muscles).

• Disappears during sleep.

• Possible association with involuntary spasm of the lower facial

musculature (orofacial dyskinesia or Meige’s Syndrome).

Hemifacial spasm

Blepharospasm:

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• Etiology:

• Adults: usually idiopathic (“essential blepharospasm”) related to

dysfunction of the basal ganglia and limbic system.

• May occur in patients with:

Parkinson’s disease

Progressive supranuclear palsy

Huntington’s disease

Multiple Sclerosis

Brainstem stroke

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Hemifacial spasm

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• Work Up – MRI of cerebellopontine angle to R/O tumor

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Hemifacial spasm

Treatment

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• Pharmachologic: Carbomazepine, Baclofen, Clonazepam,

Neurontin

• Botulinum toxin

• Surgery: Janetta procedure=posterior fossa craniotomy with

insertion of inert material b/w vascular loop and VII nerve (to

decrompress the nerve).

Sites of injection

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Sites of injection

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Sites of injection

Sites of injection

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Sites of injection

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Sites of injection

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Sites of injection for BEB

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Sites of injection

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• Ptosis (7-11%)

• Lagophthalmos (5-12%)

• Symptomatic dry eyes

• Diplopia (<1%)

• Ecchymosis

• Lower facial weakness

Potential adverse effect

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• Facial assymetry.

Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Agenda

Hemifacial spasm

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• Definition

• Aetiology


• Investigations




Case 1

• 32 ys old male.

• Unremarkable medical history.

• 3 years ago started to develop Rt HFS

• Increasing in frequency up to being continous

all over the day

• MRI Brain : Normal

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Case 1

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Case 1

Case 2

• 16 ys old girl.

• Student

• 4 years ago, she had Bell’s palsy

• Afterwhich, she started to develop Rt HFS

• Embarrasing and disfiguring

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Case 2

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Case 2

Case 3

• 42 ys old Yemenese man.

• Employee

• 2 years ago, he started to develop bilateral

facial dyskinsia (ocular, facial, orobuccal)

• Khat

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Case 3

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Case 3

Pearls

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• Up to 50 units at one treatment.

• Injection should not be made inferior to the nasolabial fold! Injections here

cause lip droop that in turns leads to very annoying lip biting by the patient.

• Avoid injecting the mid portion of the upper lid so as to avoid paralyzing the

levator palpebri and causing ptosis.

• Avoid orbicularis oris

• Smaller dose for zygomaticus major

• Lower dose if secondary to facial palsy

hemifacial spasm

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