Hemifacial spasm - Dr. Amr Hasan Neurology Clinic
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Botulinum toxin in hemifacial spasm: A real “Face Off” Amr Hasan, M.D. Associate Professor of Neurology Cairo University CairoNeuro 2 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 3 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 4 DR AMR HASAN AL HASANY Anatomy of facial muscles 5 DR AMR HASAN AL HASANY Anatomy of facial muscles 6 DR AMR HASAN AL HASANY Anatomy of facial muscles 7 DR AMR HASAN AL HASANY Anatomy of facial muscles 8 DR AMR HASAN AL HASANY Anatomy of facial muscles 9 DR AMR HASAN AL HASANY Anatomy of facial muscles 10 DR AMR HASAN AL HASANY Anatomy of facial muscles 11 DR AMR HASAN AL HASANY Anatomy of facial muscles 12 DR AMR HASAN AL HASANY Anatomy of facial muscles 13 DR AMR HASAN AL HASANY Anatomy of facial muscles (circumorbital and palpebral muscles) 14 DR AMR HASAN AL HASANY Anatomy of facial muscles (circumorbital and palpebral muscles) 15 DR AMR HASAN AL HASANY Anatomy of facial muscles (circumorbital and palpebral muscles) 16 DR AMR HASAN AL HASANY Anatomy of facial muscles (Nasal Muscles) 17 DR AMR HASAN AL HASANY Anatomy of facial muscles (Nasal Muscles) 18 DR AMR HASAN AL HASANY Anatomy of facial muscles (Nasal Muscles) 19 DR AMR HASAN AL HASANY Anatomy of facial muscles (Buccolabial muscles) 20 DR AMR HASAN AL HASANY Anatomy of facial muscles (Buccolabial muscles) 21 DR AMR HASAN AL HASANY Anatomy of facial muscles (Buccolabial muscles) 22 DR AMR HASAN AL HASANY Anatomy of facial muscles (Buccolabial muscles) 23 DR AMR HASAN AL HASANY Anatomy of facial muscles (Buccolabial muscles) 24 DR AMR HASAN AL HASANY Anatomy of facial muscles (Buccolabial muscles) 25 DR AMR HASAN AL HASANY Anatomy of facial muscles 26 DR AMR HASAN AL HASANY QUIZ 15 14 27 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 28 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 29 DR AMR HASAN AL HASANY Hemifacial Spasm Characterized by: • 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. 30 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 31 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 32 DR AMR HASAN AL HASANY Hemifacial Spasm Causes: • 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. 33 DR AMR HASAN AL HASANY Hemifacial Spasm Causes: • Secondary causes such as trauma or Bell palsy. • Familial • Idiopathic 34 DR AMR HASAN AL HASANY Hemifacial Spasm Causes: 35 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 36 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 37 DR AMR HASAN AL HASANY Hemifacial spasm Ephaptic transmission, which is electrical activity crossing from one demyelinated neuron to another resulting in a false synapse. Involves abnormal activity of axons at the facial nerve root end zone secondary to compressive damage/demyelination. or "Kindling theory" involves increased excitability of the facial nerve nucleus due to feedback from a damaged facial nerve. 38 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 39 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 40 DR AMR HASAN AL HASANY Hemifacial spasm • 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. 41 DR AMR HASAN AL HASANY Hemifacial spasm • Painless, no sensory loss. • Involves entire side of face. • HS most often begins insidiously in the orbicularis oculi muscle. 42 DR AMR HASAN AL HASANY Hemifacial spasm • Contractions gradually spread to other muscles of the face including the platysma 43 DR AMR HASAN AL HASANY Hemifacial spasm 44 DR AMR HASAN AL HASANY Hemifacial spasm Differential Dx: • 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) • Tardive Dyskinesia (Orofacial dyskinesia, associated with dystonic movements of the trunk and limbs, from long-term antipsychotic drug use) • Eyelid Myokymia (eyelid twitches; caffeine & stress) • Eyelid apraxia 45 DR AMR HASAN AL HASANY Hemifacial spasm Blepharospasm: • 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). 46 DR AMR HASAN AL HASANY Hemifacial spasm Blepharospasm: • 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 47 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 48 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 49 DR AMR HASAN AL HASANY Hemifacial spasm • Work Up – MRI of cerebellopontine angle to R/O tumor 50 DR AMR HASAN AL HASANY 51 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 52 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 53 DR AMR HASAN AL HASANY Hemifacial spasm Treatment • 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). 54 DR AMR HASAN AL HASANY Sites of injection 55 DR AMR HASAN AL HASANY Sites of injection 56 DR AMR HASAN AL HASANY Sites of injection 57 DR AMR HASAN AL HASANY Sites of injection 58 DR AMR HASAN AL HASANY Sites of injection 59 DR AMR HASAN AL HASANY Sites of injection 60 DR AMR HASAN AL HASANY Sites of injection for BEB 61 DR AMR HASAN AL HASANY Sites of injection 62 DR AMR HASAN AL HASANY Potential adverse effect • Ptosis (7-11%) • Lagophthalmos (5-12%) • Symptomatic dry eyes • Diplopia (<1%) • Ecchymosis • Lower facial weakness • Facial assymetry. 63 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 64 DR AMR HASAN AL HASANY Agenda Hemifacial spasm • Anatomy of facial muscles • Definition • Aetiology • Pathophysiology . • Clinical features and differential diagnosis. • Investigations • Botulium toxin in hemifacial spasm • Video presenations 65 DR AMR HASAN AL HASANY 66 DR AMR HASAN AL HASANY 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 67 DR AMR HASAN AL HASANY Case 1 68 DR AMR HASAN AL HASANY Case 1 69 DR AMR HASAN AL HASANY 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 70 DR AMR HASAN AL HASANY Case 2 71 DR AMR HASAN AL HASANY Case 2 72 DR AMR HASAN AL HASANY Case 3 • 42 ys old Yemenese man. • Employee • 2 years ago, he started to develop bilateral facial dyskinsia (ocular, facial, orobuccal) • Khat 73 DR AMR HASAN AL HASANY Case 3 74 DR AMR HASAN AL HASANY Case 3 75 DR AMR HASAN AL HASANY Pearls • 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. • Smaller dose for zygomaticus major • Avoid orbicularis oris • Lower dose if secondary to facial palsy 76 DR AMR HASAN AL HASANY 77 DR AMR HASAN AL HASANY 78 DR AMR HASAN AL HASANY
